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After run-off transcription by T7 RNA polymerase of mutant U6 genes, in vitro synthesized U6 snRNA molecules were obtained that contained either no U6-0 or up to five U6-5 3¢-terminal UMP

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Biochemical characterization of a U6 small nuclear RNA-specific

terminal uridylyltransferase

Ralf Trippe, Holger Richly* and Bernd-Joachim Benecke

Department of Biochemistry, Ruhr University Bochum, Germany

The HeLa cell terminal uridylyltransferase (TUTase) that

specifically modifies the 3¢-end of mammalian U6 small

nuclear RNA (snRNA) was characterized with respect to

ionic dependence and substrate requirements Optimal

enzyme activity was obtained at moderate ionic strength

(60 mMKCl) and depended on the presence of 5 mMMgCl2

In vitrosynthesized U6 snRNA without a 3¢-terminal UMP

residue was not accepted as substrate In contrast, U6

snRNA molecules containing one, two or three 3¢-terminal

UMP residues were filled up efficiently, generating the

3¢-terminal structure with four UMP residues observed in

newly transcribed cellular U6 snRNA In this reaction, the

addition of more than one UMP nucleotide depended on

higher UTP concentrations The analysis of internally

mutated U6 snRNA revealed that the fill-in reaction by the U6-TUTase was not controlled by opposite-strand nucleo-tides, excluding an RNA-dependent RNA polymerase mechanism Furthermore, electrophoretic mobility-shift analyses showed that the U6-TUTase was able to form stable complexes with the U6 snRNA in vitro On the basis of these findings, a protocol was developed for affinity purifi-cation of the enzyme In agreement with indirect labeling results, PAGE of a largely purified enzyme revealed an apparent molecular mass of 115 kDa for the U6-TUTase Keywords: 3¢ uridylation; affinity chromatography; terminal uridylyltransferase; U6 snRNA

Nuclear pre-mRNA splicing is a process by which introns

are removed from primary transcripts This two-step

trans-esterification mechanism is performed by the spliceosome,

an RNP complex of five small nuclear RNA molecules

(snRNA U1, U2, U4, U5 and U6), and more than 60

proteins (reviewed in refs [1,2]) Spliceosomes are newly

formed on each intron in a well-defined manner Initially,

the 5¢ splice site of the pre-mRNA is recognized by the U1

snRNP Then, U2 snRNP binds to the branchpoint

sequence located near the 3¢-end of the intron Finally, the

spliceosome is completed by incorporation of the U4/U6/

U5 tri-snRNP The splicing reaction requires extensive

rearrangements of RNA–RNA interactions, including the

unwinding of the U4/U6 snRNA duplex and the formation

of a U2/U6/pre-mRNA structure [3–5] The precise

mech-anism by which proteins control these RNA–RNA

inter-actions within the catalytic spliceosome remains to be

elucidated However, evidence exists that intermediate

structural variants of U6 snRNA are involved in RNA–

RNA rearrangements that take place in the center of the

spliceosome [6]

U6 snRNA differs from the other spliceosomal snRNAs

in several ways Unlike other snRNAs, it is transcribed by RNA polymerase III [7,8] and also has a different cap structure [9] Furthermore, U6 snRNA was unusually well conserved during the evolution of eukaryotes [10] and has

no binding site for Sm proteins [11,12] Instead, U6 snRNPs contain LSm-proteins (like Sm) which seem to recognize the 3¢-end oligouridylic structure of the U6 snRNA and which are thought to be involved in U4/U6 snRNP formation [13–16] Finally, U6 snRNA molecules have a remarkable heterogeneity, resulting from extensive post-transcriptional modification of their respective 3¢-termini Most ( 90%) cellular U6 snRNA molecules are blocked

by a cyclic 2¢,3¢-phosphate (> p) 3¢-end group, and  10%

of the U6 snRNA has been found to contain 3¢-oligo(U) stretches up to 20 nucleotides long [17–19] Recently, two highly specific U6 snRNA-modifying enzyme activities have been identified: a 3¢-exonuclease [20] and a terminal uridylyltransferase (TUTase) [21] Both enzymes exclusively accept the 3¢-terminus of U6 snRNA as substrate for the addition or deletion of UMP residues The functional significance of these structural variants of U6 snRNA molecules remains to be elucidated It is conceivable, however, that all of these modifications together form a cyclic process of regeneration of U6 snRNA, which in turn may be essential for the assembly and catalytic function of spliceosomes

In this report, we present a detailed characterization of the reaction catalyzed by U6-TUTase in vitro, with its high selectivity for distinct structural requirements of U6 sub-strate RNA This study also includes an analysis of the U6 snRNA–TUTase interaction in vitro, which provided the basis for further purification to near-homogeneity of this low-abundancy protein

Correspondence to B.-J Benecke, Department of Biochemistry NC6,

Ruhr-University, D-44780 Bochum, Germany.

Fax: + 49 234321 4034, Tel.: + 49 234322 4233,

E-mail: bernd.benecke@ruhr-uni-bochum.de

Abbreviations: TUTase, terminal uridylyltransferase; snRNA, small

nuclear RNA; EMSA, electrophoretic mobility-shift analysis.

*Present address: Department of Molecular Cell Biology, Max Planck

Institute of Biochemistry, D-82152 Martinsried, Germany.

