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Indeed, most neurotransmitters thus far identified are amidated peptides in cnidarians Keywords peptidylglycine a-hydroxylating monooxygenase; cytochrome b561; planarian; neuroendocrine v

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a neuropeptide processing enzyme, colocalizes with

Akikazu Asada1, Hidefumi Orii1, Kenji Watanabe1and Motonari Tsubaki1,2,3

1 Department of Life Science, Graduate School of Life Science, University of Hyogo (formerly Himeji Institute of Technology), Hyogo, Japan

2 CREST, Japan Science and Technology Agency (JST), Saitama, Japan

3 Department of Molecular Science and Material Engineering, Graduate School of Science and Technology, Kobe University, Hyogo, Japan

Neuropeptides in the brain, in the nervous system, and

in various endocrine cells are synthesized in the rough

endoplasmic reticulum as large precursor proteins After

transit to vesicles and during axonal transportation

along axons, several processing enzymes residing in the

vesicles process the peptides to convert them to mature

forms C-terminal a-amidation of the peptides occurs in

the late stage [1] and is probably a rate-limiting step in

many instances [2] Over half of peptide hormones or neuropeptides are amidated in vertebrates; in insects, greater than 90% of such peptides show the presence of

a C-terminal amide moiety [3] This C-terminal amide is very important in their functions, as its absence often disrupts the activity or receptor-binding properties of the peptide ligands [4] Indeed, most neurotransmitters thus far identified are amidated peptides in cnidarians

Keywords

peptidylglycine a-hydroxylating

monooxygenase; cytochrome b561;

planarian; neuroendocrine vesicle;

neuropeptide amidation

Correspondence

M Tsubaki, Department of Molecular

Science and Material Engineering, Graduate

School of Science and Technology, Kobe

University, Rokkodai-cho, Nada-ku, Kobe,

Hyogo 657-8501, Japan

Fax: +81 78 803 6582

Tel: +81 78 803 6582

E-mail: mtsubaki@kobe-u.ac.jp

Note

The nucleotide sequence of planarian PHM

in this article has been submitted to the

DDBJ ⁄ EMBL ⁄ GenBank databases with

accession number AB195502.

(Received 27 September 2004, revised 2

December 2004, accepted 14 December

2004)

doi:10.1111/j.1742-4658.2004.04528.x

Planarians are one of the simplest animal groups with a central nervous system Their primitive central nervous system produces large quantities

of a variety of neuropeptides, of which many are amidated at their

C terminus In vertebrates, peptide amidation is catalyzed by two enzymes [peptidylglycine a-hydroxylating monooxygenase (PHM) and peptidyl-a-hydroxylglycine a-amidating lyase] acting sequentially In mammals, both enzymatic activities are contained within a single protein that is encoded

by a single gene By utilizing PCR with degenerate oligonucleotides derived from conserved regions of PHM, we succeeded in cloning a full-length cDNA encoding planarian PHM The deduced amino acid sequence showed full conservation of five His residues and one Met residue, which bind two Cu atoms that are essential for the activity of PHM Northern blot analysis confirmed the expression of a PHM mRNA of the expected size Distribution of the mRNA was analyzed by in situ hybridization, showing specific expression in neurons with two morphologically distinct structures, a pair of the ventral nerve cords and the brain The distribution

of PHM was very similar to that of cytochrome b561 This indicates that the ascorbate-related electron transfer system operates in the planarian cen-tral nervous system to support the PHM activity and that it predates the emergence of Plathelminthes in the evolutionary history

Abbreviations

AsA, ascorbic acid; CNS, central nervous system; DBH, dopamine b-hydroxylase; DBHL, dopamine b-hydroxylase-like protein; PAL, peptidyl-a-hydroxylglycine a-amidating lyase; PAM, peptidylglycine a-amidating monooxygenase; PHM, peptidylglycine a-hydroxylating

monooxygenase; MDA, monodehydroascorbic acid; TBH, tyramine b-hydroxylase; VNC, ventral nerve cords.

