Unique variants in the GUCA1A gene

This database is one of the "Eye disease" gene variant databases.
Information The variants shown are described using the NM_000409.3 transcript reference sequence.

67 entries on 1 page. Showing entries 1 - 67.
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Effect     

Reported     

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     
+?/. 1 - c.-84322_*128532dup r.0? p.0? ACMG likely pathogenic g.42039467_42275673dup - - - C6orf132_000007 ACMG PM2, PVS1 PubMed: Weisschuh 2024 - - Germline - - - - - Johan den Dunnen
-?/. 1 - c.-458+2T>C r.spl? p.? - likely benign g.42123333T>C g.42155595T>C GUCA1A(NM_001319061.1):c.-829+2T>C - GUCA1A_000012 VKGL data sharing initiative Nederland - - - CLASSIFICATION record - - - - - VKGL-NL_Rotterdam
-?/. 2 - c.-457-8C>T r.(=) p.(=) - likely benign g.42130650C>T g.42162912C>T - - GUCA1A_000018 1 homozygous; Clinindb (India), 58 heterozygous; Clinindb (India) PubMed: Narang 2020, Journal: Narang 2020 - rs58894089 Germline - 1/2795 individuals, 58/2795 individuals - - - Mohammed Faruq
-/. 1 - c.9C>T r.(?) p.(Asn3=) - benign g.42141360C>T g.42173622C>T LOC118142757(NM_000409.5):c.9C>T (p.N3=) - GUCA1A_000013 VKGL data sharing initiative Nederland - - - CLASSIFICATION record - - - - - VKGL-NL_AMC
-?/. 1 - c.42C>T r.(?) p.(Ser14=) - likely benign g.42141393C>T g.42173655C>T GUCA1A(NM_001319061.1):c.42C>T (p.S14=) - GUCA1A_000014 VKGL data sharing initiative Nederland - - - CLASSIFICATION record - - - - - VKGL-NL_Rotterdam
?/. 1 - c.50_80del r.(?) p.(Glu17Valfs*22) ACMG VUS g.42141401_42141431del g.42173663_42173693del GUCA1A c.C50_80del p.E17VfsX22 - GUCA1A_000050 number of normal cases with GUCA1A variant: 3; heterozygous PubMed: Mizobuchi 2019 - - Unknown no - - - - LOVD
+?/. 1 - c.55C>T r.(?) (p.H19Y) - likely pathogenic g.42141406C>T g.42173668C>T GUCA1A c.55C > T (p.H19Y) - GUCA1A_000060 heterozygous PubMed: Abbas 2020 - - Unknown ? - - - - LOVD
?/. 1 - c.64T>C r.(?) p.(Tyr22His) - VUS g.42141415T>C g.42173677T>C GUCA1A(NM_001319061.1):c.64T>C (p.Y22H) - GUCA1A_000015 VKGL data sharing initiative Nederland - - - CLASSIFICATION record - - - - - VKGL-NL_Rotterdam
?/. 2 - c.124T>A r.(?) p.(Phe42Ile) ACMG VUS g.42141475T>A g.42173737T>A GUCA1A c.124T>A p.F42I, GUCA1A(NM_001319061.1):c.124T>A (p.F42I) - GUCA1A_000016 number of normal cases with GUCA1A variant: 2; heterozygous, VKGL data sharing initiative Nederland PubMed: Mizobuchi 2019 - - CLASSIFICATION record, Unknown no - - - - VKGL-NL_Rotterdam
+/., +?/., -?/., ?/. 12 3 c.149C>T r.(?) p.(Pro50Leu) ACMG likely benign, likely pathogenic, likely pathogenic (dominant), pathogenic, VUS g.42141500C>T g.42173762C>T GUCA1A c.149C>T, p.(Pro50Leu), GUCA1A P50L, 2 more items - GUCA1A_000001 VKGL data sharing initiative Nederland, 2 more items PubMed: Boulanger-Scemama 2015, PubMed: Boulanger-Scemama 2019, PubMed: Downes 2001, PubMed: Wang 2017, 3 more items - rs104893968 CLASSIFICATION record, Germline ?, no, yes - - - - Global Variome, with Curator vacancy, Kornelia Neveling, VKGL-NL_Rotterdam, VKGL-NL_AMC
