All variants in the OTX2 gene

This database is one of the "Eye disease" gene variant databases.
Information The variants shown are described using the transcript reference sequence.

75 entries on 1 page. Showing entries 1 - 75.
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Effect     

Exon     

AscendingDNA change (cDNA)     

RNA change     

Protein     

Classification method     

Clinical classification     

DNA change (genomic) (hg19)     

DNA change (hg38)     

Published as     

ISCN     

DB-ID     

Variant remarks     

Reference     

ClinVar ID     

dbSNP ID     

Origin     

Segregation     

Frequency     

Re-site     

VIP     

Methylation     

Owner     
+?/. - r.0? r.(?) p.(?) - likely pathogenic g.57269187_57925545dup g.56802469_57458827dup RDH12 (NM_152443; OMIM: 608830): c.164C>T; p.Thr55Met (het) c.295C>A; p.Leu99Ile (het) (RP), OTX2 (NM_021728.3; OMIM: 600037): duplica tion Arr[hg19]14q22.3 (57269186_57925544)dup (het) (hemifacial microsomia) - OTX2_000095 heterozygous PubMed: Ehrenberg 2019 - - Germline yes - - - - LOVD
+/. _1_5_ c.(?_-4242057)_(*3517673_?)del r.0 p.0 - pathogenic (dominant) g.(?_53750780)_(61518967_?)del g.(?_53284062)_(61052249_?)del - hg18 14q22.2q23.1(52,820,533-60,588,720)x1 OTX2_000135 7.7Mb deletion PubMed: Chassaing 2014 - - Germline/De novo (untested) - - - - - Johan den Dunnen
+?/. - c.-264604_*1000416del r.0? p.0? - likely pathogenic g.56268037_57541514del - Whole gene deletion - OTX2_000122 - - - - De novo - - - - - LOVD
+/. _1_5_ c.(?_-223426)_(*2060286_?)del r.0 p.0 - pathogenic (dominant) g.(?_55208167)_(57500336_?)del g.(?_54741449)_(57033618_?)del - hg18 14q22.2q23.1(54,277,920-56,570,089)x1 OTX2_000136 2.3Mb deletion PubMed: Chassaing 2007, PubMed: Chassaing 2014 - - Germline - - - - - Johan den Dunnen
+?/. - c.? r.(?) p.(Ser176Phefs*10) - likely pathogenic (dominant) g.? - 527del (Pro177*) - SERPINA1_000009 - PubMed: Bryant 2018 - - De novo - - - - - LOVD
+?/. - c.? r.? p.? - likely pathogenic g.? - c.G640T p.G214X - SERPINA1_000009 - PubMed: Wang 2016 - - De novo - - - - - LOVD
+/. - c.? r.(?) p.? - pathogenic g.? - p.Q181Hfs×7 - SERPINA1_000009 - PubMed: Wang-2013 - - Unknown - - - - - LOVD
+/. - c.23C>A r.(?) p.(Pro8Gln) - pathogenic g.57272152G>T g.56805434G>T - - OTX2_000090 - PubMed: Wang 2015 - - Germline - - - - - LOVD
?/. - c.38A>G r.(?) p.(Asn13Ser) - VUS g.57272137T>C - OTX2(NM_021728.4):c.38A>G (p.(Asn13Ser)) - OTX2_000131 VKGL data sharing initiative Nederland - - - CLASSIFICATION record - - - - - VKGL-NL_Leiden
+?/. - c.53C>T r.(?) p.(Thr18Ile) - likely pathogenic g.57272122G>A g.56805404G>A - - OTX2_000089 not in 624 control chromosomes PubMed: Sun 2015 - - Germline - 1/596 chromosomes - - - LOVD