(Received 12 November 2002, revised 13 January 2003,

accepted 17 January 2003)

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Materials and methods

Protein fractionation

Cytoplasmic S100 extracts (15 mgÆmL)1 protein) were

isolated from HeLa cells as described [22] For purification

of the U6-TUTase, 200 mg extract proteins were separated

in a 40-mL Q-Sepharose FF (Pharmacia) column and

fractions obtained by step elution The QS3 fraction (200–

400 mM KCl) was dialyzed against phosphate buffer

[25 mMK2HPO4/KH2PO4(pH 7.9); 0.2 mMEDTA; 20%

glycerol (v/v)] and applied to a 10-mL hydroxyapatite

(Bio-Rad) column The HA2 fraction was obtained by step

elution with 150 mM potassium phosphate in the buffer

system described above and was further fractionated in a

26/60 Superdex G200 column (Pharmacia) For affinity

chromatography, 3 lg oligoA/U6-3 RNA were heated to

70C, cooled down slowly, and incubated for 20 min at

4C with 100 mg washed oligo(dT)–cellulose (Roche

Molecular Biochemicals) in buffer D [20 mMHepes/KOH

(pH 7.9); 100 mM KCl; 5 mM MgCl2; 0.2 mM EDTA;

2 mM dithiothreitol, 20% glycerol (v/v)] This suspension

was packed into a column, loaded with 10 mL fraction

HA2, washed with buffer D, and eluted with the same

buffer containing 600 mMKCl Electrophoresis of proteins

in denaturing 7.5% polyacrylamide gels in the presence of

SDS was essentially as described by Laemmli [23]

Templates

Construction of the U6-3, U6-4 and U6-5 cDNA templates

under control of the bacteriophage T7 promoter has been

described in detail previously [21] Based on the U6-5

construct, U6-0, U6-1 and U6-2 cDNA templates were

generated by PCR with 5¢-TTTAATACGACTCACTAT

AGGGTGCTCGCTTCGGCA-3¢ as upstream primer and

5¢-TATGGAACGCTTCACGAATT-3¢ (U6-0), 5¢-ATAT

GGAACGCTTCACGAATT-3¢ (U6-1) or 5¢-AATATGG

AACGCTTCACGAATT-3¢ (U6-2) as downstream primer,

respectively PCR fragments were purified by agarose gel

electrophoresis and used for in vitro transcription by T7

RNA polymerase as described previously [24] DNA

templates with internal mutations (U6-2/C and U6-1/A)

were obtained as follows: in a first PCR, U6 snRNA-coding

sequences were amplified with a 5¢-primer carrying the

desired mutation and a wild-type 3¢-primer These

frag-ments were used in a second PCR with the same 3¢-primer

but a longer 5¢-primer carrying the T7 promoter as

upstream flanking sequence element The amplified PCR

fragments were cloned blunt-end into the EcoRV site of the

Bluescript KS+vector (Stratagene) PCR fragments

suit-able for in vitro transcription and carrying different numbers

of 3¢-end UMP residues (i.e U6-1 and U6-2) were then

obtained with the T7 promoter-specific upstream primer

and one of the downstream primers described above The

oligo(A)/U6-3 RNA construct was cloned from the

previ-ously described U6-3 RNA gene [21] From this gene, an

AspHI (+5 of U6-DNA)–HindIII (downstream of coding

sequence) fragment was recovered Two synthetic

oligonu-cleotides were hybridized and gave rise to a double-stranded

element containing the oligoA(20)flanked by an upstream

SacII overhang and the first 5 bp of the U6-coding

sequence, in the form of a downstream AspHI overhang These two DNA fragments were inserted simultaneously into the KS+vector, restricted with SacII and HindIII and purified by agarose gel electrophoresis before use After linearization of this plasmid with the DraI enz yme, T7 RNA polymerase transcription from the neighboring KS+ pro-moter yielded suffciently large quantities of oligo(A)/U6-3 RNA for coupling to oligo(dT)–cellulose Primary struc-tures of all constructs were confirmed by sequencing Assay of TUTase

Standard TUTase reaction assays were performed in buffer containing 60 mM KCl, 12 mM Hepes/KOH (pH 7.9),

5 mM MgCl2, 2 mM dithiothreitol, 0.1 mM EDTA, 12% (v/v) glycerol and 5 lCi [a-32P]UTP, in a total volume of

50 lL Substrate RNAs were either 1 lg total cellular RNA

or 50 ng in vitro synthesized U6 snRNA, and incubations were performed for 60 min at 30C Phenol-extracted RNAs were analysed in 6% polyacrylamide gels in TEB buffer [90 mMTris base, 90 mM boric acid, 2 mMEDTA (pH 8.3)] containing 6M urea Electrophoresis was for

50 min at constant 30 W To discriminate between RNA molecules differing in length by only 1 nucleotide, high-resolution gels were used in some experiments These gels contained 7% polyacrylamide, were twice as long (50 cm), and were run for 210 min at constant 37.5 W Autoradio-graphy of the dried gels was at)70 C for 16 h, using a Cronex intensifier screen

Electrophoretic mobility-shift assay (EMSA) For EMSAs, U6-3 RNAs were synthesiz ed in vitro with T7 RNA polymerase (Fermentas), in the presence of [a-32P]UTP (800 CiÆmmol)1; New England Nuclear) Labe-led U6-3 RNA (100 000 c.p.m.) was incubated with Super-dex G200 fractions for 10 min at 4C in 12 mM Hepes/ KOH (pH 7.9), containing 60 mM KCl, 5 mM MgCl2,