?/. 1 - c.152C>T r.(?) p.(Ser51Leu) - VUS g.42141503C>T - GUCA1A(NM_001319061.1):c.152C>T (p.S51L) - GUCA1A_000042 VKGL data sharing initiative Nederland - - - CLASSIFICATION record - - - - - VKGL-NL_Rotterdam
-?/. 1 - c.166G>T r.(?) p.(Val56Leu) - likely benign g.42141517G>T g.42173779G>T LOC118142757(NM_000409.5):c.166G>T (p.V56L) - GUCA1A_000003 VKGL data sharing initiative Nederland - - - CLASSIFICATION record - - - - - VKGL-NL_AMC
+?/. 1 - c.193T>G r.(?) p.(Phe65Val) - likely pathogenic g.42141544T>G g.42173806T>G - - GUCA1A_000024 - PubMed: Stone 2017 - - Germline - - - - - LOVD
-?/. 1 - c.201+11G>T r.(=) p.(=) - likely benign g.42141563G>T - LOC118142757(NM_001319061.2):c.201+11G>T - GUCA1A_000058 VKGL data sharing initiative Nederland - - - CLASSIFICATION record - - - - - VKGL-NL_AMC
-?/. 1 - c.202-16C>T r.(=) p.(=) - likely benign g.42146002C>T g.42178264C>T GUCA1A(NM_001384910.1):c.202-16C>T, LOC118142757(NM_000409.5):c.202-16C>T - GUCA1A_000004 VKGL data sharing initiative Nederland - - - CLASSIFICATION record - - - - - VKGL-NL_AMC
?/. 1 - c.204C>G r.(?) p.(Asp68Glu) ACMG VUS g.42146020C>G g.42178282C>G GUCA1A c.204C>G p.D68E - GUCA1A_000051 number of normal cases with GUCA1A variant: 1; heterozygous PubMed: Mizobuchi 2019 - - Unknown no - - - - LOVD
?/. 1 - c.212T>C r.(?) p.(Ile71Thr) - VUS g.42146028T>C g.42178290T>C - - GUCA1A_000010 VKGL data sharing initiative Nederland - - - CLASSIFICATION record - - - - - VKGL-NL_Nijmegen
-/. 1 - c.216T>C r.(?) p.(Asp72=) - benign g.42146032T>C g.42178294T>C LOC118142757(NM_000409.5):c.216T>C (p.D72=) - GUCA1A_000005 VKGL data sharing initiative Nederland - - - CLASSIFICATION record - - - - - VKGL-NL_AMC
?/. 1 - c.238C>A r.(?) p.(Leu80Ile) ACMG VUS g.42146054C>A g.42178316C>A GUCA1A c.238C>A p.L80I - GUCA1A_000052 number of normal cases with GUCA1A variant: 2; heterozygous PubMed: Mizobuchi 2019 - - Unknown no - - - - LOVD
+?/. 9 4 c.250C>T r.(?) p.(Leu84Phe) ACMG likely pathogenic, likely pathogenic (dominant) g.42146066C>T g.42178328C>T GUCA1A c.250C>T, p.L84F - GUCA1A_000043 ACMG PM2, PP2, PP5_STRONG, heterozygous PubMed: Hitti-Malin 2022, Journal: Hitti-Malin 2022, PubMed: Kamenarova_2013, PubMed: Weisschuh 2024, 1 more item - - Germline yes - - - - Johan den Dunnen, Rebekkah Hitti-Malin
+/. 3 - c.256G>C r.(?) p.(Gly86Arg) - pathogenic (dominant) g.42146072G>C g.42178334G>C GUCA1A c.256G -> C, G86R - GUCA1A_000053 1 more item PubMed: Peshenko 2019 - - Germline yes - - - - LOVD