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(coral, jellyfish, sea anemones), which possess the most

primitive form of nervous system, suggesting that the

archetype nervous systems may have relied on amidated

peptides [5] The C-terminal a-amidation of

neuro-peptides is catalyzed by two enzymes: peptidylglycine

a-hydroxylating monooxygenase (PHM; EC 1.14.17.3)

and peptidyl-a-hydroxyglycine a-amidating lyase (PAL;

EC 4.3.2.5) consecutively [4,6,7] In vertebrates, PHM

and PAL are expressed together within one polypeptide

chain and denoted as peptidylglycine a-amidating

mono-oxygenase (PAM) [8–10] In some animals, such as

Drosophila[11] and Calliactis [12], PHM is encoded by a

distinct gene In the mollusc, Lymnaea, a multifunctional

PAM containing four different copies of PHM and a

single PAL has been found [13] Endogenously, this

zymogen was converted to yield a mixture of

mono-functional isoenzymes However, in the nematoda,

Caenorhabditis elegans, both types of enzyme are

pre-sent, based on analyses carried out using the SMART

web-based resource (http://smart.embl-heidelberg.de/)

[14]

Planarians – nonparasitic free-living flatworms of

the phylum Plathelminthes, class Turbellaria – are

phylogenetically very distant from vertebrates but have

acquired a central nervous system (CNS), mesodermal

tissues, and a bilateral structure during evolution

[15] Planarians use neuropeptides [16,17] as major

neurotransmitters Planarian neuropeptides (substance

P-, FMRFamide-related peptides) are extensively

a-amidated [16–19], as observed in other invertebrates

Therefore, it is of interest to examine which form

(PAM, PHM, or a different form) does exist in

planar-ians to characterize their primitive CNS as the

puta-tive ancestor of the sophisticated CNS of higher

animals

The monooxygenase reaction catalyzed by PHM (or

the PHM domain) requires copper, ascorbate, and

molecular oxygen, as found for its close family

mem-ber, dopamine b-hydroxylase (DBH) [2] Ascorbic acid

(AsA), which is accumulated in a relatively

concentra-ted manner inside the neuroendocrine secretory vesicles

[20], is converted to monodehydroascorbic acid

(MDA) radical after supplying the electron equivalent

to the catalytic copper center of the enzyme As there

is no transmembrane transport of AsA or MDA

rad-ical in the vesicles [21], it has been postulated that

a common transmembrane electron carrier,

cyto-chrome b561, conveys a reducing equivalent from the

extravesicular AsA to the intravesicular MDA radical

[21–27] Cytochrome b561, first observed in chromaffin

vesicles of bovine adrenal medullae [28,29] and later

found to be distributed in many neuroendocrine tissues

[30–33], is structurally and functionally very different

from other cytochromes [34] It spans the vesicle mem-brane [35] and has a favorable midpoint potential for interacting with AsA and the MDA radical [36–39] Very recently, we have succeeded in cloning the planarian cytochrome b561 cDNA [40] The cyto-chrome b561 gene product was specifically expressed in the planarian nervous system [40], suggesting that the AsA-related electron transfer system, which supports amidation of the neuropeptide, might predate or coin-cide with the emergence of the CNS in animals Thus,

as the second objective of our present study, we exam-ined the colocalization of PHM with cytochrome b561

in the planarian

In the present study, we successfully accomplished the two objectives raised above, namely we obtained

an entire planarian PHM cDNA and identified its spe-cific expression in the planarian CNS as being colocal-ized with cytochrome b561

Results

Cloning of planarian PHM

By using degenerate oligonucleotide primers (PAM-S4⁄ PAM-A3) (Fig 1) coding for the conserved region

of PHMs of various animals, we cloned a 265 bp PCR product from a cDNA library of the head portion of the planarian The deduced amino acid sequence of the PCR product (67 amino acid residues, excluding the primer regions) was highly homologous (containing 10 fully conserved amino acid residues) to other PHMs and therefore consistent with a partial sequence of the planarian PHM cDNA To obtain a longer nucleotide sequence, we carried out nested PCR on the first-strand planarian head cDNA by using new specific primers (spPAM-S2 and spPAM-S3) and SK20 vector primer and a universal primer A PCR product ( 560 bp) was cloned and sequenced The clone was found to encode the 3¢ end of the PHM cDNA, inclu-ding a stop codon (TAG), the 3¢ untranslated region (3¢-UTR), and the poly(A+) tail Stepwise dilution screening of the planarian head cDNA library by PCR using two specific primers (spPAM-S3 and spPAM-A3; PCR probe size, 144 bp) was then conducted One plaque was screened, in vivo excised, recloned, and sequenced completely This clone ( 1300 bp) was found to encode a major part of the planarian PHM gene, except for the NH2-terminal region Several cycles of PCR cloning, using specific primers (PPHM-A4, PPHM-A3, and PPHM-A5) and vector primers, followed by sequencing, gradually narrowed the uncov-ered 5¢ end region Finally, the most 5¢ end PCR prod-uct was obtained, cloned, and sequenced to identify an

