Unique variants in the GJB3 gene

Information The variants shown are described using the NM_024009.2 transcript reference sequence.

66 entries on 1 page. Showing entries 1 - 66.
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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     
-/- 2 1i c.-25-477_-25-474del r.(?) p.(=) - benign g.35249862_35249865del g.34784261_34784264del 632del4 - GJB3_000015 - PubMed: Lopez-Bigas 2000 - - Unknown ? 14/103 controls, 15/110 cases - - - Johan den Dunnen
-/- 2 1i c.-25-427_-25-426del r.(=) p.(=) - benign g.35249912_35249913del g.34784311_34784312del 581del2 - GJB3_000014 - PubMed: Lopez-Bigas 2000 - - Unknown ? 30/103 controls, 33/110 cases - - - Johan den Dunnen
?/. 1 - c.-25G>C r.(?) p.(=) - VUS g.35250339G>C - GJB3(NM_024009.2):c.-25G>C (p.?) - GJB3_000063 VKGL data sharing initiative Nederland - - - CLASSIFICATION record - - - - - VKGL-NL_Leiden
-?/. 1 - c.33C>T r.(?) p.(=) - likely benign g.35250396C>T - GJB3(NM_024009.3):c.33C>T (p.S11=) - GJB3_000057 VKGL data sharing initiative Nederland - - - CLASSIFICATION record - - - - - VKGL-NL_AMC
+/. 2 2 c.34G>A r.(?) p.(Gly12Ser) - pathogenic g.35250397G>A g.34784796G>A G12S - GJB3_000022 VKGL data sharing initiative Nederland PubMed: Richard 2001 - - CLASSIFICATION record, Unknown ? - - - - Michel van Geel, VKGL-NL_Nijmegen
+/. 1 2 c.34G>C r.(?) p.(Gly12Arg) - pathogenic g.35250397G>C g.34784796G>C - - GJB3_000003 - PubMed: Richard 1998, OMIM:var0001 - - Unknown ? - - - - Michel van Geel
+/. 1 2 c.35G>A r.(?) p.(Gly12Asp) - pathogenic g.35250398G>A g.34784797G>A - - GJB3_000004 - PubMed: Richard 1998, OMIM:var0002 - - Unknown ? - - - - Michel van Geel
?/. 1 - c.48C>A r.(?) p.(Tyr16Ter) - VUS g.35250411C>A g.34784810C>A - - GJB3_000045 VKGL data sharing initiative Nederland - - - CLASSIFICATION record - - - - - VKGL-NL_Nijmegen
?/. 1 - c.64C>T r.(?) p.(Arg22Cys) - VUS g.35250427C>T - GJB3(NM_024009.3):c.64C>T (p.(Arg22Cys)) - GJB3_000065 VKGL data sharing initiative Nederland - - - CLASSIFICATION record - - - - - VKGL-NL_Leiden
?/. 1 - c.69C>G r.(?) p.(Ile23Met) - VUS g.35250432C>G - GJB3(NM_024009.3):c.69C>G (p.(Ile23Met)) - GJB3_000066 VKGL data sharing initiative Nederland - - - CLASSIFICATION record - - - - - VKGL-NL_Leiden
?/. 1 - c.70T>C r.(?) p.(Trp24Arg) - VUS g.35250433T>C - - - GJB3_000056 VKGL data sharing initiative Nederland - - - CLASSIFICATION record - - - - - VKGL-NL_Nijmegen
+/. 1 2 c.76T>A r.(?) p.(ser26Thr) - pathogenic g.35250439T>A g.34784838T>A S26T - GJB3_000024 - PubMed: Richard 2001 - - Unknown ? - - - - Michel van Geel
?/. 1 - c.79G>A r.(?) p.(Val27Met) - VUS g.35250442G>A - GJB3(NM_024009.3):c.79G>A (p.V27M) - GJB3_000064 VKGL data sharing initiative Nederland - - - CLASSIFICATION record - - - - - VKGL-NL_Groningen