?/. - c.61G>C r.(?) p.(Gly21Arg) - VUS g.57272114C>G - OTX2(NM_021728.4):c.61G>C (p.G21R) - OTX2_000132 VKGL data sharing initiative Nederland - - - CLASSIFICATION record - - - - - VKGL-NL_VUmc
-/. - c.97+12C>T r.(=) p.(=) - benign g.57272066G>A g.56805348G>A OTX2(NM_001270524.2):c.97+12C>T - OTX2_000073 VKGL data sharing initiative Nederland - - - CLASSIFICATION record - - - - - VKGL-NL_AMC
-/. 3i c.98-46C>A r.(=) p.(=) - benign g.57271127G>T g.56804409G>T - - OTX2_000010 - - - rs2277499 Germline - - - - - Andreas Laner
?/. - c.100C>T r.(?) p.(Pro34Ser) - VUS g.57271079G>A g.56804361G>A OTX2(NM_021728.4):c.100C>T (p.P34S) - OTX2_000072 VKGL data sharing initiative Nederland - - - CLASSIFICATION record - - - - - VKGL-NL_AMC
?/. - c.106G>C r.(?) p.(Ala36Pro) - VUS g.57271073C>G g.56804355C>G OTX2 c.106G>C, p.Ala36Pro - OTX2_000098 heterozygous PubMed: Thorsteinsson 2021 - - Unknown ? - - - - LOVD
?/. - c.125C>A r.(?) p.(Thr42Asn) - VUS g.57271054G>T g.56804336G>T OTX2(NM_001270524.2):c.101C>A (p.T34N), OTX2(NM_021728.4):c.125C>A (p.(Thr42Asn)) - OTX2_000071 VKGL data sharing initiative Nederland - - - CLASSIFICATION record - - - - - VKGL-NL_AMC
?/. - c.125C>A r.(?) p.(Thr42Asn) - VUS g.57271054G>T - OTX2(NM_001270524.2):c.101C>A (p.T34N), OTX2(NM_021728.4):c.125C>A (p.(Thr42Asn)) - OTX2_000071 VKGL data sharing initiative Nederland - - - CLASSIFICATION record - - - - - VKGL-NL_Leiden
?/. - c.125C>T r.(?) p.(Thr42Ile) ACMG VUS g.57271054G>A g.56804336G>A - - OTX2_000130 ACMG PP3, PM2, PP2 PubMed: Weisschuh 2024 - - Germline - - - - - Johan den Dunnen
?/. - c.126C>A r.(?) p.(Thr42=) - VUS g.57271053G>T g.56804335G>T - - OTX2_000087 - PubMed: Costa 2017 - - Germline - - - - - LOVD
-?/. - c.142C>A r.(?) p.(Arg48=) - likely benign g.57271037G>T - OTX2(NM_001270524.1):c.118C>A (p.R40=) - OTX2_000124 VKGL data sharing initiative Nederland - - - CLASSIFICATION record - - - - - VKGL-NL_Rotterdam
?/. - c.155C>A r.(?) p.(Thr52Lys) - VUS g.57271024G>T g.56804306G>T - - OTX2_000074 VKGL data sharing initiative Nederland - - - CLASSIFICATION record - - - - - VKGL-NL_Nijmegen
+?/. - c.250-1G>A r.spl p.? - likely pathogenic g.57270931A>T g.56804213A>T OTX2 c.249-1G>A, Splicing - OTX2_000125 error in annotation, from published sequence the mutation is c.250-1G>A; heterozygous PubMed: Matias-Perez 2018 - - Germline yes absent in 240 in-house exomes alleles from Mexican individuals without ocular malformations - - - LOVD
+?/. 21 c.250-1G>A r.spl p.? - likely pathogenic g.57270931A>T g.56804213A>T OTX2 c.249-1G>A, Splicing - OTX2_000125 error in annotation, from published sequence the mutation is c.250-1G>A; heterozygous PubMed: Matias-Perez 2018 - - Germline yes absent in 240 in-house exomes alleles from Mexican individuals without ocular malformations - - - LOVD