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in-frame stop codon (TAG) at the most 5¢ end and the

putative initiation codon (ATG) followed by an open

reading frame To obtain the entire planarian PHM

cDNA, stepwise dilution screening of the planarian

head cDNA library with PCR was again conducted by

using two specific primers (PPHM-S4 and PPHM-A3)

One plaque, containing the entire planarian PHM

cDNA gene, was obtained, completely sequenced and

analyzed The strategy used to obtain the planarian

PHM cDNA sequence is summarized in Fig 1

The whole planarian PHM cDNA (1384 bp)

con-tained a single open reading frame (1149 bp), which

was preceded by the 5¢-UTR of 87 bp and followed by

the 3¢-UTR of 127 bp and the first 21 nucleotides of

the poly(A+) tail (Fig 2) This indicated clearly that

planarian PHM is coded in a gene separate from that

of PAL

Northern blot

Northern blot analysis of total RNA from the asexual

planarian was carried out The cDNA probe coding

for a major region of planarian PHM hybridized with

an mRNA giving a single band of  1.4 kb (Fig 3),

being consistent with the expected size based on the

cloned cDNA This result also confirmed that there is

no PAM-like gene (i.e a fused gene with PHM and

PAL) in the planarian that will generate an mRNA of

 4 kb, as found for vertebrate PAMs

Deduced amino acid sequence of planarian PHM

The deduced amino acid sequence of planarian PHM

predicted a protein with a length of 382 residues

(Fig 2) and a theoretical molecular weight of 43701.34

A hydrophobic segment (13 amino acid residues) at the

N terminus satisfied the consensus rules for the signal sequences, with a cleavage site most likely to occur after amino acid 15, based on analyses conducted by using SignalP WWW and PSORT WWW servers An adjacent propeptide, present in vertebrate PAMs (inclu-ding human, rat, bovine and Xenopus) was absent, as observed for molluscan PHM [13]

The deduced planarian PHM amino acid sequence was compared with those of PHMs and PHM domains

of PAMs from several representative species (including Schistosoma, Drosophila, Calliactis, C elegans, rat, bovine and human) by a multiple sequence alignment (Fig 4) Five histidine and one methionine residue in the central portion of PHM were considered to bind two Cu atoms (CuA and CuB, also termed CuH and

CuM, respectively), which are essential for the catalytic reaction of PHM These six residues (indicated by #) were also conserved in planarian PHM (Fig 4) Fur-thermore, it is known that well-conserved Cys residues form putative disulfide bonds to fix the tertiary struc-ture of the PHM domain Eight of the 10 cysteine resi-dues were conserved in planarian-PHM, as found for other PHM sequences (except for C elegans-PAM) and are marked with + in Fig 4 Additionally, corres-ponding amino acid residues to the residues shown to interact with bound peptide–substrate, such as Arg240, Asn316 and Tyr318 in rat PHM [7,41] were also con-served in planarian PHM (marked with ! in Fig 4) Percentage amino acid identities between these repre-sentative PHMs and PAMs were calculated for their PHM core regions and then tabulated (Table 1) The planarian PHM sequence showed around 40% amino

Fig 1 Schematic representation of the cDNA coding for planarian peptidylglycine a-hydroxylating monooxygenase (PHM) and positions of degenerate and specific primers used in the present study The broad white bar and the two flanking small white bars represent the coding region and the noncoding regions of the planarian PHM cDNA, respectively; whereas the two flanking broad lines outside indicate the k ZAP

II vector The two gray boxes in the planarian PHM cDNA represent the conserved sequences [(P ⁄ V)FAFR(T ⁄ V)H(T ⁄ A)H for positions 231–

239 of human peptidylglycine a-amidating monooxygenase (PAM) and EMCN(F ⁄ L)Y(I ⁄ M ⁄ L) for positions 308–315 of human-PAM, respect-ively], for which two degenerate primers (PAM-S4 and PAM-A3) were designed The degenerate and specific primers used were indicated

by horizontal arrows above the planarian PHM cDNA The PCR clones and partial cDNA clones obtained were indicated below the planarian PHM cDNA in the order of nucleotide sequence determination from top to bottom Arrows indicate the direction of nucleotide sequence determination PO8 indicates the M13 reverse primer.

