1

GUC2D gene; RetGC1 gene; retina-specific guanylyl cyclase. Stand: 11.04.2006

Sequence based on [2] and Genbank: NM_000180, AJ222657, AC107913

EEEE= Extracellular - TTTT= Transmembrane - KKKK= Kinase-like - CCCC= Catalytic - IIII= Intracellular/ cytoplasmic - SSSS= Signal peptide

Nucleotide numbering - Aminoacid numbering

n: nucleotide position varying between AJ222657 and AC107913

Sequence variations (Polymorphism, rare variant, LCA1, CORD6, EOSRP, included in LCA-Microarray but no reference available)

Italics: Mutation can be tested on LCA-Microarray

-371 -361 -351 -341 -331 -321 -311

gagca gggca gtgtg gccga cggct gaaag gggaa gctgc ggctg ctttt gtgca ggggt ggtgg tgatg agggt

ctcgt cccgt cacac cggct gccga ctttc ccctt cgacg ccgac gaaaa cacgt cccca ccacc actac tccca

-301 291 -281 -271 -261 -251 -241 -231

gatgt ggggg gctgg aaggc atgga gggga aagga tctgg ctgac tacct ggaag ccagg acaga tccca cccca

ctaca ccccc cgacc ttccg tacct cccct ttcct agacc gactg atgga ccttc ggtcc tgtct agggt ggggt

-221 -211 -201 -191 -181 -171 -161

gaaag gcgca gtagg ggctc tcatc ctcca ctagc ccgcc cctcc ctacc taatt aagga cccta atcag cttgg

ctttc cgcgt catcc ccgag agtag gaggt gatcg ggcgg ggagg gatgg attaa ttcct gggat tagtc gaacc

-151 -141 -131 -121 -111 -101 -91 -81

ggaga ttaag ggctc tggcc ggctg taccc acgcc cccgc cctgg cctgg gctgg caagg aagac ctgtg ggcgg

cctct aattc ccgag accgg ccgac atggg tgcgg gggcg ggacc ggacc cgacc gttcc ttctg gacac ccgcc

del (4,22)

-71 -61 -51 |

|------> GC-1f (2) -41 -31 -21| -10

gcgCC CACAG GGGGA CCGGC CCTGT GACCC CTCAC CGGGG CCGTG GGCCC GAGCC CCGGA CTTCC CT gtaag

cgcGG GTGTC CCCCT GGCCG GGACA CTGGG GAGTG GCCCC GGCAC CCGGG CTCGG GGCCT GAAGG GA cattc

tgtca gaggc ccctc cgctg ggata gggtc ggtct gaggg cgcag gcgag tccct gctga cccct gacgc ctcc

acagt ctccg gggag gcgac cctat cccag ccaga ctccc gcgtc cgctc aggga cgact gggga ctgcg gcgtc

<------| GC-1r (2)

IVS 1

gacgg gggga ggggc aggcc gggtg ggagc gggaa gccgg ggcgg cagaa ggggg cttcg gggcg gtgtc cttgg

gagg ctgcc cccct ccccg tccgg cccac cctcg ccctt cggcc ccgcc gtctt ccccc gaagc cccgc cacag

cccca gttag tcttc ccagc ctccg gaggg ggcgg tagca gcaga atcat cccat gggtt actcg ggctt cgcag

gaacc ggggt caatc agaag ggtcg gaggc ctccc ccgcc atcgt cgtct tagta gggta cccaa tgagc ccgaa

|------> GC-2f (2,14)

ggaga aactc ggggt tacgg ggaga accct agggg aggcc ggggt ctcag tcgct cagcc tgctc cgtct gtgtt

cctct ttgag cccca atgcc cctct tggga tcccc tccgg cccca gagtc agcga gtcgg acgag gcaga cacaa