+?/. 21 c.250-1G>A r.spl p.? - likely pathogenic g.57270931A>T g.56804213A>T OTX2 c.249-1G>A, Splicing - OTX2_000125 error in annotation, from published sequence the mutation is c.250-1G>A; heterozygous PubMed: Matias-Perez 2018 - - Germline yes absent in 240 in-house exomes alleles from Mexican individuals without ocular malformations - - - LOVD
+/. - c.255G>A r.(?) p.(Trp85*) - pathogenic (dominant) g.57269068C>T g.56802350C>T - - OTX2_000084 - PubMed: Sanchez-Navarro 2018 - - De novo - - - - - LOVD
+?/. - c.259G>A r.(?) p.(Glu87Lys) - likely pathogenic g.57270920C>T g.56804202C>T OTX2(NM_001270524.2):c.235G>A (p.E79K) - OTX2_000070 VKGL data sharing initiative Nederland - - - CLASSIFICATION record - - - - - VKGL-NL_AMC
+?/. - c.259G>A r.(?) p.(Glu87Lys) - likely pathogenic g.57270920C>T g.56804202C>T - - OTX2_000070 not in 624 control chromosomes PubMed: Sun 2015 - - Germline - 1/596 chromosomes - - - LOVD
+?/. - c.263C>G r.(?) p.(Ser88Trp) - likely pathogenic g.57270916G>C g.56804198G>C OTX2 c.263C>G, p.Ser88Trp - OTX2_000097 heterozygous PubMed: Turro 2020 - - Germline/De novo (untested) ? - - - - LOVD
?/. - c.263C>T r.(?) p.(Ser88Leu) - VUS g.57270916G>A g.56804198G>A - - OTX2_000081 VKGL data sharing initiative Nederland - - - CLASSIFICATION record - - - - - VKGL-NL_Nijmegen
+/. 4 c.265C>T r.(?) p.? - pathogenic g.57270914G>A - c.265c>T - OTX2_000091 - PubMed: Gonzalez Rodriguez 2010 - - Germline - - - - - LOVD
+?/. 4 c.270A>T r.(?) p.(Arg90Ser) - likely pathogenic g.57270909T>A - c.270A>T, p.R90S - OTX2_000116 - - - - Germline - 0/96 caucasian controls - - - LOVD
+/. - c.272dup r.(?) p.(Val92Glyfs*4) ACMG pathogenic g.57270907dup g.56804189dup NM_172337.2:c.248dup - OTX2_000086 ACMG PVS1, PM2, PM6 PubMed: Patel 2017 - - Germline/De novo (untested) - - - - - LOVD
+/. - c.273G>C r.(?) p.(Gln91His) - pathogenic (dominant) g.57270906C>G g.56804188C>G - - OTX2_000082 - PubMed: Slavotinek 2015 - - De novo - - - - - Johan den Dunnen
+/. - c.278G>A r.(?) p.(Trp93*) ACMG pathogenic (dominant) g.57269069C>T g.56802351C>T NM_172337.2:c.254G>A - OTX2_000085 ACMG PVS1, PS2, PM2 PubMed: Patel 2017 - - De novo - - - - - LOVD
+/. - c.289C>T r.(?) p.(Arg97Ter) - pathogenic (dominant) g.57269058G>A g.56802340G>A - - OTX2_000137 - PubMed: Chassaing 2014 - - Germline/De novo (untested) - - - - - Johan den Dunnen
+/. - c.289C>T r.(?) p.(Arg97Ter) - pathogenic (dominant) g.57269058G>A g.56802340G>A - - OTX2_000137 - PubMed: Chassaing 2014 - - Germline - - - - - Johan den Dunnen