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acid identity with all the sequences listed in Table 1 The closest similarity was found to Schistosomas PHM (46.0%) and the most distant one was C elegans PAM (33.6%) To further understand the evolutionary rela-tionship, we conducted a detailed phylogenetic analysis

of planarian PHM in comparison with other PHMs

or PHM domains and DBHs [including tyramine b-hydroxylases (TBHs) and DBH-like proteins (DBHLs)] from various animals (28 sequences in total) The analysis showed that planarian PHM was very distant from DBH, TBH and DBHL (Fig 5) Pla-narian PHM was classified as a member of a subfamily consisting of PHMs from Drosophila, Heterodera,

C elegans and Schistosoma and was distinct from PHM domains of vertebrate PAMs and cnidarian PHMs (Fig 5)

Expression of PHM along the planarian CNS The planarian CNS is basically constituted by an anterior ‘brain’ and a pair of longitudinal ventral nerve cords (VNCs) that run through the length of the body The expression of the planarian PHM gene, as shown

by in situ hybridization, was specific in these neurons (Fig 6A,B) The planarian cytochrome b561 gene was also expressed to a similar extent in the brain and in a pair of VNCs (Fig 6C,D), in accordance with our pre-vious observation [40] The pattern of gene expression

Fig 2 Nucleotide and deduced amino acid sequences of planarian peptidylglycine a-hydroxylating monooxygenase (PHM) The nucleotides are numbered from the 5¢ to the 3¢ end, and the amino acid residues are numbered starting with the first ATG in the open reading frame The translation termination codon is indicated by an asterisk The sequence obtained by the first degenerate PCR is singly underlined, whereas the sequences corresponding to the two specific primers PPHM-S1 and PPHM-A1, used for production of the PCR probe for the Northern blot analysis, are underlined by broken lines.

28S

18S

Fig 3 Northern blot analysis of peptidylglycine a-hydroxylating

monooxygenase (PHM) mRNA in the planarian A PCR probe

pro-duced by two specific primers – PPHM-S1 and PPHM-A1 – was

used for Northern blot analysis Total RNA (10 lg) derived from the

whole planarian body was probed with the planarian PHM cDNA.

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Fig 4 Multiple alignment of peptidylglycine a-hydroxylating monooxygenase (PHM) or PHM-domain sequences of planarian, Schistosoma, Drosophila, Calliactis, Caenor-habditis elegans, rat, bovine and human ori-gin Amino acid residues common to all sequences are denoted by asterisks below the sequences, whereas conservative resi-dues are indicated by colons (:) or a full stop (.) The five His and one Met residue that form complexes with two Cu 2+ atoms are indicated by # in the uppermost line, whereas eight conserved Cys residues that form four putative Cys-Cys disulfide bridges and three conservative residues that might interact with the bound peptide substrate are marked with + and !, respectively The sequences were obtained from S mansoni (Schistosoma-PHM; AY172995), Drosophila (Drosophila-PHM; AY069103), Calliactis (Cal-liactis-PHM; AF036337), C elegans (C.ele-PHM; AC025726 and C.ele-PAM; U80438), rat (Rat-PAM; X59687 and M82845), bovine (Bovine-PAM; M18683), and human (Human-PAM; S75038) origin PAM, pept-idylglycine a-amidating monooxygenase.

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for these two proteins showed close similarities to

those of neuron-specific proteins, including a

synapto-tagmin homologue, DjSYT [42], a homologue of PC2

(prohormone convertase 2) [15], a synapsin

homo-logue, a carboxypeptidase E homologue [43] and a

homologue of the nicotinic acetylcholine receptor a7-1

subunit [44] All of these proteins are related to

neuro-transmission, including neurotransmitters, receptors⁄

channels and synaptic vesicles [44]

Expression of PHM in the planarian brain

The closer view of the head region by in situ

hybridiza-tion using the planarian PHM antisense probe showed

that numerous cells, which are considered to be

neu-rons, were clustered around the inverted U-shaped

axon bundles and that these cells were specifically

stained (Fig 7A; red) No appreciable segmental

ture was observed inside the inverted U-shaped

struc-ture, where the neuropil-containing axons and

dendrites locate (Fig 7A) Higher magnification views

showed that indeed only the neuronal cell bodies were

stained with the PHM probe (Fig 7D; brown)