-/-, -/., -?/. 7 2 c.94C>T r.(?) p.(Arg32Trp) - benign, likely benign g.35250457C>T g.34784856C>T GJB3(NM_024009.3):c.94C>T (p.R32W) - GJB3_000011 VKGL data sharing initiative Nederland PubMed: Eisenberger 2018, PubMed: Kelsell 2000, PubMed: Lopez-Bigas 2000 - rs1805063 CLASSIFICATION record, Germline, Unknown ? 2/110 controls, 7/153 cases, 8/46 controls - - - Johan den Dunnen, VKGL-NL_Groningen, VKGL-NL_AMC
+/. 2 2 c.101T>C r.(?) p.(Leu34Pro) - pathogenic g.35250464T>C g.34784863T>C - - GJB3_000026 - PubMed: Gottfried 2002 - rs28937583 Unknown ? - - - - Michel van Geel
?/. 1 2 c.124C>T r.(?) p.(Arg42Cys) ACMG VUS g.35250487C>T - - - GJB3_000052 inherited from unaffected mother PubMed: Sampaio-Silva 2018 - rs976148533 Germline no - - - - Karina Lezirovitz Mandelbaum
+/. 3 2 c.125G>C r.(?) p.(Arg42Pro) - pathogenic g.35250488G>C g.34784887G>C - - GJB3_000006 - PubMed: Richard 1998, OMIM:var0008, PubMed: Richard 2000, PubMed: Wilgoss 1999, OMIM:var0008 - - Unknown ? - - - - Michel van Geel
+/. 1 2 c.134G>A r.(?) p.(Gly45Glu) - pathogenic g.35250497G>A g.34784896G>A G45E - GJB3_000023 - PubMed: Richard 2001 - - Unknown ? - - - - Michel van Geel
-?/. 1 - c.186C>T r.(?) p.(=) - likely benign g.35250549C>T - GJB3(NM_024009.3):c.186C>T (p.N62=) - GJB3_000059 VKGL data sharing initiative Nederland - - - CLASSIFICATION record - - - - - VKGL-NL_AMC
-?/. 1 - c.187G>A r.(?) p.(Val63Ile) - likely benign g.35250550G>A g.34784949G>A GJB3(NM_024009.2):c.187G>A (p.V63I) - GJB3_000047 VKGL data sharing initiative Nederland - - - CLASSIFICATION record - - - - - VKGL-NL_Rotterdam
+/., ?/. 2 2 c.196_198del r.(?) p.(Asp66del) - pathogenic, VUS g.35250559_35250561del g.34784958_34784960del GJB3(NM_024009.3):c.196_198del (p.(Asp66del)) - GJB3_000021 VKGL data sharing initiative Nederland PubMed: Lopez-Bigas 2001, OMIM:var0009 - - CLASSIFICATION record, Germline yes - - - - Michel van Geel, VKGL-NL_Leiden
?/. 1 - c.197A>G r.(?) p.(Asp66Gly) - VUS g.35250560A>G - GJB3(NM_024009.3):c.197A>G (p.D66G) - GJB3_000053 VKGL data sharing initiative Nederland - - - CLASSIFICATION record - - - - - VKGL-NL_Groningen
+?/., ?/. 2 - c.250G>A r.(?) p.(Val84Ile) - likely pathogenic (dominant), VUS g.35250613G>A g.34785012G>A c.G250A, GJB3(NM_024009.3):c.250G>A (p.(Val84Ile)) - GJB3_000036 rs145751680, VKGL data sharing initiative Nederland PubMed: Choi 2013 - - CLASSIFICATION record, Germline - - - - - Johan den Dunnen, VKGL-NL_Leiden
+/. 1 2 c.256T>A r.(?) p.(Cys86Ser) - pathogenic g.35250619T>A g.34785018T>A - - GJB3_000005 - PubMed: Richard 1998, OMIM:var0003 - - Unknown ? - - - - Michel van Geel
-?/. 2 - c.264G>A r.(?) p.(Ser88=) - likely benign g.35250627G>A g.34785026G>A GJB3(NM_024009.3):c.264G>A (p.S88=) - GJB3_000048 VKGL data sharing initiative Nederland - - - CLASSIFICATION record - - - - - VKGL-NL_Utrecht, VKGL-NL_AMC
?/. 1 - c.268C>A r.(?) p.(Leu90Met) - VUS g.35250631C>A - GJB3(NM_024009.3):c.268C>A (p.(Leu90Met)) - GJB3_000067 VKGL data sharing initiative Nederland - - - CLASSIFICATION record - - - - - VKGL-NL_Leiden