A=Lys

|A/C=Ile (1,16) |------

-9 1|| 15 30 45 |

AAGC CGGCA ATG ACC GCC TGC GCC CGC CGA GCG GGT GGG CTT CCG GAC CCC GGG CTC TGC GGT CCC

TTCG GCCGT TAC TGG CGG ACG CGG GCG GCT CGC CCA CCC GAA GGC CTG GGG CCC GAG ACG CCA GGG

M T A C A R R A G G L P D P G L C G P

Met Thr Ala Cys Ala Arg Arg Ala Gly Gly Leu Pro Asp Pro Gly Leu Cys Gly Pro

5 10 15

SSSSSSSSSSSSSSSSSSSSSSSSSSSSSSSSSSSSSSSSSSSSSSSSSSSSSSSSSSSSSSSSSSSSSSSSSSS

------dup (16) ------|

C=Arg (1) insC | (1,16) Phe=T

60 | 75 | 90 | 105 120 |

GCG TGG TGG GCT CCG TCC CTG CCC CGC CTC CCC CGG GCC CTG CCC CGG CTC CCG CTC CTG CTG CTC

CGC ACC ACC CGA GGC AGG GAC GGG GCG GAG GGG GCC CGG GAC GGG GCC GAG GGC GAG GAC GAC GAG

A W W A P S L P R L P R A L P R L P L L L L

Ala Trp Trp Ala Pro Ser Leu Pro Arg Leu Pro Arg Ala Leu Pro Arg Leu Pro Leu Leu Leu Leu

20 25 30 35 40

SSSSSSSSSSSSSSSSSSSSSSSSSSSSSSSSSSSSSSSSSSSSSSSSSSSSSSSSSSSSSSSSSSSSSSSSSSSSSSSSSSSSSS

|------> GC-2/2f (14)

T=Ser (2)

135 150 | 165 180

CTG CTT CTG CTG CAG CCC CCC GCC CTC TCC GCC GTG TTC ACG GTG GGG GTC CTG GGC CCC TGG GCT

GAC GAA GAC GAC GTC GGG GGG CGG GAG AGG CGG CAC AAG TGC CAC CCC CAG GAC CCG GGG ACC CGA

L L L L Q P P A L S A V F T V G V L G P W A

Leu Leu Leu Leu Gln Pro Pro Ala Leu Ser Ala Val Phe Thr Val Gly Val Leu Gly Pro Trp Ala

45 50 55 60 <-

SSSSSSSSSSSSSSSSSSSSSSSSSSSSSSSSSSSSSSSSEEEEEEEEEEEEEEEEEEEEEEEEEEEEEEEEEEEEEEEEEEEEEEE

|--del (16)------|

195 210 225 | | 255

TGC GAC CCC ATC TTC TCT CGG GCT CGC CCG GAC CTG GCC GCC CGC CTG GCC GCC GCC CGC CTG AAC

ACG CTG GGG TAG AAG AGA GCC CGA GCG GGC CTG GAC CGG CGG GCG GAC CGG CGG CGG GCG GAC TTG

C D P I F S R A R P D L A A R L A A A R L N

Cys Asp Pro Ile Phe Ser Arg Ala Arg Pro Asp Leu Ala Ala Arg Leu Ala Ala Ala Arg Leu Asn

------| GC-2/1r (14)

65 70 75 80 85

EEEEEEEEEEEEEEEEEEEEEEEEEEEEEEEEEEEEEEEEEEEEEEEEEEEEEEEEEEEEEEEEEEEEEEEEEEEEEEEEEEEEEEEE

(17,3,4) Tyr=A

C=Glu A=Lys |

270 285 | 300 | |

CGC GAC CCC GGC CTG GCA GGC GGT CCC CGC TTC GAG GTA GCG CTG CTG CCC GAG CCT TGC CGG ACG

GCG CTG GGG CCG GAC CGT CCG CCA GGG GCG AAG CTC CAT CGC GAC GAC GGG CTC GGA ACG GCC TGC

R D P G L A G G P R F E V A L L P E P C R T

Arg Asp Pro Gly Leu Ala Gly Gly Pro Arg Phe Glu Val Ala Leu Leu Pro Glu Pro Cys Arg Thr

90 95 100 105

EEEEEEEEEEEEEEEEEEEEEEEEEEEEEEEEEEEEEEEEEEEEEEEEEEEEEEEEEEEEEEEEEEEEEEEEEEEEEEEEEEEEEEE

|------

-SmaI (2,16) del

(16,1) Lys=A

330 345 360 375 |

CCG GGC TCG CTG GGG GCC GTG TCC TCC GCG CTG GCC CGC GTG TCG GGC CTC GTG GGT CCG GTG AAC

GGC CCG AGC GAC CCC CGG CAC AGG AGG CGC GAC CGG GCG CAC AGC CCG GAG CAC CCA GGC CAC TTG

P G S L G A V S S A L A R V S G L V G P V N

Pro Gly Ser Leu Gly Ala Val Ser Ser Ala Leu Ala Arg Val Ser Gly Leu Val Gly Pro Val Asn

110 115 120 125

EEEEEEEEEEEEEEEEEEEEEEEEEEEEEEEEEEEEEEEEEEEEEEEEEEEEEEEEEEEEEEEEEEEEEEEEEEEEEEEEEEEEEEE

------> GC-2/3f (2,4)