+/. - c.296C>A r.(?) p.(Ala99Asp) - pathogenic (!) g.57269051G>T g.56802333G>T - - OTX2_000129 mother likely incomplete penetrant carrier PubMed: Riera 2017 - - Germline - - - - - Johan den Dunnen
+/. - c.316del r.(?) p.(Gln106AsnfsTer11) - pathogenic (dominant) g.57269031del g.56802313del 316delC - OTX2_000134 - PubMed: Chassaing 2012, PubMed: Chassaing 2014 - - Germline - - - - - Johan den Dunnen
+/. 5 c.402dup r.(?) p.(Thr135Asnfs*10) - likely pathogenic g.57268945dup - c.402insC - OTX2_000110 - - - - De novo - - - - - LOVD
+/. 5 c.413C>G r.(?) p.(Ser138*) - pathogenic g.57268934G>C - c.413C>G p.S138X - OTX2_000109 - - - - De novo - 0/181 controls - - - LOVD
?/. - c.425C>G r.(?) p.(Pro142Arg) - VUS g.57268922G>C g.56802204G>C OTX2(NM_001270523.1):c.401C>G (p.(Pro134Arg)), OTX2(NM_001270524.1):c.401C>G (p.P134R) - OTX2_000080 VKGL data sharing initiative Nederland - - - CLASSIFICATION record - - - - - VKGL-NL_Rotterdam
?/. - c.425C>G r.(?) p.(Pro142Arg) - VUS g.57268922G>C g.56802204G>C OTX2(NM_001270523.1):c.401C>G (p.(Pro134Arg)), OTX2(NM_001270524.1):c.401C>G (p.P134R) - OTX2_000080 VKGL data sharing initiative Nederland - - - CLASSIFICATION record - - - - - VKGL-NL_Leiden
-/. - c.444G>C r.(?) p.(Pro148=) - benign g.57268903C>G g.56802185C>G OTX2(NM_001270524.1):c.420G>C (p.P140=), OTX2(NM_001270524.2):c.420G>C (p.P140=) - OTX2_000069 VKGL data sharing initiative Nederland - - - CLASSIFICATION record - - - - - VKGL-NL_AMC
-?/. - c.444G>C r.(?) p.(Pro148=) - likely benign g.57268903C>G - OTX2(NM_001270524.1):c.420G>C (p.P140=), OTX2(NM_001270524.2):c.420G>C (p.P140=) - OTX2_000069 VKGL data sharing initiative Nederland - - - CLASSIFICATION record - - - - - VKGL-NL_Rotterdam
?/. - c.448A>G r.(?) p.(Ile150Val) - VUS g.57268899T>C g.56802181T>C OTX2(NM_001270524.2):c.424A>G (p.I142V) - OTX2_000068 VKGL data sharing initiative Nederland - - - CLASSIFICATION record - - - - - VKGL-NL_AMC
-/. - c.459C>T r.(?) p.(Ser153=) - benign g.57268888G>A g.56802170G>A OTX2(NM_001270524.2):c.435C>T (p.S145=) - OTX2_000079 VKGL data sharing initiative Nederland - - - CLASSIFICATION record - - - - - VKGL-NL_AMC
+/. 5 c.485del r.(?) p.(Pro162Glnfs*24) - pathogenic g.57268862del - c.485delC (p.Pro162G.Infs*24) - OTX2_000105 - - - - Unknown ? - - - - LOVD
?/. - c.494T>A r.(?) p.(Ile165Asn) - VUS g.57268853A>T g.56802135A>T OTX2(NM_001270524.1):c.470T>A (p.I157N), OTX2(NM_001270524.2):c.470T>A (p.I157N) - OTX2_000067 VKGL data sharing initiative Nederland - - - CLASSIFICATION record - - - - - VKGL-NL_AMC
?/. - c.494T>A r.(?) p.(Ile165Asn) - VUS g.57268853A>T g.56802135A>T OTX2(NM_001270524.1):c.470T>A (p.I157N), OTX2(NM_001270524.2):c.470T>A (p.I157N) - OTX2_000067 VKGL data sharing initiative Nederland - - - CLASSIFICATION record - - - - - VKGL-NL_Rotterdam