+?/. 3 - c.265G>A r.(?) p.(Glu89Lys) ACMG likely pathogenic (dominant) g.42146081G>A g.42178343G>A GUCA1A c.265G>A (p.Glu89Lys), GUCA1A c.265G>A, p.Glu89Lys - GUCA1A_000039 ACMG PM2, PM1, PP2, PP5, heterozygous PubMed: Kitiratschky 2009, PubMed: Kohl 2012, PubMed: Weisschuh 2024 986883 - Germline, Germline/De novo (untested), Unknown ?, yes - - - - Johan den Dunnen
+/. 4 - c.295T>A r.(?) p.(Tyr99Asn) ACMG pathogenic (dominant) g.42146111T>A g.42178373T>A GUCA1A c.295 T>A (p.Y99N) - GUCA1A_000054 heterozygous PubMed: Mizobuchi 2019 - - Germline yes - - - - LOVD
+/. 2 - c.296A>C r.(?) p.(Tyr99Ser) ACMG pathogenic (dominant) g.42146112A>C g.42178374A>C GUCA1A c.296 A> C (p.Y99S) - GUCA1A_000055 heterozygous PubMed: Mizobuchi 2019 - - Germline yes - - - - LOVD
+/., +?/. 32 2, 4 c.296A>G r.(?) p.(Tyr99Cys) ACMG likely pathogenic, likely pathogenic (dominant), pathogenic, pathogenic (dominant) g.42146112A>G g.42178374A>G 6:42146112A>G ENST00000394237.1:c.296A>G (Tyr99Cys), c.296A>G, GUCA1A c.296A>G, p.Tyr99Cys, 1 more item - GUCA1A_000019 carries likely causative variants in more than one gene, heterozygous, 2 more items PubMed: Bryant 2018, PubMed: Carss 2017, PubMed: Downes 2001, PubMed: Liu-2020, PubMed: Payne 1998, 5 more items - rs104893967 De novo, Germline, Germline/De novo (untested) ?, yes - - - - Rebekkah Hitti-Malin
+?/. 3 - c.299A>G r.(?) p.(Asp100Gly) - likely pathogenic (dominant) g.42146115A>G g.42178377A>G GUCA1A c.299 A>G p.Asp100Gly (D100G) - GUCA1A_000044 heterozygous PubMed: Nong 2014 - - Germline yes - - - - LOVD
+?/. 2 - c.300T>A r.(?) p.(Asp100Glu) - likely pathogenic (dominant) g.42146116T>A g.42178378T>A GUCA1A c.300T>A (p.Asp100Glu), GUCA1A c.300T>A, Asp100Gln - GUCA1A_000040 error in annotation, should be p.(Asp100Glu); heterozygous, heterozygous PubMed: Kitiratschky 2009, PubMed: Kohl 2012 - - Germline yes - - - - LOVD
+?/. 3 - c.302_304delTAG r.(?) p.(Val101del) - likely pathogenic (dominant) g.42146118_42146120del g.42178380_42178382del GUCA1A c.302_304delTAG (p.Val101del) - GUCA1A_000045 heterozygous PubMed: Manes 2019 - - Germline yes - - - - LOVD
+?/. 1 - c.304G>C r.(?) p.(Asp102His) - likely pathogenic (dominant) g.42146120G>C g.42178382G>C - - GUCA1A_000028 - PubMed: Huang 2016 - - Germline - - - - - Johan den Dunnen
+?/. 1 - c.306T>A r.(?) p.(Asp102Glu) - likely pathogenic g.42146122T>A g.42178384T>A - - GUCA1A_000025 - PubMed: Stone 2017 - - Germline - - - - - LOVD
+/., +?/. 4 4 c.312C>A r.(?) p.(Asn104Lys) - likely pathogenic (dominant), pathogenic (recessive) g.42146128C>A g.42178390C>A 819_826del8, GUCA1A C312A, N104K - GUCA1A_000022 obsolete nucleotide annotation, extrapolated from protein and databases; heterozygous PubMed: Jiang 2008, PubMed: Wawrocka 2018 - - Germline yes - - - - LOVD
+?/. 1 - c.320T>C r.(?) p.(Ile107Thr) - likely pathogenic (dominant) g.42146136T>C g.42178398T>C GUCA1A c.320T>C, p.I107T - GUCA1A_000046 heterozygous PubMed: Kamenarova_2013 - - Germline yes - - - - LOVD