2 mM dithiothreitol, 0.1 mM EDTA, 1 lg yeast tRNA (Roche Molecular Diagnostics) and 12% (v/v) glycerol Electrophoresis in nondenaturing 6% polyacrylamide gels with 0.25· TEB buffer was at 6 VÆcm)1for 4 h at 4C

Indirect labeling analysis Affinity-purified TUTase was incubated with labeled U6-3 RNA (100 000 c.p.m.) as described for shift analyses UV cross-linking (Hoefer UVC 500) was at 0.3 JÆcm)2Æmin)1for

10 min on ice Subsequently, half of the reaction mixture was incubated with 10 lg RNase A for 10 min at room temperature After acetone precipitation, proteins from both fractions were analysed in 7.5% polyacrylamide/SDS Laemmli gels

Results Ionic requirements of HeLa cell TUTases Earlier experiments had established that, aside from an unspecific enzyme, HeLa cells contain a highly specific TUTase that exclusively modifies the 3¢-ends of U6 snRNA molecules To separate this activity from the unspecific

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enzyme and also to remove contaminating RNases, a

protocol had been developed for the preparation of partially

purified enzyme fractions [21] This purification started with

phosphocellulose P11 chromatography of HeLa cell S100

extracts Subsequently, the unspecific TUTase was

separ-ated from the U6-TUTase by gel filtration in Superdex

G200 These two crude enzyme fractions were analysed in

parallel for magnesium and salt (KCl) dependence of their

respective transferase reactions, with 1 lg total RNA from

HeLa cells added as substrate As shown in Fig 1A,B, the

two activities separated by gel filtration were clearly

distinguishable by their acceptance of substrate RNA In

agreement with our previous results [21], the unspecific

enzyme (unspec; left panels of Fig 1A,B) modified a variety

of cellular RNA molecules, whereas the U6-specific TUTase

(spec; right panels) exclusively accepted U6 snRNA as substrate However, the two enzymes revealed similar salt optima As seen in Fig 1A, both activities showed a clear optimum at 5 mMMgCl2(lanes 2 and 6), with no activity detectable in the absence of Mg2+ ions (lanes 4 and 8) Corresponding results for the two enzymes were also observed with respect to ionic strength (Fig 1B) With both enzymes, optimal conditions for the transfer of UMP residues were obtained in the presence of 60 mM KCl (Fig 1B, lanes 1 and 6) This is the amount of KCl already provided by the protein fractions, with no further salt added

to the reaction It should be noted that lower amounts of KCl (40 mM), obtained after dialysis of protein fractions, did not further increase the enzyme activity (not shown) However, both enzymes were inhibited significantly by higher salt concentrations (150 mMKCl; lanes 3 and 8) and showed no detectable activity at and above 200 mM KCl (lanes 4 and 5 and 9 and 10) Therefore, although slight differences between the two enzymes were observed in response to higher salt conditions, these two TUTase activities basically depended on similar reaction conditions, but with clearly different substrate requirements These results on Mg2+ and KCl dependence were primarily required to establish the affinity-purification protocol for the U6-TUTase, described below Therefore, we did not analyse in more detail the suitability of other bivalent cations and/or salts such as manganese or ammonium sulfate

Restoration of authentic 3¢-ends by the U6 snRNA-specific TUTase

Next, we wanted to determine RNA substrate requirements

of the U6-TUTase For this, a U6-TUTase fraction was used that was prepurified by Q-Sepharose and hydroxy-apatite chromatography, followed by gel filtration in Superdex G200 (see Materials and methods section) U6 snRNA molecules were synthesized as substrates that differed with respect to the number of UMP residues at their 3¢-ends respectively After run-off transcription by T7 RNA polymerase of mutant U6 genes, in vitro synthesized U6 snRNA molecules were obtained that contained either

no (U6-0) or up to five (U6-5) 3¢-terminal UMP residues To ensure homogeneity of the RNA molecules applied, in vitro synthesized RNA was first separated in high-resolution polyacrylamide gels, and transcripts of the correct length were recovered, before their use as substrate in a standard TUTase reaction with [a-32P]UTP Subsequently, labeled U6 snRNA molecules were analysed again in high-resolu-tion gels In this analysis, U6-3 marker RNA, labeled by T7 RNA polymerase transcription of the corresponding tem-plate, was included as a size standard (m in Fig 2A) From the results presented in Fig 2A it is evident that U6-1, U6-2 and U6-3 RNA molecules (lanes 2–4) were efficient substrates for the U6-TUTase reaction Furthermore, the high-resolution capacity of our gel system allowed the resolution of closely related molecules, differing in length by only one nucleotide A close examination of the bands in lanes 2–4 of Fig 2A revealed that concomitant with increasing length of the UMP tails of the substrate RNA,

a decreasing number of distinguishable labeled RNA products was obtained: three bands in lane 2, two in