390 405 420 435 450

CCT GCG GCC TGC CGG CCA GCC GAG CTG CTC GCC GAA GAA GCC GGG ATC GCG CTG GTG CCC TGG GGC

GGA CGC CGG ACG GCC GGT CGG CTC GAC GAG CGG CTT CTT CGG CCC TAG CGC GAC CAC GGG ACC CCG

<------|GC2/2r (14)

P A A C R P A E L L A E E A G I A L V P W G

Pro Ala Ala Cys Arg Pro Ala Glu Leu Leu Ala Glu Glu Ala Gly Ile Ala Leu Val Pro Trp Gly

130 135 140 145 150

EEEEEEEEEEEEEEEEEEEEEEEEEEEEEEEEEEEEEEEEEEEEEEEEEEEEEEEEEEEEEEEEEEEEEEEEEEEEEEEEEEEEEEE

465 480 495 510

TGC CCC TGG ACG CAG GCG GAG GGC ACC ACG GCC CCT GCC GTG ACC CCC GCC GCG GAT GCC CTC TAC

ACG GGG ACC TGC GTC CGC CTC CCG TGG TGC CGG GGA CGG CAC TGG GGG CGG CGC CTA CGG GAG ATG

C P W T Q A E G T T A P A V T P A A D A L Y

Cys Pro Trp Thr Gln Ala Glu Gly Thr Thr Ala Pro Ala Val Thr Pro Ala Ala Asp Ala Leu Tyr

155 160 165 170

EEEEEEEEEEEEEEEEEEEEEEEEEEEEEEEEEEEEEEEEEEEEEEEEEEEEEEEEEEEEEEEEEEEEEEEEEEEEEEEEEEEEEEE

525 540 555 570 585

GCC CTG CTT CGC GCA TTC GGC TGG GCG CGC GTG GCC CTG GTC ACC GCC CCC CAG GAC CTG TGG GTG

CGG GAC GAA GCG CGT AAG CCG ACC CGC GCG CAC CGG GAC CAG TGG CGG GGG GTC CTG GAC ACC CAC

A L L R A F G W A R V A L V T A P Q D L W V

Ala Leu Leu Arg Ala Phe Gly Trp Ala Arg Val Ala Leu Val Thr Ala Pro Gln Asp Leu Trp Val

175 180 185 190 195

EEEEEEEEEEEEEEEEEEEEEEEEEEEEEEEEEEEEEEEEEEEEEEEEEEEEEEEEEEEEEEEEEEEEEEEEEEEEEEEEEEEEEEE

del (2,16) +BspMI

600 615 | 630 645

GAG GCG GGA CGC TCA CTG TCC ACG GCA CTC AGG GCC CGG GGC CTG CCT GTC GCC TCC GTG ACT TCC

CTC CGC CCT GCG AGT GAC AGG TGC CGT GAG TCC CGG GCC CCG GAC GGA CAG CGG AGG CAC TGA AGG

E A G R S L S T A L R A R G L P V A S V T S

Glu Ala Gly Arg Ser Leu Ser Thr Ala Leu Arg Ala Arg Gly Leu Pro Val Ala Ser Val Thr Ser

200 205 210 215

EEEEEEEEEEEEEEEEEEEEEEEEEEEEEEEEEEEEEEEEEEEEEEEEEEEEEEEEEEEEEEEEEEEEEEEEEEEEEEEEEEEEEEE

660 675 690 705

ATG GAG CCC TTG GAC CTG TCT GGA GCC CGG GAG GCC CTG AGG AAG GTT CGG GAC GGG CCC AGG GTC

TAC CTC GGG AAC CTG GAC AGA CCT CGG GCC CTC CGG GAC TCC TTC CAA GCC CTG CCC GGG TCC CAG

M E P L D L S G A R E A L R K V R D G P R V

Met Glu Pro Leu Asp Leu Ser Gly Ala Arg Glu Ala Leu Arg Lys Val Arg Asp Gly Pro Arg Val

220 225 230 235

EEEEEEEEEEEEEEEEEEEEEEEEEEEEEEEEEEEEEEEEEEEEEEEEEEEEEEEEEEEEEEEEEEEEEEEEEEEEEEEEEEEEEEE

721

ACA G gtagg ctccc ttgca gggtg cgagg aggtc ggctg gtcct gccgg cagcc

TGT C catcc gaggg aacgt cccac gctcc tccag ccgac cagga cggcc gtcgg

T A <------| GC-2/3r (2,14)