+/. 2 4 c.328_337del r.(?) p.(Asp110Serfs*18) ACMG pathogenic, pathogenic (!) g.42146144_42146153del g.42178406_42178415del 328_337delGATGAGCTGC - GUCA1A_000011 ACMG PVS1, PM2, PP4, dominant, reduced risk PubMed: Zenteno 2020 - - Germline - 1/143 cases - - - Johan den Dunnen, Juan Carlos Zenteno
+/. 1 - c.331G>A r.(?) p.(Glu111Lys) - pathogenic g.42146147G>A g.42178409G>A LOC118142757(NM_000409.5):c.331G>A (p.E111K) - GUCA1A_000017 VKGL data sharing initiative Nederland - - - CLASSIFICATION record - - - - - VKGL-NL_AMC
+/., +?/. 3 - c.332A>C r.(?) p.(Glu111Ala) - likely pathogenic, pathogenic (dominant) g.42146148A>C g.42178410A>C 6:42146148A>C ENST00000394237.1:c.332A>C (Glu111Ala), GUCA1A c.332A>C, p.Glu111Ala - GUCA1A_000020 heterozygous PubMed: Carss 2017, PubMed: Turro 2020 - - Germline, Germline/De novo (untested) ? - - - - LOVD
+/. 3 - c.332A>T r.(?) p.(Glu111Val) - pathogenic (dominant) g.42146148A>T g.42178410A>T GUCA1A c.332A>T; p.(Glu111Val) - E111V - GUCA1A_000049 1 more item PubMed: Marino 2018 - - Germline yes - - - - LOVD
+?/. 1 4 c.333G>C r.(?) p.(Glu111Asp) - likely pathogenic g.42146149G>C - c.333G>C - GUCA1A_000035 - PubMed: Maggi_2021 - - Germline yes - - - - LOVD
+/., +?/. 2 4 c.341C>T r.(?) p.(Thr114Ile) - likely pathogenic (dominant), pathogenic g.42146157C>T g.42178419C>T c.341C>T(p.T114I), GUCA1A c.341C>T, T114I - GUCA1A_000034 heterozygous PubMed: Nishiguchi_2004, PubMed: Wang 2016 - - Germline, Unknown ? - - - - LOVD
-?/. 1 - c.351+11C>T r.(=) p.(=) - likely benign g.42146178C>T - LOC118142757(NM_001319061.2):c.351+11C>T - GUCA1A_000031 VKGL data sharing initiative Nederland - - - CLASSIFICATION record - - - - - VKGL-NL_AMC
?/. 1 - c.359G>A r.(?) p.(Arg120His) - VUS g.42146547G>A g.42178809G>A - - GUCA1A_000030 0/1266 control chromosomes PubMed: Xu 2015 - - Germline - 1/314 case chromosomes - - - LOVD
+/., +?/. 4 5 c.359G>T r.(?) p.(Arg120Leu) ACMG likely pathogenic, likely pathogenic (dominant), pathogenic g.42146547G>T g.42178809G>T GUCA1A, variant 1: c.359G>T/p.R120L - GUCA1A_000036 ACMG PM2, PM1_SUPPORTING, PP2, PP5_STRONG, possibly solved, heterozygous PubMed: Hitti-Malin 2024, Journal: Hitti-Malin 2024, PubMed: Weisschuh 2020, PubMed: Weisschuh 2024 802212 - Germline, Unknown ? - - - - Johan den Dunnen, Rebekkah Hitti-Malin
+?/. 18 - c.359_360delinsTT r.(?) p.(Arg120Leu) - likely pathogenic (dominant) g.42146547_42146548delinsTT g.42178809_42178810delinsTT GUCA1A c.359_360delinsTT, p.R120L - GUCA1A_000047 1 more item PubMed: Chen 2017 - - Germline yes - - - - LOVD
?/. 1 - c.371C>T r.(?) p.(Pro124Leu) - VUS g.42146559C>T - GUCA1A(NM_001384910.1):c.371C>T (p.P124L) - GUCA1A_000064 VKGL data sharing initiative Nederland - - - CLASSIFICATION record - - - - - VKGL-NL_AMC
+?/. 1 - c.424A>G r.(?) p.(Lys142Glu) - likely pathogenic g.42146612A>G g.42178874A>G - - GUCA1A_000021 - PubMed: Taylor 2017 - - Germline - - - - - LOVD
