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In this report we have determined the phosphorylation pattern of human as1-casein by a combi-nation of matrix-assisted laser desorption mass spectrometry and amino acid sequence analysis

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The phosphorylation pattern of human as1-casein is markedly different from the ruminant species

Esben S Sørensen, Lise Møller, Maria Vinther, Torben E Petersen and Lone K Rasmussen*

Protein Chemistry Laboratory, Department of Molecular Biology, University of Aarhus, Denmark

Caseins are highly phosphorylated milk proteins assembled

in large colloidal structures termed micelles In the milk of

ruminants, as1-casein has been shown to be extensively

phosphorylated In this report we have determined the

phosphorylation pattern of human as1-casein by a

combi-nation of matrix-assisted laser desorption mass spectrometry

and amino acid sequence analysis Three phosphorylation

variants were identified A nonphosphorylated form, a

variant phosphorylated at Ser18 and a variant

phosphory-lated at Ser18 and Ser26 Both phosphorylation sites are located in the amino acid recognition sequence of the mammary gland casein kinase Notably, no phosphoryla-tions were observed in the conserved region covering resi-dues Ser70–Glu78, which is extensively phosphorylated in the ruminant as1-caseins

Keywords: as1-casein; human milk; mammary gland casein kinase; phosphorylation

Caseins are the predominant milk proteins of most

mammalian species [1] In ruminants, about 75% of the

milk protein content is constituted of caseins The

corresponding figure for human milk is only about 40%

[2] In the milk of ruminants, caseins interact with calcium

phosphate forming large stable colloidal particles termed

micelles These micellar complexes make it possible to

maintain a supersaturated calcium phosphate

concentra-tion in milk, providing the newborn with sufficient

calcium phosphate for the mineralization of the rapidly

growing calcified tissues In this context, the

phosphory-lation of the individual caseins plays a significant role in

the interaction with calcium phosphate and thereby the

organization of the micelles The ruminant caseins, which

are the most intensely studied, comprise as1-, as2-, b- and

j-casein Their phosphorylation pattern has been the basis of

many studies and the general feature is that they are highly

phosphorylated proteins, phosphorylated by the mammary

gland casein kinase [3–7] The primary requirement for

phosphorylation by this kinase is a glutamate, a

phospho-serine or an aspartate two residues to the C-terminal side of

the phosphoacceptor site (S-x-E/Sp/D) [8,9]

Compared with ruminants, human milk contains a very

low concentration of calcium phosphate and the function

of casein in delivering calcium to the neonate is therefore

muted in this species In human milk, the predominant

caseins are j- and b-casein, which differ from its ruminant

counterpart by a lower degree of phosphorylation [10] For many years it was generally accepted that as1-casein was absent or present in only very small amounts in human milk [2] In the mid-1990s, two groups isolated and sequenced a minor 27-kDa casein component that was identified as being the human counterpart of as1-casein [11,12] In addition, it was shown that this as1-casein component forms disulfide-bonded heteromultimers with j-casein in human milk [12] The molecular cloning and sequencing of mRNA transcripts revealed the presence of three forms of as1-casein in human milk [13,14] In the present study, we report the phosphorylation pattern of human as1-casein

Materials and methods Materials

Trypsin (EC 3.4.21.4) was obtained from Worthington Biochemical Corporation (Freehold, NJ, USA) Vydac C4 and C18 reverse-phase resins were from The Separations Group (Hesperia, CA, USA) and the RP C2/C18column was from Amersham Biosciences AB (Uppsala, Sweden) Reagents used for sequencing were from Applied Biosys-tems (Foster City, CA, USA) All other reagents were of analytical reagent grade

Purification of human as1-casein Human as1-casein was purified from human milk as described [12] During this procedure, the protein was reduced and alkylated to dissociate the disulfide-linked complex consisting of as1- and j-casein To remove small residual amounts of j-casein, the protein was subjected to reverse-phase chromatography on a Vydac C4reverse-phase column The purity of the resulting as1-casein was verified

by SDS/PAGE and N-terminal amino acid sequence analysis

Correspondence to E S Sørensen, Protein Chemistry Laboratory,

Department of Molecular Biology, University of Aarhus, Science

Park, Gustav Wieds Vej 10, DK-8000 Aarhus, Denmark.