Thr Ala

240 241

EEEEEEE

IVS 2

|------> GC3f (2)

ggacg gcgcc gcgag ccaag cctct gtccg cag

cctgc cgcgg cgctc ggttc ggaga caggc gtc

C=Pro

722 735 750 765 |

CA GTG ATC ATG GTG ATG CAC TCG GTG CTG CTG GGT GGC GAG GAG CAG CGC TAC CTC CTG GAG GCC

GT CAC TAG TAC CAC TAC GTG AGC CAC GAC GAC CCA CCG CTC CTC GTC GCG ATG GAG GAC CTC CGG

A V I M V M H S V L L G G E E Q R Y L L E A

Ala Val Ile Met Val Met His Ser Val Leu Leu Gly Gly Glu Glu Gln Arg Tyr Leu Leu Glu Ala

241 245 250 255 260

EEEEEEEEEEEEEEEEEEEEEEEEEEEEEEEEEEEEEEEEEEEEEEEEEEEEEEEEEEEEEEEEEEEEEEEEEEEEEEEEEEEEEEE

795 810 825 840

GCA GAG GAG CTG GGC CTG ACC GAT GGC TCC CTG GTC TTC CTG CCC TTC GAC ACG ATC CAC TAC GCC

CGT CTC CTC GAC CCG GAC TGG CTA CCG AGG GAC CAG AAG GAC GGG AAG CTG TGC TAG GTG ATG CGG

A E E L G L T D G S L V F L P F D T I H Y A

Ala Glu Glu Leu Gly Leu Thr Asp Gly Ser Leu Val Phe Leu Pro Phe Asp Thr Ile His Tyr Ala

265 270 275 280

EEEEEEEEEEEEEEEEEEEEEEEEEEEEEEEEEEEEEEEEEEEEEEEEEEEEEEEEEEEEEEEEEEEEEEEEEEEEEEEEEEEEEEE

855 870 885 900 915

TTG TCC CCA GGC CCG GAG GCC TTG GCC GCA CTC GCC AAC AGC TCC CAG CTT CGC AGG GCC CAC GAT

AAC AGG GGT CCG GGC CTC CGG AAC CGG CGT GAG CGG TTG TCG AGG GTC GAA GCG TCC CGG GTG CTA

L S P G P E A L A A L A N S S Q L R R A H D

Leu Ser Pro Gly Pro Glu Ala Leu Ala Ala Leu Ala Asn Ser Ser Gln Leu Arg Arg Ala His Asp

285 290 295 300 305

EEEEEEEEEEEEEEEEEEEEEEEEEEEEEEEEEEEEEEEEEEEEEEEEEEEEEEEEEEEEEEEEEEEEEEEEEEEEEEEEEEEEEEE

T=Met (18,19)

| T=Cys (1,16) C=Pro (3,23,17)

930 | | 945 960 |

GCC GTG CTC ACC CTC ACG CGC CAC TGT CCC TCT GAA GGC AGC GTG CTG GAC AGC CTG CGC AGG GCT

CGG CAC GAG TGG GAG TGC GCG GTG ACA GGG AGA CTT CCG TCG CAC GAC CTG TCG GAC GCG TCC CGA

A V L T L T R H C P S E G S V L D S L R R A

Ala Val Leu Thr Leu Thr Arg His Cys Pro Ser Glu Gly Ser Val Leu Asp Ser Leu Arg Arg Ala

310 315 320 325

EEEEEEEEEEEEEEEEEEEEEEEEEEEEEEEEEEEEEEEEEEEEEEEEEEEEEEEEEEEEEEEEEEEEEEEEEEEEEEEEEEEEEEE

990 1005 1020 1026

CAA GAG CGC CGC GAG CTG CCC TCT GAC CTC AAT CTG CAG CAG gtaga cggtc ccggg aggag ggaag

GTT CTC GCG GCG CTC GAC GGG AGA CTG GAG TTA GAC GTC GTC catct gccag ggccc tcctc ccttc

Q E R R E L P S D L N L Q Q <-

Gln Glu Arg Arg Glu Leu Pro Ser Asp Leu Asn Leu Gln Gln

330 335 340 342

EEEEEEEEEEEEEEEEEEEEEEEEEEEEEEEEEEEEEEEEEEEEEEEEEEEEEEE

aaggc aaggg agagg ggaga ggaca gccaa agcag ggaga ggagg atgca gccaa tggag aaaga accac tggca

ttccg ttccc tctcc cctct cctgt cggtt tcgtc cctct cctcc tacgt cggtt acctc tttct tggtg accgt