-?/. 1 - c.290A>C r.(?) p.(Tyr97Ser) - likely benign g.35250653A>C g.34785052A>C - - GJB3_000046 VKGL data sharing initiative Nederland - - - CLASSIFICATION record - - - - - VKGL-NL_Nijmegen
?/. 1 - c.311G>A r.(?) p.(Arg104Gln) - VUS g.35250674G>A - GJB3(NM_024009.3):c.311G>A (p.R104Q) - GJB3_000055 VKGL data sharing initiative Nederland - - - CLASSIFICATION record - - - - - VKGL-NL_AMC
-/. 1 - c.313C>A r.(?) p.(His105Asn) - benign g.35250676C>A - GJB3(NM_024009.3):c.313C>A (p.H105N) - GJB3_000058 VKGL data sharing initiative Nederland - - - CLASSIFICATION record - - - - - VKGL-NL_AMC
-/-, -/. 3 2 c.357C>T r.(?) p.(=), p.(Asn119=) - benign g.35250720C>T g.34785119C>T GJB3(NM_024009.3):c.357C>T (p.N119=), N119N - GJB3_000012 VKGL data sharing initiative Nederland PubMed: Richard 2000 - - CLASSIFICATION record, Unknown ? 0.3 - - - Johan den Dunnen, VKGL-NL_Nijmegen, VKGL-NL_AMC
+/. 1 2 c.409T>C r.(?) p.(Phe137Leu) - pathogenic g.35250772T>C g.34785171T>C - - GJB3_000019 de novo, in patient PubMed: Richard 2000 - - De novo - - - - - Michel van Geel
+/. 2 2 c.421A>G r.(?) p.(Ile141Val) - pathogenic g.35250784A>G g.34785183A>G - - GJB3_000010 - PubMed: Liu 2000, OMIM:var0007 - - Unknown yes - - - - Michel van Geel
+/. 2 2 c.421_423del r.(?) p.(Ile141del) - pathogenic g.35250784_35250786del g.34785183_34785185del - - GJB3_000009 - PubMed: Liu 2000, OMIM:var0006 - - Unknown yes - - - - Michel van Geel
?/. 1 - c.422_423insCA r.(?) p.(Glu142Metfs*127) - VUS g.35250785_35250786insCA - GJB3(NM_024009.3):c.422_423insCA (p.(Glu142MetfsTer127)) - GJB3_000060 VKGL data sharing initiative Nederland - - - CLASSIFICATION record - - - - - VKGL-NL_Leiden
?/. 1 - c.424dup r.(?) p.(Glu142Glyfs*64) - VUS g.35250787dup - GJB3(NM_024009.3):c.424dup (p.(Glu142GlyfsTer64)) - GJB3_000061 VKGL data sharing initiative Nederland - - - CLASSIFICATION record - - - - - VKGL-NL_Leiden
?/. 1 - c.476C>T r.(?) p.(Pro159Leu) - VUS g.35250839C>T - GJB3(NM_024009.3):c.476C>T (p.(Pro159Leu)) - GJB3_000062 VKGL data sharing initiative Nederland - - - CLASSIFICATION record - - - - - VKGL-NL_Leiden
-/-, -/. 3 2 c.477G>A r.(?) p.(=), p.(Pro159=) - benign g.35250840G>A g.34785239G>A GJB3(NM_024009.3):c.477G>A (p.P159=), P159P - GJB3_000016 not in 92 control chromosomes, VKGL data sharing initiative Nederland PubMed: Lopez-Bigas 2000 - - CLASSIFICATION record, Unknown ? 24/153 cases, 6/46 controls - - - Johan den Dunnen, VKGL-NL_AMC
?/. 1 - c.478C>T r.(?) p.(Arg160Cys) - VUS g.35250841C>T - GJB3(NM_024009.3):c.478C>T (p.(Arg160Cys)) - GJB3_000068 VKGL data sharing initiative Nederland - - - CLASSIFICATION record - - - - - VKGL-NL_Leiden
-?/. 2 - c.479G>A r.(?) p.(Arg160His) - likely benign g.35250842G>A g.34785241G>A GJB3(NM_024009.3):c.479G>A (p.R160H) - GJB3_000037 VKGL data sharing initiative Nederland - - - CLASSIFICATION record - - - - - VKGL-NL_Utrecht, VKGL-NL_AMC