Immu-nohistochemical staining of the head region, by using

antiserum against planarian cytochrome b561, showed

that both the inverted U-shaped axon bundles and the

surrounding neurons were stained (Fig 7B,C; red) A

higher magnification view (Fig 7E), and its merged

image with that stained for nuclei (Fig 7F; blue),

con-firmed this observation

Expression of PHM in the planarian eyes

The planarian eye is composed of two types of cells,

namely photoreceptor cells and pigmented cells [45]

The pigment cells form an eye cup and the

photorecep-tor cells project their microvilli into the inside of the cup, as indicated by immunohistochemical staining with antiplanarian arrestin (green) (Fig 7H,J) Planarian PHM was expressed only in the photoreceptor cells adjacent to the eye cup In particular, signals of the PHM were observable in the region surrounding the nuclei (brown in Fig 7G) The staining image with anti-planarian cytochrome b561was, however, slightly differ-ent from that of planarian arrestin; only the outside of the eye cup was stained (Fig 7J), suggesting that the expression level of cytochrome b561in the microvilli is not so significant

Discussion

This report describes the successful molecular cloning

of planarian PHM cDNA In mammals, PHM and PHL are fused as a bifunctional protein – PAM – that

is encoded by a single gene In some invertebrates, such as Drosophila [11] and Calliactis [12], PHM is encoded by a distinct gene In the mollusc, Lymnaea, a multifunctional PAM was found, which comprised four different copies of PHM and a single copy of PAL [13] Very recently it was reported that a human parasite S mansoni, which belongs to the same phylum (Plathelminthes) as planarian, but a different class (Trematoda), possessed a monofunctional PHM lack-ing a PAL domain [46] In the nematoda C elegans, both types of enzyme precursors, a monofunctional PHM and a bifunctional PAM, are present, based on analysis conducted using the SMART web-based resource (http://smart.embl-heidelberg.de/) (Figs 4 and 5) In our present study, the cDNA probe coding for the major region of planarian PHM hybridized with

an mRNA, giving a single band of  1.4 kb (Fig 2), being consistent with the expected size based on the

Table 1 Percentage identity of amino acid residues between peptidylglycine a-hydroxylating monooxygenases (PHMs) and PHM domains of peptidylglycine a-amidating monooxygenase (PAMs) from various animal species (including Schistosoma-PHM, Drosophila-PHM, Calliactis-PHM, C elegans-Calliactis-PHM, C elegans-PAM, rat-PAM, bovine-PAM, and human-PAM) were calculated by using the FASTA program of the DDBJ server (DNA Data Bank of Japan, Mishima, Japan) for the corresponding PHM core regions The PHM core regions were identical to those used in Fig 5.

Planaria-PHM

Schistosoma-PHM

Drosophila-PHM

Calliactis-PHM

C.ele-PAM

C.ele-PHM

Rat-PAM

Bovine-PAM

Human-PAM Planaria-PHM 100 46.0 41.5 43.3 33.6 38.9 40.0 40.0 41.6 Schistosoma-PHM 100 40.9 39.5 35.7 41.0 36.7 37.0 37.6 Drosophila-PHM 100 45.4 37.5 47.0 40.5 40.5 44.4 Calliactis-PHM 100 41.3 39.0 42.3 42.7 42.3

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cloned cDNA Furthermore, we were unable to find

any PCR clones longer than that shown in Fig 1 In

addition, there were four in-frame stop codons

down-stream of the terminator codon in the cloned PHM

cDNA (Fig 2) These results suggest that there is no PAM-like gene (i.e a fused gene containing both PHM and PAL) in the planarians, which would gener-ate a mRNA of 4 kb, as found for higher vertebrate PAMs Therefore, PHM and PAL are coded for by different genes in the planarians and only later in the evolution do these genes appear to have fused, forming

a single gene coding for the bifunctional PAM enzymes that we know from vertebrates

High-resolution (2.1 A˚) X-ray structures of rat PHM domain have been reported previously [41,47] The structure shows two copper-binding domains con-nected by a three-residue linker Domain 1 binds one copper (CuH also termed as CuA) with three histidine

Nd ligands (His107, His108 and His172), while domain

2 binds the other copper (CuM, also termed as CuB) with two histidine Neligands (His242 and His244) and

a methionine sulfur (Met314) [47] The two coppers are 11 A˚ apart and face the interdomain space in such

a way that the cleft between them is fully accessible to solvent and peptide substrates [47] As the peptide sub-strate, a-N-acetyl-diiodo-Tyr-Gly, binds in a pocket close to CuM, this leads to the proposal that dioxygen binds to the CuMsite, as previously suggested (on the basis of spectroscopic and kinetic data) for the related enzyme, DBH [48,49] Therefore, it was proposed that the bound peptide substrate mediates the electron transfer from CuA to CuB, where the activation of molecular oxygen takes place On the basis of FT-IR spectroscopy of carbon monoxide-bound PHM and EXAFS data, however, a superoxide-channeling mech-anism was proposed [50–52] This proposal is based on the finding that both copper sites are reactive towards