------| GC-3r (2)

gctct agctg tttgg agttt cctgg gtaat gtaga ggctc tggcc ccact tgggg tctct tagtg tacga ggatt

cgaga tcgac aaacc tcaaa ggacc catta catct ccgag accgg ggtga acccc agaga atcac atgct cctaa

gcctc tagag agtag gcaat tcgac ctctc aagtc caaca tcaag cagta aactc taatg aactg taaac tgcaa

cggag atctc tcatc cgtta agctg gagag ttcag gttgt agttc gtcat ttgag attac ttgac atttg acgtt

IVS 3 (+1730 bp)

gggag gaaga gagac ccagt gggga gggag gaaga gagag ccaat gggga gggag gaaga gagcc aatgg ggagg

ccctc cttct ctctg ggtca cccct ccctc cttct ctctc ggtta cccct ccctc cttct ctcgg ttacc cctcc

gagga agaga tagcc aatgg ggagg gatcc tggga acaac taact ggaag gtggc aacag tggat accct gggct

ctcct tctct atcgg ttacc cctcc ctagg accct tgttg attga ccttc caccg ttgtc accta tggga cccga

|------> GC-4r (2)

tgaca ggcag tgaaa gaatc tggtc tgtct gtggg ctgtg acccc gacct ctgag cccct actct ccttc tccag

actgt ccgtc acttt cttag accag acaga caccc gacac tgggg ctgga gactc gggga tgaga ggaag aggtc

G=Cys T=Ser

1027 1035 1050 | 1065 1080 |

GTC TCC CCA CTC TTT GGC ACC ATC TAT GAC GCG GTC TTC TTG CTG GCA AGG GGC GTG GCA GAA

CAG AGG GGT GAG AAA CCG TGG TAG ATA CTG CGC CAG AAG AAC GAC CGT TCC CCG CAC CGT CTT

V S P L F G T I Y D A V F L L A R G V A E

Val Ser Pro Leu Phe Gly Thr Ile Tyr Asp Ala Val Phe Leu Leu Ala Arg Gly Val Ala Glu

343 345 350 355 360

EEEEEEEEEEEEEEEEEEEEEEEEEEEEEEEEEEEEEEEEEEEEEEEEEEEEEEEEEEEEEEEEEEEEEEEEEEEEEEEEEEEE

A=Val (5)

1095 1110 | 1125 1140 1155

GCG CGG GCT GCC GCA GGT GGC AGA TGG GTG TCC GGA GCA GCT GTG GCC CGC CAC ATC CGG GAT GCG

CGC GCC CGA CGG CGT CCA CCG TCT ACC CAC AGG CCT CGT CGA CAC CGG GCG GTG TAG GCC CTA CGC

A R A A A G G R W V S G A A V A R H I R D A

Ala Arg Ala Ala Ala Gly Gly Arg Trp Val Ser Gly Ala Ala Val Ala Arg His Ile Arg Asp Ala

365 370 375 380 385

EEEEEEEEEEEEEEEEEEEEEEEEEEEEEEEEEEEEEEEEEEEEEEEEEEEEEEEEEEEEEEEEEEEEEEEEEEEEEEEEEEEEEEEE

1170 1185 1200 1215

CAG GTC CCT GGC TTC TGC GGG GAC CTA GGA GGA GAC GAG GAG CCC CCA TTC GTG CTG CTA GAC ACG

GTC CAG GGA CCG AAG ACG CCC CTG GAT CCT CCT CTG CTC CTC GGG GGT AAG CAC GAC GAT CTG TGC

Q V P G F C G D L G G D E E P P F V L L D T

Gln Val Pro Gly Phe Cys Gly Asp Leu Gly Gly Asp Glu Glu Pro Pro Phe Val Leu Leu Asp Thr

390 395 400 405

EEEEEEEEEEEEEEEEEEEEEEEEEEEEEEEEEEEEEEEEEEEEEEEEEEEEEEEEEEEEEEEEEEEEEEEEEEEEEEEEEEEEEEE

T=Asp (4,5

1230 | 1245 1260 1275

GAC GCG GCG GGA GAC CGG CTT TTT GCC ACA TAC ATG CTG GAT CCT GCC CGG GGC TCC TTC CTC TCC

CTG CGC CGC CCT CTG GCC GAA AAA CGG TGT ATG TAC GAC CTA GGA CGG GCC CCG AGG AAG GAG AGG

D A A G D R L F A T Y M L D P A R G S F L S