+?/. 10 - c.428delinsACAC r.(?) p.(Ile143delinsAsnThr) - likely pathogenic, likely pathogenic (dominant) g.42146616delinsACAC g.42178878delinsACAC 428delTinsACAC, GUCA1A c.428delTinsACAC - GUCA1A_000026 no nucleotide annotation, extrapolated from protein and databases; heterozygous PubMed: Song 2018, PubMed: Stone 2017 - - Germline yes - - - - LOVD
+?/. 2 5 c.428delTinsACAC r.(?) p.(Ile143delinsAsnThr) - likely pathogenic (dominant) g.42146616delinsACAC g.42178878delinsACAC GUCA1A c.428delTinsACAC, I143NT - GUCA1A_000026 heterozygous PubMed: Nishiguchi_2004 - - Germline yes - - - - LOVD
+?/. 1 - c.428T>C r.(?) p.(Ile143Thr) - likely pathogenic g.42146616T>C g.42178878T>C GUCA1A c.429_430insACA; c.428T>C - GUCA1A_000032 no protein change given, compound heterozygous PubMed: Zanolli 2020 - - Unknown ? - - - - LOVD
+?/. 1 - c.429_430insACA r.(?) p.(Ile143_Asp144insThr) - likely pathogenic g.42146617_42146618insACA g.42178879_42178880insACA GUCA1A c.429_430insACA; c.428T>C - GUCA1A_000033 no protein change given, compound heterozygous PubMed: Zanolli 2020 - - Unknown ? - - - - LOVD
+?/. 1 5 c.430G>A r.(?) p.(Asp144Asn) ACMG likely pathogenic g.42146618G>A g.42178880G>A - - GUCA1A_000063 - PubMed: Hitti-Malin 2024, Journal: Hitti-Malin 2024 - - Germline - - - - - Rebekkah Hitti-Malin
?/. 1 - c.431A>C r.(?) p.(Asp144Ala) - VUS g.42146619A>C - LOC118142757(NM_001319061.2):c.431A>C (p.D144A) - GUCA1A_000059 VKGL data sharing initiative Nederland - - - CLASSIFICATION record - - - - - VKGL-NL_AMC
+/. 7 5 c.431A>G r.(?) p.(Asp144Gly) - pathogenic (dominant) g.42146619A>G g.42178881A>G GUCA1A c.431A>G, p.D144G - GUCA1A_000056 1 more item PubMed: Tang 2019 - - Germline yes - - - - LOVD
+?/. 2 - c.443A>C r.(?) p.(Asp148Ala) - likely pathogenic g.42146631A>C g.42178893A>C GUCA1A, variant 1: c.443A>C/p.D148A - GUCA1A_000037 possibly solved, heterozygous PubMed: Weisschuh 2020 - - Germline yes - - - - LOVD
+?/. 3 - c.444T>A r.(?) p.(Asp148Glu) - likely pathogenic (dominant) g.42146632T>A g.42178894T>A GUCA1A c.444T>A (p.Asp148Glu) - GUCA1A_000048 heterozygous PubMed: Manes 2019 - - Germline yes - - - - LOVD
-/. 1 - c.446-18C>A r.(=) p.(=) - benign g.42146963C>A g.42179225C>A LOC118142757(NM_001319061.2):c.446-18C>A - GUCA1A_000006 VKGL data sharing initiative Nederland - - - CLASSIFICATION record - - - - - VKGL-NL_AMC
+/., +?/. 43 5, 6 c.451C>T r.(?) p.(Leu151Phe) ACMG likely pathogenic, likely pathogenic (dominant), pathogenic (dominant) g.42146986C>T g.42179248C>T GUCA1A C451T, l L151F, GUCA1A c.451 C> T (p.L151F), GUCA1A c.451C>T (p.Leu151Phe), GUCA1A L151F, 2 more items - GUCA1A_000023 ACMG PM2, PP2, PP5_STRONG, heteozygous, heterozygous, 1 more item PubMed: Birtel 2018, PubMed: Gliem 2020, PubMed: Jiang 2005, PubMed: Kitiratschky 2009, 6 more items - - Germline, Unknown ?, yes - - - - Johan den Dunnen