Fig 1 Ionic requirements of HeLa cell TUTases The U6

snRNA-specific (spec.) and the unsnRNA-specific (unspec.) TUTase of HeLa cell

extracts were separated in a Superdex G200 column [21] and analysed

with 1 lg total cellular RNA as substrate (A) Peak fractions of the

two activities were tested in the presence of various concentrations of

MgCl 2 (Mg ++ ): 10 m M (lanes 1,5), 5 m M (lanes 2,6), 2.5 m M (lanes

3,7) and 0.0 m M MgCl 2 (lanes 4,8) (B) Same analysis as in (A), except

that standard TUTase reactions (5 m M MgCl 2 ) were performed in the

presence of increasing amounts of KCl: 60 m M (lanes 1,6), 110 m M

(lanes 2,7), 150 m M (lanes 3,8), 200 m M (lanes 4,9) and 250 m M KCl

(lanes 5,10) Analysis of labeled RNA products was in 6%

poly-acrylamide gels containing 6 M urea Exposure of the dried gels to

Kodak X-ray films was for 16 h using a Cronex intensifier screen.

The position of labeled U6 snRNA is indicated by an arrow and m

represents labeled marker DNA.

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lane 3, and a single band in lane 4 In comparison with the

labeled U6-3 marker RNA, these results indicate that the

U6-TUTase has a clear preference to fill up the 3¢-ends of

U6 snRNA molecules to the four UMP residues found in

newly transcribed cellular U6 snRNA The 3¢-ends were not

elongated further, as evidenced by the absence of labeled

products observed with U6-4 (lane 5) and U6-5 (lane 6) as

substrate RNA Furthermore, the U6-TUTase did not

accept U6-0 RNA (lane 1) as substrate, indicating that at

least one pre-existing UMP residue at the 3¢-end of U6

snRNA is a prerequisite for this modification reaction to

take place In addition, the finding of intermediates of the

transferase reaction (Fig 2A, lanes 2 and 3) seemed to point

to dependence on the nucleotide concentration of the chain

elongation rate Therefore, TUTase reactions were

per-formed as before with U6-1 RNA and [a-32P]UTP, but this

time in the presence of increasing amounts of unlabeled

UTP (Fig 2B) Again, U6-3 RNA labeled by T7

transcrip-tion in vitro was included as size marker (m) The

comparison in Fig 2B of the standard reaction products,

i.e in the presence of labeled UTP only (lane 1), with those obtained in the presence of increasing amounts of unlabeled UTP (lanes 2–5) shows a clear shift to the full-length modification product (U6-4 RNA, lane 5), at the expense of the intermediate bands seen in lanes 2–4 of Fig 2B It should be noted that the significant reduction in overall signal intensity (lanes 3–5) is simply due to the competition for incorporation of labeled nucleotides by the excess of unlabeled UTP Together, we conclude that the U6 snRNA-specific TUTase reaction depends on both the 3¢-end structure of the template RNA and the concentration

of substrate nucleotides present in the reaction mixture Structural requirements for the U6 substrate RNA The finding that the U6-TUTase preferentially fills in the 3¢-end of U6 snRNA only to four UMP residues raises the question of how this elongation reaction is controlled This

is even more intriguing because the proposed secondary structure of U6 snRNA (Fig 3A [25]) has an extended 3¢–stem–loop structure with exactly four internal AMP nucleotides (+27/+30) opposing the four 3¢-terminal UMP residues (+103/106) found in newly transcribed cellular U6 snRNA Therefore, it is tempting to speculate that the U6-TUTase may be guided by the sequence of the opposite strand, establishing some sort of substrate-specific RNA-dependent RNA polymerase reaction To test this hypo-thesis, we analysed two U6 mutant genes One (U6-2/C) consisted of a U6-2 clone that contained one additional CMP nucleotide, inserted between positions +27/+28 (two

of the four AMP residues mentioned above) The corres-ponding mutant U6-2/C substrate RNA was tested either in

a standard TUTase reaction (with labeled UTP) or in the presence of32P-labeled GTP, supplemented with unlabeled UTP The analysis of the modified RNA is shown in Fig 3B, with unmodified U6-2/C RNA, labeled during T7 transcription, as size marker (lanes 1 and 4) The result shown in lane 2 of Fig 3B confirmed that, in a standard TUTase reaction, U6-2/C RNA still served as an efficient substrate for the transferase reaction Surprisingly, however, the TUTase reaction stopped after the addition of one UMP residue and did not fill-up this U6 mutant RNA to the four UMP residues observed previously (compare with lane 3 of Fig 2A) This is evident from the size comparison between the modified U6-2/C RNA (Fig 3B, lane 2) and the unmodified control RNA (lanes 1 and 4) In contrast, the TUTase reaction performed with the same substrate RNA und unlabeled UTP, but in the presence of labeled GTP as tracer, did not give rise to any labeled RNA product (Fig 3B, lane 3) This indicates that the newly introduced CMP nucleotide at position +28 of the mutant gene was not functional as a template-strand nucleotide, able to direct the incorporation of GMP residues into the opposite 3¢-end of the mutant RNA

A second mutation of the U6 substrate RNA consisted of the introduction of two additional AMP nucleotides in front

of the oligo(A) stretch, beginning at +27 of the wild-type sequence This mutation was introduced into a U6-1 construct (resulting in U6-1/A) and aimed to extend the internal oligo(A) sequence from four to six AMP residues The analysis of this mutant RNA as substrate for the U6-TUTase is shown in Fig 3C We have shown above

Fig 2 RNA substrate requirements of the U6-TUTase (A) A partially

purified U6-TUTase was tested under standard reaction conditions

with 50 ng in vitro synthesized U6 snRNA substrate molecules

containing zero (lane 1), one (lane 2), two (lane 3), three (lane 4), four

(lane 5) or five (lane 6) 3¢-terminal UMP residues A size standard

consisting of unmodified U6-3 RNA, labeled by T7 RNA polymerase

transcription in vitro, is indicated (m) Labeled RNA products were

analysed in high-resolution gels as described in Materials and methods.