Fax: + 45 8 6136597, Tel.: + 45 8 9425092,

E-mail: ess@imsb.au.dk

Enzyme: Trypsin (EC 3.4.21.4)

*Present address: Symphogen A/S, DK-2800 Denmark.

(Received 5 May 2003, revised 14 July 2003,

accepted 16 July 2003)

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Generation, separation and characterization of peptides

Approximately 300 lg of reduced and alkylated human

as1-casein was digested with trypsin using a ratio of enzyme

to substrate of 1 : 100 (w/w) in 0.1M ammonium

bicar-bonate, pH 8.1, at 37C for 6 h Separation of the peptides

was carried out by reverse-phase HPLC on a Vydac C18

column and detected in the effluent by measuring the

absorbency at 226 nm (as described in the legend to Fig 1)

Fraction 35 (Fig 1) was rechromatographed by

reverse-phase HPLC on a SMART-system equipped with a

2.1· 100 mm C2/C18 RPC column using a gradient of

acetonitrile in 0.05% heptafluorobutyric acid at 25C

Peptides were characterized by mass spectrometric- and

amino acid sequence analysis Mass spectrometric analyses

of the peptides were performed using a MALDI-TOF mass

spectrometer (Voyager DE PRO, Applied Biosystems Inc.)

Theoretical peptide masses were calculated using the

GPMAW program (Lighthouse Data, Odense, Denmark)

Amino acid sequence analysis was performed on an

automated amino acid sequencer (ABI 477A/120A; Applied

Biosystems Inc.) To locate phosphoserines in the sequence,

phosphopeptides were treated with ethanethiol to convert

phosphoserine into S-ethylcysteine [15] which can be

identified by amino acid sequence analysis as

PTH-S-ethylcysteine after its release in the corresponding cycle

PTH-S-ethylcysteine eluted just before the diphenylthiourea

peak in the system used [16]

Results and discussion

Human as1-casein was purified in a reduced and

carboxy-methylated state as described [12] The protein was digested

with trypsin and the resulting peptides were separated

by reverse-phase chromatography (Fig 1) Fractions were

collected and the peptides were characterized by mass

spectrometric- and sequence analysis The combined results

are shown in Table 1 The amino acid sequence of human

as1-casein is shown in Fig 2 Peptides identified by mass spectrometric analysis and/or sequence analysis are under-lined As seen in the Fig 2, peptides covering the entire

Fig 1 Reversed-phase separation of a trypsin digest of human a s1 -casein Human a s1 -casein was digested with trypsin as described in Materials and methods Peptides were eluted with a gradient of 80% acetonitrile in 0.1% trifluoroacetic acid (dotted line) on a Vydac

C 18 (10 lm) column (4 · 250 mm) The col-umn was operated at 40 EC and the flow rate was 0.85 mLÆmin)1 Peptides were detected in the effluent by recording the absorbance at

226 nm (solid line), collected manually and characterized as described in the text.

Table 1 Characterization of peptides from the tryptic digest of human

a s1 -casein Peak numbers designations correspond to those of Fig 1 The amino acid sequence was identified by sequence analysis and/or MALDI-TOF MS Calculated MH+, calculated protonated mono-isotopic masses; observed MH + , molecular monoisotopic protonated mass determined by MALDI-TOF MS.

Peak number Sequence Calculated MH+ Observed MH+

19 45–53 1105.55 1105.5

25 54–67 1605.71 1605.77

26 28–36 1143.46 1143.38

35–1 a 12–27+2P 1942.82 1942.78 35–2a 68–83 1718.75 1718.75

37 12–27+1P 1862.86 1862.78

38 12–27 1782.89 1782.86

41 12–36+2P 3067.34 3067.26

43 12–36+1P 2987.34 2987.26

44 91–109 2267.16 2267.15

49 164–171 904.44 904.38

53 142–163 2580.21 2580.44

54 111–132 2591.24 2591.18

54 133–141 1170.57 1170.5

62 133–163 3731.75 3731.12

65 111–163 6303.97 6303.61

a

Peaks from rechromatography of fraction 35 from Fig 1.

