Unique variants in the GJB6 gene

Information The variants shown are described using the NM_006783.4 transcript reference sequence.

41 entries on 1 page. Showing entries 1 - 41.
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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     
+/+, +/., +?/. 15 _1_3 c.-553_443{0} r.0, r.0? p.0, p.0? - pathogenic, pathogenic (recessive) g.20797177_21105945del g.20223038_20531806del c.-301126_443del, del(GJB6-D13S1830), c.-301126_443del - GJB6_000016 309 kb deletion; not in 400 control chromosomes, 342 kb deletion, 1 more item PubMed: Dalamon 2013, PubMed: del Castillo 2002, PubMed: del Castillo 2005, 2 more items - - Germline yes 1/210 cases (no GJB2 variant), 1/476 cases, 2/3 cases, 2/476 cases, 20/30 cases, 4/476 cases - - - Johan den Dunnen, Viviana Karina Dalamón
+/. 3 _1_3 c.-553_443{0} r.? p.? - pathogenic g.20797177_21105945del g.20223038_20531806del del(GJB6-D13S1830), g.20222988_20531806del, g.20222988_20531806del - GJB6_000016 variant description published was not correct (D.Baux) PubMed: Baux 2017, Journal: Baux 2017 - - Germline, Germline/De novo (untested) yes - - - - David Baux
+/+, +?/. 9 _1_2i c.-553_-16+992{0} r.0? p.0? - pathogenic (recessive) g.20802727_21034768del g.20228588_20460629del del(GJB6-D13S1854), c.-229949_-16+992del - GJB6_000017 232 kb deletion, 232 kb deletion; fusion transcript CRYL1_GJB6 created? PubMed: Dalamon 2013, PubMed: del Castillo 2005, Journal: del Castillo 2005, OMIM:var0006 - - Germline ? 1/16 cases, 1/47 cases, 1/53 cases, 10/47 cases, 3/476 cases, 4/18 cases - - - Johan den Dunnen, Viviana Karina Dalamón
+/+ 2 _1_2i c.-553_(-16+1_-15-1){0} r.0? p.0? - pathogenic g.(20797635_20803718)_(20805372_?)del - - - GJB6_000015 >140 kb deletion, >140 kb deletion; normal expression GJB2 gene on same chromosome PubMed: Lerer 2001, Journal: Lerer 2001 - - Germline yes - - - - Johan den Dunnen
?/. 4 - c.1A>G r.(?) p.(Met1?) - VUS g.20797619T>C g.20223480T>C 1 more item - GJB6_000028 VKGL data sharing initiative Nederland - - - CLASSIFICATION record - - - - - VKGL-NL_Leiden, VKGL-NL_Rotterdam, VKGL-NL_Groningen, VKGL-NL_Nijmegen
-?/. 1 - c.6T>C r.(?) p.(Asp2=) - likely benign g.20797614A>G - GJB6(NM_006783.4):c.6T>C (p.D2=) - GJB6_000034 VKGL data sharing initiative Nederland - - - CLASSIFICATION record - - - - - VKGL-NL_Rotterdam
+/. 1 3 c.14C>T r.(?) p.(Thr5Met) - pathogenic g.20797606G>A g.20223467G>A - - GJB6_000003 - PubMed: Grifa 1999, OMIM:var0001 - - Germline yes - - - - Connexin-deafness
+/. 11 3 c.31G>A r.(?) p.(Gly11Arg) - pathogenic g.20797589C>T g.20223450C>T GJB6(NM_006783.5):c.31G>A (p.G11R) - GJB6_000002 VKGL data sharing initiative Nederland PubMed: Lamartine 2000, OMIM:var0002 - rs104894415 CLASSIFICATION record, Germline yes - - - - Johan den Dunnen, VKGL-NL_Groningen, VKGL-NL_Nijmegen
-?/., ?/. 3 - c.61G>A r.(?) p.(Gly21Arg) - likely benign, VUS g.20797559C>T g.20223420C>T GJB6(NM_001110219.3):c.61G>A (p.G21R) - GJB6_000020 VKGL data sharing initiative Nederland PubMed: Sommen 2016, Journal: Sommen 2016 - - CLASSIFICATION record, Germline - - - - - Manou Sommen, VKGL-NL_Rotterdam, VKGL-NL_Nijmegen