+/. 1 2 c.497A>G r.(?) p.(Asn166Ser) - pathogenic g.35250860A>G g.34785259A>G - - GJB3_000033 GJB2 1 bp deletion; possible di-genic inheritance PubMed: Liu 2009 - - Unknown ? - - - - Michel van Geel
-/. 1 - c.529T>G r.(?) p.(Tyr177Asp) - benign g.35250892T>G - GJB3(NM_024009.3):c.529T>G (p.Y177D) - GJB3_000031 VKGL data sharing initiative Nederland - - - CLASSIFICATION record - - - - - VKGL-NL_AMC
+/. 1 2 c.538C>T r.(?) p.(Arg180*) - pathogenic g.35250901C>T g.34785300C>T - - GJB3_000008 - PubMed: Xia 1998, OMIM:var0005 - - Germline yes - - - - Michel van Geel
+/. 1 2 c.547G>A r.(?) p.(Glu183Lys) - pathogenic g.35250910G>A g.34785309G>A - - GJB3_000007 - PubMed: Xia 1998, OMIM:var0004 - - Germline yes - - - - Michel van Geel
-/-, -/. 3 2 c.567C>T r.(?) p.(=), p.(Tyr189=) - benign g.35250930C>T g.34785329C>T GJB3(NM_024009.3):c.567C>T (p.Y189=), Y189Y - GJB3_000017 not in 92 control chromosomes, VKGL data sharing initiative Nederland PubMed: Lopez-Bigas 2000 - - CLASSIFICATION record, Unknown ? 1/110 cases, 3/153 cases - - - Johan den Dunnen, VKGL-NL_AMC
-/. 2 - c.579C>T r.(?) p.(Gly193=) - benign g.35250942C>T g.34785341C>T GJB3(NM_024009.2):c.579C>T (p.G193=), GJB3(NM_024009.3):c.579C>T (p.G193=) - GJB3_000049 VKGL data sharing initiative Nederland - - - CLASSIFICATION record - - - - - VKGL-NL_Rotterdam, VKGL-NL_AMC
+/., -/., ?/. 3 2 c.580G>A r.(?) p.(Ala194Thr) - benign, pathogenic, VUS g.35250943G>A g.34785342G>A GJB3(NM_024009.2):c.580G>A (p.(Ala194Thr)), GJB3(NM_024009.3):c.580G>A (p.A194T) - GJB3_000034 GJB2 1 bp deletion; possible di-genic inheritance, VKGL data sharing initiative Nederland PubMed: Liu 2009 - - CLASSIFICATION record, Unknown ? - - - - Michel van Geel, VKGL-NL_Leiden, VKGL-NL_AMC
?/. 1 - c.586G>A r.(?) p.(Ala196Thr) - VUS g.35250949G>A g.34785348G>A GJB3(NM_024009.3):c.586G>A (p.A196T) - GJB3_000038 VKGL data sharing initiative Nederland - - - CLASSIFICATION record - - - - - VKGL-NL_AMC
-/- 2 2 c.597G>A r.(?) p.(Val200Ile) - benign g.35250960G>A g.34785359G>A - - GJB3_000018 1 more item PubMed: Lopez-Bigas 2000 - - Unknown ? 1/110 cases, 3/153 cases - - - Johan den Dunnen
-?/. 1 - c.598G>A r.(?) p.(Val200Ile) - likely benign g.35250961G>A g.34785360G>A GJB3(NM_024009.3):c.598G>A (p.V200I) - GJB3_000039 VKGL data sharing initiative Nederland - - - CLASSIFICATION record - - - - - VKGL-NL_AMC
+/. 1 2 c.625C>T r.(?) p.(Leu209Phe) - pathogenic g.35250988C>T g.34785387C>T L209F - GJB3_000025 - PubMed: Richard 2001 - - Unknown ? - - - - Michel van Geel
+/. 1 2 c.652_663del r.(?) p.(Leu218_Asp221del) - pathogenic g.35251015_35251026del g.34785414_34785425del - - GJB3_000020 - PubMed: Richard 2000 - - Unknown ? - - - - Michel van Geel
+/. 2 2 c.667C>A r.(?) p.(Pro223Thr) - pathogenic g.35251030C>A g.34785429C>A GJB3(NM_024009.2):c.667C>A (p.(Pro223Thr)) - GJB3_000027 VKGL data sharing initiative Nederland PubMed: Uyguner 2003 - - CLASSIFICATION record, Unknown yes - - - - Michel van Geel, VKGL-NL_Leiden