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sequence of human as1-casein have been identified and

characterized in this study

Glycosylation

Three asparagine residues in human as1-casein (Asn14,

Asn54, Asn154) are located in the putative glycosylation

sequence Asn-X-Ser/Thr In the case of Asn14 and Asn154,

a neighbouring proline residue in position X corrupts the

glycosylation sequence and renders it unfit for glycosylation

Regarding Asn54, this study did not show any evidence for

glycosylation of this residue in human as1-casein Mass

spectrometric analysis of peak 25 (Fig 1) containing the

peptide Asn54–Lys67 showed a mass of 1605.77 Da which

corresponds to the calculated protonated monoisotopic

mass (1605.71 Da) of the unmodified peptide sequence,

thereby showing that Asn54 was not glycosylated in human

as1-casein

Likewise in this study, we observed no O-glycosylations

in human as1-casein

Phosphorylation

Human as1-casein contains 16 serines and four threonines,

where nine of the serines and one threonine are located

in the recognition sequence of the mammary gland casein

kinase [8,9] The recognition sequence (Ser/Thr-X-Glu/

Ser(P)/Asp), comprises an acidic residue, glutamic acid,

aspartic acid or a phosphorylated residue, as the second

amino acid to the C-terminal side of the serine or threonine

to be targeted Especially interesting, human as1-casein

contains a serine rich region, SSISSSSEE(70–78), where five

of the serines are located in the recognition sequence of

the mammary gland casein kinase This region is highly

conserved among all species with known as1-casein

sequences (for alignment see [13]), and in all analyzed

species a high degree of phosphorylation has been observed

in this region [3–5]

Our laboratory has much experience of employing MALDI-TOF mass spectrometric analysis for identification and localization of phosphorylation sites in proteins [16–18] MALDI-TOF mass spectrometric analysis of a phospho-peptide results in a spectrum with an easily identifiable fragmentation pattern which is characteristic for phospho-rylated serines These spectra contain a series of peaks separated by approximately 98 Da, which represents the fragmentation of a phosphoserine to dehydroalanine

In this work, we have analyzed all fractions from the reverse-phase HPLC separation of the tryptic digest of human as1-casein (Fig 1) by MALDI-TOF mass spectro-metric and N-terminal sequence analysis We identified four fractions with the characteristic fragmentation pattern of peaks at 35, 37, 41 and 43 A representative MALDI-TOF spectrum of peak 41 showing the characteristic fragmenta-tion of a phosphopeptide is shown in Fig 3 Peak 35 was found to contain two peptides which potentially could be phosphorylated, thus the fraction was rechromatographed

by reversed-phase HPLC on a SMART HPLC system to separate the two components, 35–1 and 35–2 Peak 35–2 gave a mass spectrum with only one mass at 1718.75 Da which is identical with the calculated protonated mass for the tryptic peptide covering residues 68–83, thereby showing that this peptide is not phosphorylated in human as1-casein This observation was confirmed by Edman sequencing of the peptide, which showed normal yields of PTH-serine in all relevant cycles Mass analysis of peak 35–1 showed a mass of 1942.78 Da, as well as two populations of ions at approximately )98 Da and )196 Da This triplet of ions, each separated by approximately 98 Da, indicates that peak 35–1 contains a phosphopeptide with two phosphoserines Furthermore, the observed mass at 1942.78 Da correlates with the calculated protonated mass (1942.82 Da) for the tryptic peptide covering residues 12–27 and containing two phosphorylations (159.93 Da) Mass analysis of peak 37 showed a mass of 1862.78 Da, and a single fragmentation ion at)98 Da was observed, indicating the presence of a single phosphorylation in the peptide Furthermore, the

Fig 2 Localization of phosphorylations in human a s1 -casein The

amino acid sequence was deduced from the cDNA sequence [11] Solid

lines indicate isolated and characterized peptides (Table 1) P denotes

identified phosphorylation Peptides are numbered according to the

reversed-phase elution profile in Fig 1.