(B) The same enz yme as in (A) was tested with [a-32P]UTP and U6-1

RNA as substrate under standard reaction conditions In this case,

however, increasing amounts of unlabeled UTP were added to the

reaction: 0.0 l M (standard reaction; lane 1), 0.25 l M (lane 2), 0.5 l M

(lane 3) 1.0 l M (lane 4) and 2.0 l M (lane 5) As above, unmodified

labeled U6-3 RNA was included as a size standard (m).

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that, in these TUTase reactions, the nucleotide

concentra-tions may be a limiting factor for the prolongation of U6

substrate RNA Therefore, as in Fig 2B, the TUTase

reactions were performed either under standard conditions,

i.e in the absence of unlabeled UTP (lane 1), or with

increasing amounts of unlabeled UTP added to the reaction

(lanes 2–5) As before, increasing amounts of unlabeled

nucleotides led to the disappearance of modified

inter-mediates and to an overall reduction in signal intensities

However, comparison of the longest labeled products in

lanes 2–5 with the two different unmodified marker RNAs

(m1, m2) revealed that the TUTase-catalysed reaction again

stopped exactly at a position corresponding to four

3¢-terminal UMP residues In this analysis, the unmodified

marker RNAs, labeled during T7 transcription, were U6-3

RNA (m1) and U6-2/A RNA (m2) In its unmodified form,

the length of U6-3 RNA exactly matches that of the

unmodified U6-1/A sequence The U6-2/A marker RNA

corresponds to the U6-1/A mutant analysed here, but

containing two instead of one 3¢-terminal UMP residues

Consequently, migration of this unmodified marker RNA

should correspond exactly to the smallest labeled band of

the modified U6-1/A RNA This is confirmed by

compar-ison of the lower bands of lanes 1–4 with the labeled m2

band (Fig 3C) These data provide evidence that the

enzyme was not able to read as template the two additional

AMP nucleotides of the opposite RNA strand Therefore,

we conclude that the TUTase does not act as an

RNA-dependent RNA polymerase Rather the enzyme catalyses a

strictly selective modification reaction, solely to regenerate

the authentic 3¢-structure of newly transcribed cellular U6

snRNA, constituted by four UMP residues

Complex formation of the U6-TUTase

with substrate RNA

The U6 snRNA-specific TUTase described here differs from

the unspecific enzyme of HeLa cells by its high selectivity for

its substrate RNA Therefore, we wanted to know whether

this highly specific RNA–protein interaction might provide

a useful tool for affinity purification of the enzyme To test this possibility, we first studied binding of the TUTase to U6 snRNA by EMSA For this, a partially purified U6-TUTase was run in a preparative Superdex G200 gel filtration column and individual fractions tested with 1 lg total cellular RNA (Fig 4A) As indicated by the labeled U6 snRNA products, the specific enzyme was obtained as a broad peak with fractions 27 through 51, corresponding to a size range from 70 to 130 kDa for elution from this column

Fig 3 Substrate analysis of the TUTase with structural mutants of the

U6snRNA (A) Proposed secondary structure of U6 snRNA [25].

Mutant RNAs were cloned by insertion of CMP or AMP nucleotides

into the internal oligo(A) stretch (+27/+30) of the wild-type

sequence (B) U6-2/C substrate RNA represents a U6 snRNA

con-taining two 3¢-terminal UMP residues and one additional internal

CMP nucleotide, inserted at position +28 of the wild-type sequence.

50 ng of this RNA were tested with labeled UTP under standard

reaction conditions (lane 2) or with labeled [a-32P]GTP in the presence

of 2.0 l M unlabeled UTP (lane 3) The position of unmodified U6-2/C

RNA is indicated on the right This marker RNA (lanes 1,4) was

labeled during T7 transcription in vitro and served as a size standard.

(C) U6-1/A RNA contains one 3¢-terminal UMP residue and an

insertion of two additional AMP residues at position +27 of the

wild-type sequence U6-1/A RNA was analysed either under standard

conditions (lane 1) or in the presence of increasing amounts of

unlabeled UTP: 0.25 l M (lane 2), 0.5 l M (lane 3), 1.0 l M (lane 4) and

2.0 l M (lane 5) T7 RNA polymerase-labeled marker RNAs were:

U6-3 (m 1 ) and U6-2/A (m 2 ); the latter corresponds to U6-1/A, but with

two 3¢-terminal UMP residues (see text).