+?/. 1 - c.464A>C r.(?) p.(Glu155Ala) - likely pathogenic (dominant) g.42146999A>C g.42179261A>C - - GUCA1A_000029 - PubMed: Huang 2013, PubMed: Huang 2016 - - Germline - - - - - Johan den Dunnen
+?/. 29 6 c.464A>G r.(?) p.(Glu155Gly) - likely pathogenic, likely pathogenic (dominant) g.42146999A>G g.42179261A>G GUCA1A A464G, E155G - GUCA1A_000027 heterozygous PubMed: Stone 2017, PubMed: Wilkie_2001 - - Germline yes - - - - LOVD
?/. 1 - c.465G>T r.(?) p.(Glu155Asp) - VUS g.42147000G>T - - - GUCA1A_000062 VKGL data sharing initiative Nederland - - - CLASSIFICATION record - - - - - VKGL-NL_Nijmegen
+?/. 1 6 c.475G>T r.(?) p.(Gly159Cys) - likely pathogenic (dominant) g.42147010G>T - c.475G>T - GUCA1A_000038 - PubMed: Liu-2020 - - Germline - - - - - LOVD
+?/. 3 - c.476G>T r.(?) p.(Gly159Val) - likely pathogenic (dominant) g.42147011G>T g.42179273G>T GUCA1A c.476G>T (p.Gly159Val), GUCA1A c.476G>T, p.Gly159Val - GUCA1A_000041 heterozygous PubMed: Kitiratschky 2009, PubMed: Kohl 2012 - - Germline yes - - - - LOVD
+?/. 1 - c.479T>G r.(?) (p.V160G) - likely pathogenic g.42147014T>G g.42179276T>G GUCA1A c.479T > G (p.V160G) - GUCA1A_000061 heterozygous PubMed: Abbas 2020 - - Unknown ? - - - - LOVD
+/., +?/. 20 6 c.526C>T r.(?) p.(Leu176Phe) - likely pathogenic, likely pathogenic (dominant), pathogenic (dominant) g.42147061C>T g.42179323C>T GUCA1A c.526C> T, p.L176F, GUCA1A c.526C>T, p.Leu176Phe, GUCA1A, variant 1: c.526C>T/p.L176F, 1 more item - GUCA1A_000007 heterozygous, solved, heterozygous, VKGL data sharing initiative Nederland PubMed: Birtel 2018, PubMed: Gliem 2020, PubMed: Vocke 2017, PubMed: Weisschuh 2016, 1 more item - - CLASSIFICATION record, Germline, Unknown ?, yes - - - - VKGL-NL_AMC
?/. 1 - c.551A>G r.(?) p.(Gln184Arg) ACMG VUS g.42147086A>G g.42179348A>G GUCA1A c.551A>G p.Q184R - GUCA1A_000057 number of normal cases with GUCA1A variant: 2; heterozygous PubMed: Mizobuchi 2019 - - Unknown no - - - - LOVD
-?/. 1 - c.567C>T r.(?) p.(Asp189=) - likely benign g.42147102C>T g.42179364C>T GUCA1A(NM_000409.4):c.567C>T (p.D189=) - GUCA1A_000008 VKGL data sharing initiative Nederland - - - CLASSIFICATION record - - - - - VKGL-NL_Rotterdam
?/. 1 6 c.598G>A r.(?) p.(Ala200Thr) - VUS g.42147133G>A g.42179395G>A - - GUCA1A_000002 - PubMed: Neveling 2012 - - Germline no - - - - Kornelia Neveling
-/. 1 - c.*10C>A r.(=) p.(=) - benign g.42147151C>A g.42179413C>A LOC118142757(NM_000409.5):c.*10C>A - GUCA1A_000009 VKGL data sharing initiative Nederland - - - CLASSIFICATION record - - - - - VKGL-NL_AMC
-?/. 1 - c.*5445G>A r.(=) p.(=) - likely benign g.42152586G>A g.42184848G>A GUCA1B(NM_002098.5):c.570C>T (p.L190=) - GUCA1B_000004 VKGL data sharing initiative Nederland - - - CLASSIFICATION record - - - - - VKGL-NL_Rotterdam
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