Fig 3 MALDI-TOF MS of peak 41 from Fig 1 The protonated mass at m/z 3067.26 corresponds to the peptide 12–36 including to phosphorylations The characteristic fragmentation pattern confirms the presence of two phosphorylations in the peptide.

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mass 1862.78 Da correlates with the calculated protonated

mass of residues 12–27 containing a single phosphorylation

(1862.86 Da) Finally, mass analysis of peak 38 showed a

mass which correlates with the mass of the peptide covering

residues 12–27 without any modifications In conclusion, we

have observed the peptide 12–27 in three different forms,

with zero, and one and two phosphate groups attached

Peaks 41 and 43 represent peptide 12–36 with two and one

phosphorylated groups, respectively These peptides,

result-ing from incomplete cleavage at Arg27, do not contain any

additional serines or threonines compared with peaks 35

and 37, and thus they were not characterized further

The peptide, LQNPSESSEPIPLESR(12–27) (peak

35–1), contains three serines located in the recognition

sequence of the mammary gland casein kinase (Ser16, Ser18

and Ser26) To determine which of the serines are in fact

phosphorylated, we subjected the two peptides to an

ethanethiol treatment followed by Edman sequencing as

outlined in Materials and methods The ethanethiol

treat-ment converts the labile phosphoserine residues into

S-ethylcysteine, which is more stable and able to withstand

the relatively harsh Edman chemistry during automated

sequencing [15] Furthermore, PTH-S-ethylcysteine elutes in

an open window just before the diphenylthiourea peak in

the on-line HPLC system used in these studies Sequence

analysis of peptide 35–1 succeeding the ethanethiol

treat-ment revealed PTH-S-ethylcysteine in cycles 7 and 15,

corresponding to Ser18 and Ser26 in human as1-casein

Likewise, sequence analysis of peptide 37, after the

ethanethiol treatment gave PTH-S-ethylcysteine in sequence

cycle 7, corresponding to Ser18 in human as1-casein The

yields of PTH-serine in cycles corresponding to Ser16 and

Ser19 were as expected, indicating that these residues were

not phosphorylated

As a control experiment (data not shown) human and

bovine b-casein were purified, tryptic digests were generated

and these were separated by reversed-phase HPLC using the

system and column described in Materials and methods In

MALDI-TOF MS analyses of the human b-casein digest,

two peptides with protonated masses of 2407.85 and

2327.80 were identified These masses correspond to the

expected masses of the peptide 1–18 of human b-casein with

four phosphorylations (2408.00) and three

phosphoryla-tions (2328.00), respectively Likewise in the digest of bovine

b-casein, a peptide with a protonated mass of 3122.27 was

observed, corresponding to the peptide 1–25 of bovine

b-casein with four phosphorylations (3122.40 Da) These

results indicate that the protocol used for identification of

phosphorylation sites is capable of handling highly

phos-phorylated peptides Furthermore, the methods used in the

present study have previously been used for identification

of phosphorylation sites in several milk proteins in our

laboratory, most prominently the 28 phosphorylation sites

in bovine milk osteopontin [16] Therefore it is not likely

that the lack of identification of a highly phosphorylated

peptide in as1-casein is due to limitations of the techniques

used

Finally, MALDI-TOF MS analysis of native human

as1-casein showed ions corresponding to a mass of

approxi-mately 20 232 Da, which correlates well with the calculated

mass of human as1-casein including two phosphate groups

(20246 Da) (Fig 4)