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A clear maximum of enzyme activity was detected in

fractions 39–45 Subsequently, aliquots of the gel filtration

fractions were incubated with U6-3 RNA, labeled by T7

RNA polymerase transcription in vitro Complexes were

separated in 6% polyacrylamide gels, as described in

Materials and methods As shown in Fig 4B, the fractions

with maximum enzyme activity, mainly fractions 39–45,

clearly shifted the free U6 snRNA (arrowhead) to a

complex of higher molecular mass (arrow) Furthermore,

the distribution between fractions of the major shifted

complex paralleled that of the enzyme activity observed in

Fig 4A Additional minor complexes did not correspond to

the pattern of enzyme activity, and may represent other

cellular proteins capable of binding U6 snRNA, either

specifically (such as LSm proteins) or in an unspecific way

These results confirm that in vitro stable complexes may

be obtained between U6 snRNA and the corresponding

U6-TUTase

In a second step, we wanted to generate a U6

snRNA-based affinity column for purification of the enzyme As

proteins have a significant tendency to bind nonspecifically

to a variety of matrices, the choice of carrier is important in

affinity purification Previous experiments had shown that cellulose may be superior to other materials (unpublished observation) Another important point is the accessibility of the immobilized RNA TUTase is expected to primarily recognize the 3¢-terminal structure of U6 snRNA However, bulky compounds such as biotinylated nucleotides may interfere with the correct folding of the target RNA, thereby changing the structural motif recognized by the TUTase Therefore, we decided to couple U6 snRNA to oligo(dT)– cellulose via an oligo(rA) linker, fused to the 5¢-end of the wild-type sequence For this, a mutant gene was cloned with

an oligo(A)(20)linker preceding the U6-3 RNA sequence

In vitrotranscription of this template by T7 RNA poly-merase gave rise to U6-3 transcripts 149 nucleotides in length A control experiment confirmed that the presence of the oligo(rA) linker and the joining element did not interfere with the TUTase reaction This is shown in Fig 5A Comparison of lanes 2 and 3 shows that the amount of modified U6 RNA labeled in a standard TUTase reaction stayed the same, irrespective of whether U6-3 RNA (lane 2)

or oligo(A)/U6-3 RNA (lane 3) was applied In this standard U6-TUTase reaction, a control was included with

1 lg total cellular RNA (Fig 5A, lane 1) It should be noted that the slightly slower migration observed with the modified U6-3 RNA (lane 2), compared with cellular U6 snRNA (lane 1), is due to two additional 5¢-terminal GMP residues in the U6-3 RNA The resulting three 5¢-terminal GMP nucleotides of such U6 constructs are required for efficient initiation of transcription by T7 RNA polymerase

Fig 5 Affinity chromatography of U6-TUTase with immobilized 5¢-adenylated U6snRNA (A) In vitro transcribed oligo(A)/U6-3 RNA (149 nucleotides in length; 50 ng) was tested in a standard TUTase reaction (lane 3) in comparison with the authentic U6-3 sequence (lane 2) Lane 1 shows a control reaction with 1 lg total cellular RNA Labeled DNA fragments are included as marker (m) Products were analysed in 6% polyacrylamide gels containing 6 M urea (B) After dialysis against 100 m M KCl, a partially purified U6-TUTase fraction (hydroxyapatite step, see Materials and methods) was applied to an oligo(dT)–cellulose column loaded with oligoA/U6-3 RNA The load fraction (lane 1), the flow-through material (lane 2), and the fractions eluted with 600 m M KCl (lane 3) or 2000 m M KCl (lane 4) were analysed in a standard TUTase reaction with 1 lg total cellular RNA Electrophoretic analysis of labeled RNA products in 6% polyacryl-amide gels and autoradiography were as before.

Fig 4 Complex formation of U6-TUTase with substrate RNA (A)

Prepurified U6-TUTase was run in a Superdex G200 column and the

fractions indicated above each lane tested under standard conditions

with U6-3 RNA as substrate The loaded material is indicated by l and

labeled DNA used as a marker indicated (m) (B) EMSA with the same

TUTase fractions as in (A) and T7-transcribed labeled U6-3 RNA as

substrate Electrophoresis was in nondenaturing 6% polyacrylamide

gels (see Materials and methods) Lane 1 shows a minus-protein

control, with the free U6-3 RNA marked by an arrowhead The shifted

complex is indicated by an arrow (left side).

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This system was used for affinity chromatography of the

U6-TUTase For this, in vitro synthesized oligoA/U6-3

RNA was coupled to an oligo(dT)–cellulose matrix via A/T

base-pairing In an initial experiment, a fairly crude protein

fraction (hydroxyapatite step) was applied to the column

At this level of purification, residual amounts of the

nonspecific cellular TUTase are still present This is evident

from the standard TUTase reaction with total cellular RNA

as substrate (Fig 5B) As seen in lane 1 of Fig 5B, the load

fraction of the affinity column was able to transfer UMP

residues to more than just U6 snRNA, although the latter

was by far the most abundant labeled reaction product In

contrast, very little (if any) TUTase activity was associated

with the flow-through fraction (lane 2) It should be noted

that most of the minor bands seen with the load material

(lane 1) were obtained in this flow-through fraction As

these labeled bands probably reflect residual activity of the

nonspecific TUTase, it appears that the nonspecific enzyme

did not bind to the affinity matrix Subsequently, two more

fractions were step-eluted with 600 mM KCl (lane 3) and

2M KCl (lane 4) Before being analysed for TUTase

activity, these fractions were dialysed against 100 mMKCl

This confirmed that most TUTase molecules were eluted

from the affinity column at 600 mMKCl (lane 3)