CO and, by inference, O2 [50] The substrate-free enzyme in the reduced state binds a single CO, assign-able to CuM–CO, with a frequency of 2092 cm)1 Binding of the peptidylglycine substrate caused the appearance of a second CO frequency at 2062 cm)1, assignable to CuH–CO As the binding of CO to the

CuHcenter suggests the potential binding of O2to the

CuH center, it was suggested that dioxygen is first reduced to superoxide at the CuH center and that the subsequent electron transfer is mediated by a super-oxide molecule, which channels from CuHto CuM[50] Tyr79 of rat PHM was considered to have an assisting role in the channeling of superoxide, formed at CuH, towards CuM The corresponding Tyr33 in planarian PHM probably has a similar role, if the latter scenario

is indeed valid

It was suggested that the evolution of the vertebrate CNS might have begun with free-living flatworms – planarians that evolved before the divergence of meta-zoans into invertebrate and chordate branches [53,54]

Fig 5 Phylogenetic relationships of peptidylglycine a-hydroxylating

monooxygenases (PHMs) or PHM domains from various animals.

Phylogenetic relationships of PHMs [or PHM domains of

peptidyl-glycine a-amidating monooxygenase (PAM)], dopamine

b-hydroxy-lases (DBHs), tyramine b-hydroxyb-hydroxy-lases (TBHs) and dopamine

b-hydroxylase-like proteins (DBHLs) were determined as described

in the text Amino acid sequences for Bovine-PAM (M18683),

Bovine-DBH (J05160), Equus-PAM (D29625), Equus-DBH

(AB029430), Human-PAM (S75038), Human-DBHL (BC018756),

Human-DBH (X13256), Mouse-PAM (U79523), Mouse-DBH

(S50200), Mouse-DBHL (AB065134), Mouse-MonX

(monooxyge-nase X) (AK081586), Rat-PAM (M82845 and X59687), Rat-DBH

(L12407), Ciona-PAM (AK113539), Xenopus-PAM (X62771),

C.ele-PHM (AC025726), C.ele-PAM (U80438), C.elegans-TBH (Z99942),

Chicken-DBHL (AF327450), planarian-PHM (this study),

Drosophila-PHM (AY069103), Drosphila-TBH (AE003442), Schistosoma-Drosophila-PHM

(AY172995), Lymnae-PHM4, Lymnae-PHM3, Lymnae-PHM2,

Lym-nae-PHM1 (AF109920), Calliactis-PHM (AF036337), Aplysia-PAM

(AF140271) and Heterodera-PHM (AY242521) were obtained from

the DNA Data Bank of Japan (Mishima, Japan) The phylogenetic

tree was created from the distance matrix by using the

neighbor-joining method.

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Planarians are among the simplest animals to develop

a body plan of bilateral symmetry and axes of growth

with gradients of genetic expression, enabling

cephali-zation, dorsal and ventral surfaces, medial and lateral

regions, and an aggregate of neural cells in the head

that form a bilobed brain The brain, a neural

struc-ture located in the head, differs from a ganglion by the

following characteristics, [53,54] namely (a) a brain

serves the entire body, not just restricted segments, (b)

it has functionally specialized parts, (c) it is bilobar

with commissures, (d) neurons form the surface with

axons in the central core, (e) interneurons are more

numerous than primary motor or primary sensory

neu-rons, and (f) multisynaptic rather than monosynaptic

circuits predominate The planarian brain fulfills all

the criteria listed above Neurons of the planarian

brain are known to more closely resemble those of

ver-tebrates than those of advanced inverver-tebrates,

exhibit-ing typical vertebrate features of multipolar shape,

extensive dendritic branching, the presence of dendritic

spines for synaptic contact, a single axon per neuron,

expression of certain vertebrate-like neural proteins,

and relatively slow, spontaneously generated electrical

activity [54] Thus, the planarian CNS appears to offer

insight into the origin of the vertebrate CNS by being

the simplest and most remote animal to exhibit these

vertebrate features [54]