In conclusion, these studies show that human as1-casein exists in three phosphorylation variants A nonphosphory-lated form, a variant containing a single phosphorylation

at Ser18 and a variant phosphorylated at Ser18 and Ser26

It is difficult to determine the quantitative relation between the three phosphorylation variants, but judged by the reversed-phase HPLC trace in Fig 1, the variant containing a single phosphorylation at Ser18 is the major variant ( 50%), followed by the nonphosphorylated form ( 30%) and the doubly phosphorylated variant ( 20%) The degree of identity between human and other known

as1-casein sequences is overall low (alignment of sequences is shown in [6,13]) The phosphorylations have been charac-terized in ovine (Ovis aries), caprine (Capra hircus), bovine (Bos taurus), water buffalo (Bubalus bubalis) and camel (Camelus dromedarius) as1-casein Generally, all of these species have been reported to have a significant higher number of phosphorylations than shown to be the case for human as1-casein in this study Bovine as1-casein is phos-phorylated at up to nine positions depending on the genetic variants [3], the ovine as1-casein is phosphorylated at up to

11 positions [5], the water buffalo as1-casein is phospho-rylated at 6–8 positions [6], the caprine counterpart is phosphorylated at 9–10 positions [4], and camel as1-casein is phosphorylated at up to six serines [7] The most striking difference in the phosphorylation pattern between human

as1-casein and its ruminant counterparts is the shortage of phosphorylation in the serine-rich region consisting of residues Ser70–Glu78 in the human sequence This region has been shown to be highly phosphorylated in all the above mentioned species In this study, we have isolated the tryptic peptide covering residues Met68–Lys83, which contains the serine-rich region, in a nonphosphorylated form, and no traces of a phosphorylated form of this peptide were observed

The lack of phosphorylation at other positions reported

to be modified in ovine, caprine and bovine as1-casein (serines 41, 46, 48, 75 and 115 in all species, and Ser12 in ovine as1-casein, all numbers referring to the ruminant sequences), can simply be explained by sequence substitu-tions at these posisubstitu-tions, leaving no hydroxyamino acids to

be phosphorylated at these positions in human as1-casein The region containing the phosphorylated residues, Ser18

Fig 4 MALDI-TOF MS of intact human a s1 -casein The peak at m/z

20233 corresponds well to the calculated mass of human a s1 -casein including two phosphate groups (20246 Da) The peak at m/z 10121 represents the doubly protonated species (M2H+).

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and Ser26, in human as1-casein is not especially well

conserved among the other analyzed species The sequence

containing Ser18 in the human as1-casein is part of exon

3 in the human as1-casein gene, which is not present in the

ruminant species [13] Ser26, situated in exon 5 of the human

as1-casein gene, is not conserved in any other species except

the wallaby, in which the phosphorylation pattern has not

been determined [19]

During the review of these results, we were puzzled by the

lack of phosphorylation in the conserved region Ser70–

Glu78, which is so extensively phosphorylated in the

ruminant species To test whether our results were

repre-sentative, milk from three different women was analyzed

as1-Casein was purified and the reversed-phase traces of

tryptic digests of the protein were compared and found to be

identical in all cases, thereby showing similar

phosphoryla-tion of the protein in different individuals The

phosphory-lation pattern of human as1-casein described here, and

especially the lack of phosphorylations in the region Ser70–

Glu78, is therefore unlikely to be a result of intra-species

post-translational polymorphism in the protein However, it

should be emphasized that it is more difficult to show the

absence of a modification convincingly than its presence;

hence the existence of minor species partially

phosphory-lated at the region discussed can not be entirely excluded

The deletion of 11 amino acids at positions 59–69 and of

37 amino acids at positions 59–95 in caprine as1-casein leads

to the variants D and F In both cases these deletions, which

start at the same position of the polypeptide chain, include

the major phosphorylation site of the protein [20] In

ruminant milk, as1-casein, as well as the other three caseins

as2-, b- and j-casein is present in micellar structures

responsible for the calcium transport to the neonates

Compared with ruminant milk, the milk of primates holds a

much lower concentration of calcium and a function of as1

-casein in calcium transport in human milk is not likely

Recent studies of caprine as1-casein suggest that the protein

interacts with the other caseins in the rough endoplasmic

reticulum and that the formation of this complex is required

for their efficient export to the Golgi apparatus [21]

Whether a similar scenario exists in the human system

remains to be elucidated

Acknowledgments

Special thanks to H Breinholt and K.-E Højbjerg, Department of

Obstetrics and Gynaecology, University Hospital of Aarhus, for

providing the individual milk samples.

References

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