Further-more, the upper section of lane 3 in Fig 5B indicates that a

small amount of the oligo(A)/U6-3 target RNA was also

eluted from the affinity column One has to keep in mind,

however, that in this analysis the TUTase reaction was

performed in the presence of 1 lg total cellular RNA as

substrate Taking into account the low concentration of U6

snRNA in total cellular RNA, even the smallest amounts of

oligo(A)/U6-3 RNA coeluted from the column would

attract considerable labeling by the U6-TUTase Such

artificial leakage of a relatively small proportion of the

immobilized target RNA, however, would not affect the

general suitability of this purification step

With this information to hand, a purification scheme was

developed for the U6 snRNA-specific TUTase Starting

with HeLa cell S100 extracts, the enzyme was prepurified by

Q-Sepharose, hydroxyapatite chromatography, and gel

filtration in Superdex G200 This partially purified

U6-TUTase fraction was subjected to affinity

chromato-graphy on the oligo(A)/U6-3 RNA column described above

As proteins tend to bind to any matrix in an unspecific way,

the combined peak fractions of the G200 column were split

and run in parallel in two affinity columns: one consisting of

oligo(dT)–cellulose only (– RNA column) and a second

one loaded with poly(A)/U6-3 RNA (+ RNA column)

The TUTase assay performed with the material eluted from

both columns at 600 mMKCl confirmed that binding of the

enzyme strictly depended on the presence of the substrate

RNA (Fig 6A) As seen in lane 2 of Fig 6A, the TUTase

activity was exclusively eluted from the + column In

contrast, virtually no enzyme activity was detectable in lane

1, representing the elution fraction of the mock column (–)

In agreement with these findings, the distribution of enzyme

activities associated with the two flow-through fractions was

exactly the other way around, i.e full activity in the case of

the mock column and less than 5% of TUTase passing

through the + column (data not shown) To determine

more precisely the molecular mass of the U6-TUTase,

indirect labeling experiments were performed with the

affinity-purified enzyme Labeled U6-3 RNA was incubated with the protein under shift conditions, followed by UV cross-linking Analysis of labeled proteins in SDS/poly-acrylamide gels was either directly (–) or after RNase A digestion (+) Figure 6B shows that a distinctly labeled RNP complex  145 kDa in size was obtained without RNase A treatment (lane 1) As expected, RNase treatment

of the cross-linked material resulted in an overall loss of radioactivity associated with proteins (lane 2) Furthermore, albeit low in intensity, now one additional new band appeared that was not observed in lane 1 This band (arrow) had an apparent molecular mass of 115 kDa All other labeled bands visible in lane 2 of Fig 6B (mainly corres-ponding to a size range of 45–60 kDa) were already detectable in the absence of RNase treatment These bands probably represent cross-linking products labeled by resid-ual amounts of free UTP

Finally, the protein composition of the affinity-purified material was analysed in silver-stained SDS/polyacrylamide gels Initial results showed that high-salt elution from the affinity matrix still resulted in a very complex spectrum of polypeptides, not allowing unambiguous identification of the TUTase (data not shown) The high-salt conditions obviously mobilized a large number of proteins unspecifi-cally bound to the matrix Therefore, a more gentle mode of elution was applied, avoiding changes in ionic strength This approach consisted of RNase A treatment of the affinity columns, and indeed resulted in recovery of vastly reduced

Fig 6 Affinity purification and indirect labeling of U6-TUTase (A) U6-TUTase prepurified by Q-Sepharose, hydroxyapatite and Super-dex G200 was loaded in parallel to an oligo(dT)–cellulose column (–)

or an oligo(dT) column coupled with oligoA/U6-3 RNA (+) After being washed with 100 m M KCl, material eluted at 600 m M KCl was tested for TUTase activity with U6-3 substrate RNA in a standard reaction Labeled products obtained with the elution fraction of the – column (lane 1) and of the + RNA column (lane 2) were analysed as before DNA marker fragments are shown on the left (m) (B) Affinity-purified TUTase was incubated with labeled U6-3 RNA under shift conditions, followed by UV cross-linking Half of the material was analysed directly (without nuclease digestion, –, lane 1) and half after RNase A treatment (+, lane 2) in SDS/polyacrylamide gels (see Materials and methods) Numbers on the right indicate the positions

of unlabeled marker proteins (kDa).

Trang 8

numbers of proteins Electrophoretic analysis of proteins

obtained from the + and – RNA affinity columns is

presented in Fig 7 As is evident from lanes 1 and 2, a

simple protein spectrum was obtained after RNase A

elution (lane 3 shows a control with the RNase A alone)