+?/. - c.506C>G r.(?) p.(Ser169*) ACMG likely pathogenic g.57268841G>C g.56802123G>C OTX2, gene that can display both dominant and recessive patterns of inheritance, c.506C>G, p.Ser169*, heterozygous - OTX2_000099 - PubMed: Perea-Romero 2021 - - Unknown ? - - - - LOVD
+?/. - c.534C>A r.(?) p.(Cys178*) - likely pathogenic g.57268813G>T g.56802095G>T - - OTX2_000088 - PubMed: Ellingford 2016 - - Germline - - - - - LOVD
+?/. 5 c.538C>T r.(?) p.(Gln180*) - likely pathogenic g.57268809G>A - c.538C>T - OTX2_000126 - PubMed: Panneman 2023 - - Unknown - - - - - Daan Panneman
+/. - c.559C>T r.(?) p.(Gln187*) - pathogenic (dominant) g.57268764G>A g.56802046G>A - - OTX2_000083 - PubMed: Sanchez-Navarro 2018 - - De novo - - - - - LOVD
+/. 5 c.576_577insCT r.(?) p.(Tyr193Leufs*22) - pathogenic g.57268770_57268771insAG - c.576-577insCT p.S136fsX178 - OTX2_000102 - - - - Unknown - - - - - LOVD
+/. - c.591T>A r.(?) p.(Tyr197Ter) - pathogenic g.57268756A>T - OTX2(NM_001270524.2):c.567T>A (p.Y189*) - OTX2_000075 VKGL data sharing initiative Nederland - - - CLASSIFICATION record - - - - - VKGL-NL_VUmc
-/. - c.663C>T r.(?) p.(Pro221=) - benign g.57268684G>A g.56801966G>A OTX2(NM_001270524.1):c.639C>T (p.P213=), OTX2(NM_001270524.2):c.639C>T (p.P213=) - OTX2_000066 VKGL data sharing initiative Nederland - - - CLASSIFICATION record - - - - - VKGL-NL_AMC
-?/. - c.663C>T r.(?) p.(Pro221=) - likely benign g.57268684G>A g.56801966G>A OTX2(NM_001270524.1):c.639C>T (p.P213=), OTX2(NM_001270524.2):c.639C>T (p.P213=) - OTX2_000066 VKGL data sharing initiative Nederland - - - CLASSIFICATION record - - - - - VKGL-NL_Rotterdam
+?/. 5 c.664G>M r.(?) p.(His337Arg) - likely pathogenic g.57268683C>K - p.OTX2-G222R - OTX2_000093 - PubMed: Schorderet-2013 - - Unknown ? - - - - LOVD
+/. 5 c.675del r.(?) p.(Thr226Hisfs*76) - pathogenic g.57268673del g.56801955del NM_001270523.1:c.651del - OTX2_000061 - - - - Unknown - - - - - Elena Semina
?/. - c.677_678insTGTG r.(?) p.(Leu227Valfs*34) - VUS g.57268669_57268670insCACA g.56801951_56801952insCACA OTX2 c.677_678insTGTG, p.Leu227ValfsTer34 - OTX2_000096 unsolved PubMed: Zampaglione 2020 - - Unknown ? - - - - LOVD
+?/. - c.686_696del r.(?) p.(Pro229GlnfsTer27) - likely pathogenic g.57268652_57268662del g.56801934_56801944del OTX2(NM_021728.3):c.686_696del (p.(Pro229GlnfsTer27)) - OTX2_000078 VKGL data sharing initiative Nederland - - - CLASSIFICATION record - - - - - VKGL-NL_Leiden
?/. - c.713A>T r.(?) p.(His238Leu) - VUS g.57268634T>A - OTX2(NM_172337.3):c.689A>T (p.H230L) - OTX2_000123 VKGL data sharing initiative Nederland - - - CLASSIFICATION record - - - - - VKGL-NL_Groningen