Agata and coworkers described in detail the

struc-ture of the CNS of the freshwater planarian

Duge-sia japonica by using several molecular markers,

including prohormone convertase PC2 [15] and

synaptotagmin [42] By using both in situ hybridization

and immunohistochemical techniques, they found that

the CNS is composed of two morphologically distinct

structures, two longitudinal VNC and an anterior

brain The brain forms independent, inverted U-shaped

lobes located dorsally to the VNC The brain contains

nine branched structures (first-ninth) on each side of the body, and nine branches project away from each lobe towards the periphery of the head In spite of this unique structure of the CNS in the planarian in com-parison with those in vertebrates, the basic architecture

of the planarian CNS has undergone functional regio-nalization, based on the comparative genomic analysis

of planarian expressed sequence tags (ESTs) [44] and DNA microarray analysis [43] Furthermore, most of these nervous system-related genes were found to be shared among human, fruit fly and nematode These include genes for neurotransmission, such as the pro-duction of neurotransmitters, receptors⁄ channels for the neurotransmitters, and synaptic vesicles

In higher animals, such as human, neuropeptides are synthesized as preprohormones at the rough endoplas-mic reticulum During translocation across the rough endoplasmic reticulum membranes, the prepart is removed and the prohormone region is sorted into the Golgi apparatus and subsequently into neurosecretory dense-core vesicles [6] In the Golgi apparatus and neu-rosecretory vesicles, the prohormones are processed to yield the bioactive neuropeptides The following pro-cessing steps are involved, namely (a) prohormone convertase, which cleaves at the C-terminal sites of certain dibasic and monobasic residues and liberates immature propeptides, (b) carboxypeptidase specific for basic residues, which removes the C-terminal basic amino acid residues, and (c) PHM and PAL, two con-secutive enzymatic reactions where the C-terminal Gly residue is converted into an amide group [6] In pla-narians, a homolog of PC2 (prohormone convertase 2) [15] and a carboxypeptidase E homolog [43] were iden-tified and both genes were expressed in the planarian CNS [15,43], although a homolog of PAL has not been identified These observations, together with our find-ings on PHM (present study) and cytochrome b561

Fig 6 Whole mount in situ hybridization

with a planarian peptidylglycine

a-hydroxylat-ing monooxygenase (PHM) antisense RNA

probe (A, B) and a cytochrome b561

anti-sense RNA probe (C, D) (A, C) Dorsal view.

(B, D) Ventral view Anterior is left and

pos-terior is right Positive signals for the PHM

were detected in the brain (br), eyes (e),

and ventral nerve cords (vnc) The

distribu-tion was very similar to that of the planarian

cytochrome b561(C, D).

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[40], indicate that the amidated

neuropeptide-produ-cing neurons had already acquired the neuroendocrine

vesicles equivalent to those seen in the present-day

neurons of higher animals by the time of the

emer-gence of the Plathelminthes in the evolutionary

his-tory

The eye of the planarian is one of the most ancestral

and primitive types of visual systems in animals and is

of particular interest in light of the proposal that all eyes originate from a common evolutionary precursor [55] The planarian eyes consist of two cell types, namely pigment cells and photoreceptors [45] The pig-ment cells form a cup-shaped structure [56], while the photoreceptors are located outside the pigment eye cup and have two types of processes One type of process enters the eye cup and forms rhabdomeres, an

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assem-bly of microvilli which may be associated with

photo-pigments The other processes are optic nerve fibers

projecting onto the brain [15] As both PHM and a

homologue of PC2 (prohormone convertase), together

with cytochrome b561, are expressed in visual neurons

of the planarian, amidated peptides must have

import-ant mediators during the light perception in the

pla-narian It should be noted that cytochrome b561 was

present mainly in the region between the nucleus and

the rhabdomeres of the photoreceptor cells, but little

in the rhabdomeres The region where cytochrome b561

was present is termed ‘stalk’ and contains smooth

endoplasmic reticulum, Golgi apparatus, and smooth

surfaced and coated vesicles [57] Electron microscopic

studies have also shown that, in the stalk region,

effer-ent nerve fibers form synapses with dendritic spines

protruding from stalks of the photoreceptor cells The

structure appeared similar to that in the neuropil of

the brain [57] In the stalk region of the photoreceptor

cells, neuropeptides may be synthesized and work to

modulate the transmission of light stimulus To clarify

the role of the amidated neuropeptides in the planarian

eyes and visual neurons, more detailed morphological

and physiological studies, including identification of

the neuropeptides, will be required

Experimental procedures

Organisms

All planarians in this study were derived from a single

worm of Dugesia japonica collected in the Irima River in

Gifu, Japan and maintained as described (clonal strain: GI)