Most importantly, the fractions obtained from the + (lane

1) and the – (lane 2) columns differed by one prominent

polypeptide only (arrow) Apart from a few minor

quan-titative differences, all other bands were identical between

the two fractions The prominent band selectively eluted

from the + column showed an apparent molecular mass of

115 kDa, which is in full agreement with the size obtained

previously for the U6-TUTase by indirect labeling

(Fig 6B) Together, these lines of evidence suggest that

the 115-kDa protein is probably the human U6

snRNA-specific TUTase

Discussion

By several criteria, U6 snRNA is remarkable among the

small stable RNA molecules of eukaryotic cells (see the

Introduction) Furthermore, it seems to play a major role in

the center of the active spliceosome The finding of

accompanying enzymes responsible for the highly specific

modification of this particular RNA, such as a 3¢-nuclease

and a 3¢-terminal uridylyltransferase [20,21], supports the

structural significance of U6 snRNA Consequently, one

would expect that the combined action of these two proteins

(and presumably others) is closely associated with the biological function of U6 snRNA in pre-mRNA splicing Therefore, detailed information on the reactions catalysed

by these two U6-specific enzymes may provide a valuable tool for studying internal events within the spliceosome Here, we have reported on the catalytic properties and substrate requirements of the U6 snRNA-specific TUTase With respect to its general properties, it seems to correspond closely to the nonspecific TUTase (Fig 1) [26] Both enzymes were inhibited by high salt concentrations and showed maximal stimulation by 5 mM Mg2+, but with clearly different RNA substrate specificities In contrast with the U6-TUTase, however, the reaction catalysed by the nonspecific enzyme was found to be strictly limited to the transfer of only one UMP residue [26], irrespective of whether or not higher concentrations of UTP were applied The transfer of more than one UMP residue by the U6-TUTase (Fig 2B) was not observed in our initial study [21], because it depends on the presence of higher UTP concentrations In addition, these experiments were per-formed with substrate RNAs that contained three (or more) 3¢-terminal UMP residues At first glance, the finding that the U6-TUTase is able to transfer more than one UMP may classify it as a common poly(U) polymerase However, for this class of enzyme, the length of the poly(U) product is a function of the incubation time Certainly, this was not the case here The addition of UMP residues was strictly limited

to four, thereby restoring the 3¢-terminal structure of newly transcribed cellular U6 snRNA Therefore, the mode of action of the U6-TUTase appears to be tightly controlled by the structure of the RNA substrate This notion is supported

by the observation that a synthetic U6-0 RNA, containing

no 3¢-UMP at all, was not accepted as substrate As U6-0 RNA carries an AMP nucleotide at its 3¢-end, this finding was reminiscent of results obtained with various substrate RNAs in unfractionated HeLa cell extracts and frog oocytes [27] One has to bear in mind, however, that the uridylating activity analysed in those experiments did not show any substrate RNA specificity

The four 3¢-terminal UMP residues of newly tran-scribed U6 snRNA exactly match four internal AMP residues Consequently, it is tempting to speculate that the U6-TUTase functions as an RNA replicase How-ever, analysis of constructs with internal opposite strand mutations definitely excluded such a mode of action Surprisingly, even the introduction of two additional AMP residues into the internal oligo(A) stretch did not give rise to an extended TUTase reaction product, now containing six instead of four complementary 3¢-terminal UMP residues Therefore, it appears that the underlying principle is not simply to ensure a double strand at the basis of the 3¢-terminal stem-loop structure of U6 snRNA Rather, these results suggest that a sophisticated mechanism controls a highly restrictive elongation pro-cess, only allowing restoration of the authentic 3¢-end of U6 snRNA Such a scenario attributes a special import-ance to the 3¢-terminal structure of this RNA, with four UMP residues being involved in base-pairing Apart from a more general contribution to the overall folding

of U6 snRNA, this A/U RNA duplex element may ensure the appropriate stability for melting and reasso-ciation of this spliceosomal RNA, a prerequisite to the

Fig 7 SDS/PAGE of proteins recovered from affinity

chromato-graphy U6-TUTase was affinity-purified as described in Fig 6 In this

case, however, elution of the bound material from the plus (+, lane 1)

and the minus (–, lane 2) column was by treatment with 100 lg

RNase A Lane 3 shows the control analysis of the RNase A used for

elution Protein bands were visualized by silver staining of the gel The

molecular mass (kDa) of marker proteins (m) is indicated on the left.

Trang 9

extensive RNA rearrangements that occur during the

splicing procedure

Several uridylating enzyme activities have been

charac-terized [26,28–30] However, the TUTase analysed here

differs from those by its pronounced RNA substrate

specificity [21] This RNA selectivity is superimposed by

the highly specific control of the elongation reaction

described above In this context, it is interesting to note

that the molecular mass of the U6-TUTase activity obtained

under native conditions in the gel-filtration column

(Fig 4A) exactly corresponded to that of the specific

polypeptide observed in denaturing gels, after affinity

chromatography (Fig 7) This supports the notion that

the catalytic activity of the U6-TUTase, together with its

two specificities outlined above, is associated with a single

polypeptide chain Therefore, binding of the U6-TUTase to

its target RNA will certainly establish an interesting model

system for studying a very specific but transient RNA–

protein interaction For such a detailed analysis, however, a

recombinant TUTase will be required Such a recombinant

protein would also allow a structural comparison of the

enzyme with other previously cloned TUTases [30,31] In

addition, the availability of a recombinant U6-TUTase

would give access to monoclonal antibodies, which in turn

should provide a valuable tool to study the functional

significance of this U6 snRNA modification Such

func-tional studies would provide clues to why evolution allowed

this small stable U6 snRNA the unique luxury of acquiring

its own modifying enzyme

Acknowledgements

We thank Dr Andre´ Frontzek for skilful introduction into the RNA

electrophoretic mobility-shift analysis technique, and Nadine Pieda for

expert technical assistance Thanks are also due to Klaus Grabert for

the photographs This work was supported by a grant from the

Deutsche Forschungsgemeinschaft (Be 531/19-1).

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