-?/. - c.727C>G r.(?) p.(Pro243Ala) - likely benign g.57268620G>C - OTX2(NM_001270523.1):c.703C>G (p.(Pro235Ala)) - OTX2_000127 VKGL data sharing initiative Nederland - - - CLASSIFICATION record - - - - - VKGL-NL_Leiden
?/. - c.743C>T r.(?) p.(Thr248Ile) - VUS g.57268604G>A g.56801886G>A OTX2(NM_001270524.1):c.719C>T (p.T240I), OTX2(NM_021728.4):c.743C>T (p.(Thr248Ile)) - OTX2_000065 VKGL data sharing initiative Nederland - - - CLASSIFICATION record - - - - - VKGL-NL_Rotterdam
?/. - c.743C>T r.(?) p.(Thr248Ile) - VUS g.57268604G>A - OTX2(NM_001270524.1):c.719C>T (p.T240I), OTX2(NM_021728.4):c.743C>T (p.(Thr248Ile)) - OTX2_000065 VKGL data sharing initiative Nederland - - - CLASSIFICATION record - - - - - VKGL-NL_Leiden
+/. - c.749del r.(?) p.(Gly250Aspfs*52) - pathogenic (dominant) g.57268600del g.56801882del 749delG - OTX2_000076 - - - - De novo ? - - - - Isabel Filges
+?/. 5 c.761C>A r.(?) p.(Ser254*) - likely pathogenic g.57268586G>T g.56801868G>T - - OTX2_000060 seems not associated with ID phenotype PubMed: Gilissen 2014 - - De novo no - - - - Marianne Vos (LOVD-team)
+/. 5 c.781_784del r.(?) p.(Thr261LeufsTer40) - pathogenic (dominant) g.57268565_57268568del g.56801847_56801850del - - OTX2_000128 - PubMed: Nambot 2018 - - De novo - - - - - Johan den Dunnen
+?/. - c.811del r.(?) p.(Thr271Leufs*31) - likely pathogenic g.57268538del g.56801820del OTX2;NM_021728.2;c.[811del];[811=];p.[(Thr271Leufs*31)];[(Thr271=)]; - OTX2_000094 heterozygous PubMed: Jiman 2020 - - Unknown ? - - - - LOVD
-?/. - c.831C>T r.(?) p.(Asn277=) - likely benign g.57268516G>A g.56801798G>A OTX2(NM_001270524.1):c.807C>T (p.N269=), OTX2(NM_001270524.2):c.807C>T (p.N269=) - OTX2_000064 VKGL data sharing initiative Nederland - - - CLASSIFICATION record - - - - - VKGL-NL_Rotterdam
-/. - c.831C>T r.(?) p.(Asn277=) - benign g.57268516G>A - OTX2(NM_001270524.1):c.807C>T (p.N269=), OTX2(NM_001270524.2):c.807C>T (p.N269=) - OTX2_000064 VKGL data sharing initiative Nederland - - - CLASSIFICATION record - - - - - VKGL-NL_AMC
?/. - c.843C>A r.(?) p.(Asp281Glu) - VUS g.57268504G>T - OTX2(NM_021728.4):c.843C>A (p.(Asp281Glu)) - OTX2_000133 VKGL data sharing initiative Nederland - - - CLASSIFICATION record - - - - - VKGL-NL_Leiden
+?/. - c.844T>A r.(?) p.(Cys282Ser) - likely pathogenic g.57268503A>T g.56801785A>T c.844?T>A; p.C282S - OTX2_000092 - PubMed: Kersten 2018 - - Germline/De novo (untested) - - - - - LOVD
?/. - c.872C>T r.(?) p.(Ser291Leu) - VUS g.57268475G>A g.56801757G>A - - OTX2_000077 VKGL data sharing initiative Nederland - - - CLASSIFICATION record - - - - - VKGL-NL_Nijmegen
?/. 5 c.*340del r.(=) p.(=) - VUS g.57268113del g.56801395del - - OTX2_000063 - - - rs3215889 Germline - - - - - Andreas Laner
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