?/. 1 - c.670C>T r.(?) p.(Arg224*) - VUS g.35251033C>T - - - GJB3_000054 VKGL data sharing initiative Nederland - - - CLASSIFICATION record - - - - - VKGL-NL_Nijmegen
-?/. 1 - c.697G>A r.(?) p.(Ala233Thr) - likely benign g.35251060G>A g.34785459G>A GJB3(NM_024009.3):c.697G>A (p.A233T) - GJB3_000040 VKGL data sharing initiative Nederland - - - CLASSIFICATION record - - - - - VKGL-NL_AMC
?/. 1 - c.764C>G r.(?) p.(Pro255Arg) - VUS g.35251127C>G g.34785526C>G GJB3(NM_024009.3):c.764C>G (p.P255R) - GJB3_000050 VKGL data sharing initiative Nederland - - - CLASSIFICATION record - - - - - VKGL-NL_AMC
-?/. 1 - c.778C>T r.(?) p.(Leu260=) - likely benign g.35251141C>T g.34785540C>T GJB3(NM_024009.2):c.778C>T (p.L260=) - GJB3_000051 VKGL data sharing initiative Nederland - - - CLASSIFICATION record - - - - - VKGL-NL_Rotterdam
-/., -?/? 2 2 c.798C>T r.(?) p.(=), p.(Asn266=) - benign, likely benign g.35251161C>T g.34785560C>T 801C>T (N267N), GJB3(NM_024009.3):c.798C>T (p.N266=) - GJB3_000028 VKGL data sharing initiative Nederland PubMed: Uyguner 2003 - - CLASSIFICATION record, Unknown yes - - - - Michel van Geel, VKGL-NL_AMC
-/- 4 2 c.798G>T r.(?) p.(=) - benign g.35251161G>T g.34785560G>T 1931C>T (N266N), N266N - GJB3_000013 1 more item PubMed: Lopez-Bigas 2000, PubMed: Richard 2000 - - Unknown ? 0.1, 1/110 cases, 3/153 cases, 6/110 cases - - - Johan den Dunnen
-?/. 1 - c.*13G>A r.(=) p.(=) - likely benign g.35251189G>A g.34785588G>A GJB3(NM_024009.3):c.*13G>A - GJB3_000041 VKGL data sharing initiative Nederland - - - CLASSIFICATION record - - - - - VKGL-NL_AMC
-/. 1 - c.*43C>A r.(=) p.(=) - benign g.35251219C>A g.34785618C>A GJB3(NM_024009.3):c.*43C>A - GJB3_000001 VKGL data sharing initiative Nederland - - - CLASSIFICATION record - - - - - VKGL-NL_AMC
-/- 1 2 c.856C>A r.(?) p.(=) - benign g.35251219C>A g.34785618C>A - - GJB3_000001 1 more item PubMed: Richard 1998 - - Unknown ? 0.24 - - - Johan den Dunnen
-/. 1 - c.*53G>A r.(=) p.(=) - benign g.35251229G>A g.34785628G>A GJB3(NM_024009.3):c.*53G>A - GJB3_000002 VKGL data sharing initiative Nederland - - - CLASSIFICATION record - - - - - VKGL-NL_AMC
-/- 1 2 c.866G>A r.(?) p.(=) - benign g.35251229G>A g.34785628G>A - - GJB3_000002 1 more item PubMed: Richard 1998 - - Unknown ? 0.34 - - - Johan den Dunnen
-/. 1 - c.*369G>C r.(=) p.(=) - benign g.35251545G>C g.34785944G>C GJB3(NM_024009.3):c.*369G>C - GJB3_000042 VKGL data sharing initiative Nederland - - - CLASSIFICATION record - - - - - VKGL-NL_AMC
-/. 1 - c.*493C>T r.(=) p.(=) - benign g.35251669C>T g.34786068C>T GJB3(NM_024009.3):c.*493C>T - GJB3_000043 VKGL data sharing initiative Nederland - - - CLASSIFICATION record - - - - - VKGL-NL_AMC
-/. 1 - c.*559G>C r.(=) p.(=) - benign g.35251735G>C g.34786134G>C GJB3(NM_024009.3):c.*559G>C - GJB3_000044 VKGL data sharing initiative Nederland - - - CLASSIFICATION record - - - - - VKGL-NL_AMC
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