[58]

Cloning and sequencing of planarian PHM cDNA

A cDNA library of the planarian made with k ZAP II

vec-tor (Stratagene, La Jolla, CA, USA) [15] was used as a

tem-plate for PCR The degenerate primers used for PCR were

designed based on the conserved amino acid sequences in the PHM domain of human PAM, bovine PAM, rat PAM, Xenopus PAM, and C elegans PAM The primers were PAM-S4 (5¢-TTYGCITWYMGIGTICAYRCNCA-3¢)

(P⁄ V)FAFR(T ⁄ V)H(T ⁄ A)H; positions 231–238 of human PAM underlined in Fig 5] and PAM-A3 (5¢-RTACA TIADRTAIARRTTRCACATYTC-3¢) [coding for the con-served amino acid sequence of EMCN(F⁄ L)Y(I ⁄ M ⁄ L); positions 308–315 of human PAM underlined in Fig 5] The reaction mixture consisted of 1 lL each of 10· Taq buffer, 2.5 mm dNTPs, 10 lm forward primer, 10 lm reverse primer, and planarian head cDNA library mixture,

5 lL of autoclaved H2O, and 0.05 lL of AmpliTaq DNA polymerase (Applied Biosystems) in a final volume of

10 lL Thermal cycling was performed with 40 cycles of the following step program: 94C for 1 min, 43.5 C for 1 min (primer annealing), and 72C for 1 min (primer extension) Final primer extension was made at 72C for 5 min The PCR products were separated in a 6.0% polyacrylamide gel The band with expected size (265 bp) was isolated and used as a template for re-amplification with the same set of primers (PAM-S4⁄ PAM-A3) The PCR product was cloned into pT7Blue T vector (Novagen) by using TA cloning and sequenced with a DNA sequencer (Model DSQ-1000 L; Shimadzu Corp., Kyoto, Japan) Based on the nucleotide sequence obtained, two specific forward primers – spPAM-S2 (5¢-AAACTGCAGAGAAATTGGTAAGAAATCTCC-3¢)

TGCCAGATG-3¢) –were synthesized The former primer had a PstI site as an adaptor The 3¢ end of the cDNA was amplified by the 3¢-RACE method [59] using these primers and cDNA from the planarian head, cloned and sequenced This led to a clarification of the 3¢ half of the planarian PHM cDNA sequence including the terminal poly(A) sequence Based on the nucleotide sequence obtained, one specific reverse primer, spPAM-A3 (5¢-ATGAAGCTTAT CATTTCTTTGAACGCTTCG-3¢), was synthesized By using by using spPAM-S3 and spPAM-A3, the planarian cDNA library was screened by stepwise dilution, as previously described [60] A single phage plaque containing

Fig 7 Expression of the peptidylglycine a-hydroxylating monooxygenase (PHM) gene in the planarian brain and eyes A horizontal section of the planarian head was subjected to in situ hybridization (brown) with the planarian PHM antisense RNA probe (A) and to immunocytochemi-cal staining (red) with antibody against planarian cytochrome b561(B) (C) The image for nuclei staining (blue) with Hoechst 33342 was super-imposed on (B) (A), (B), and (C) are the same frame (D), (E), and (F) are a high magnification of (A), (B), and (C), respectively, and are the same frame White and yellow arrowheads point the same cell in (D), (E), and (F), respectively The expression signal for the PHM gene was observable in the cell body of neurons in the peripheral region of the brain Cytochrome b561was observable in neuropil (np), composed

of axons and dendrites of the neurons, as well as in their cell bodies (G) In situ hybridization of a horizontal section of eye with antisense PHM probe (brown) (H) Immunohistochemical staining with anti-arrestin (green) was superimposed on nuclei-staining (blue) with Hoechst

33342 The frame was the same as that in (G) The PHM gene was expressed in photoreceptor cells (I) Normarski image of a horizontal section of the eye was superimposed on the nuclei-staining (blue) with Hoechst 33342 (J) Double stained image with anti-arrestin immuno-globulin (green) and with anti-cytochrome b 561 immunoglobulin (red) The frame is the same as that in (I) Note that cytochrome b 561 present

in the ‘stalk’ region adjacent to the opening of the pigment eye cup showed a marked contrast to the even distribution of arrestin in photo-receptor cells Scale bars,  0.1 mm.

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