+/+, +/. 2 3 c.63del r.(?) p.(Lys22Argfs*13) - pathogenic g.20797559del g.20223420del - - GJB6_000010 - Pandya, PC, PubMed: Gardner 2006 - - Unknown - - - - - Julia Lopez, Connexin-deafness
+/. 1 3 c.110T>A r.(?) p.(Val37Glu) - pathogenic g.20797510A>T g.20223371A>T - - GJB6_000013 - PubMed: Smith 2002, OMIM:var0005 - - Unknown - - - - - Julia Lopez
?/. 1 - c.110T>C r.(?) p.(Val37Ala) - VUS g.20797510A>G - - - GJB6_000039 - PubMed: Caengprasath 2023 - - Germline - - - - - Johan den Dunnen
-?/. 1 - c.111G>A r.(?) p.(Val37=) - likely benign g.20797509C>T g.20223370C>T GJB6(NM_001110219.3):c.111G>A (p.V37=) - GJB6_000027 VKGL data sharing initiative Nederland - - - CLASSIFICATION record - - - - - VKGL-NL_Rotterdam
-/- 1 3 c.117T>C r.(=) p.(Ala39=) - benign g.20797503A>G g.20223364A>G A39A - GJB6_000004 - PubMed: Kelley 1999 - - Unknown - - - - - Connexin-deafness
+?/. 1 3 c.119C>T r.(?) p.(Ala40Val) - likely pathogenic g.20797501G>A g.20223362G>A - - GJB6_000014 - PubMed: Yang 2007 - - Unknown - 1/260 cases - - - Julia Lopez
-?/. 1 - c.138C>T r.(?) p.(Asp46=) - likely benign g.20797482G>A - GJB6(NM_006783.4):c.138C>T (p.D46=) - GJB6_000033 VKGL data sharing initiative Nederland - - - CLASSIFICATION record - - - - - VKGL-NL_Rotterdam
+/. 1 3 c.148G>A r.(?) p.(Asp50Asn) - pathogenic g.20797472C>T g.20223333C>T - - GJB6_000012 - PubMed: Baris 2008 - - De novo yes - - - - Julia Lopez
?/. 1 - c.148G>T r.(?) p.(Asp50Tyr) - VUS g.20797472C>A - GJB6(NM_001110219.3):c.148G>T (p.D50Y) - GJB6_000032 VKGL data sharing initiative Nederland - - - CLASSIFICATION record - - - - - VKGL-NL_Groningen
-/- 1 3 c.153C>T r.(=) p.(Phe51=) - benign g.20797467G>A g.20223328G>A - - GJB6_000019 - - - rs185764408 Unknown - - - - - Johan den Dunnen
+/. 1 - c.159C>A r.(?) p.(Cys53Ter) - pathogenic g.20797461G>T g.20223322G>T GJB6(NM_001110219.3):c.159C>A (p.C53*) - GJB6_000022 VKGL data sharing initiative Nederland - - - CLASSIFICATION record - - - - - VKGL-NL_Rotterdam
-/- 1 3 c.174G>A r.(=) p.(Pro85=) - benign g.20797446C>T g.20223307C>T - - GJB6_000005 - PubMed: Kelley 1999 - - Unknown - - - - - Connexin-deafness
+/. 1 3 c.175G>C r.(?) p.(Gly59Arg) - pathogenic g.20797445C>G g.20223306C>G - - GJB6_000011 - PubMed: Nemoto-Hasebe 2009 - - Unknown - - - - - Julia Lopez
?/. 2 - c.209C>T r.(?) p.(Pro70Leu) - VUS g.20797411G>A g.20223272G>A GJB6(NM_006783.5):c.209C>T (p.P70L) - GJB6_000024 VKGL data sharing initiative Nederland - - - CLASSIFICATION record - - - - - VKGL-NL_Groningen, VKGL-NL_Nijmegen
?/. 2 - c.212T>C r.(?) p.(Val71Ala) - VUS g.20797408A>G g.20223269A>G GJB6(NM_006783.4):c.212T>C (p.V71A), GJB6(NM_006783.5):c.212T>C (p.V71A) - GJB6_000030 VKGL data sharing initiative Nederland - - - CLASSIFICATION record - - - - - VKGL-NL_Rotterdam, VKGL-NL_Groningen
?/. 1 - c.250G>A r.(?) p.(Val84Ile) - VUS g.20797370C>T - GJB6(NM_001110219.3):c.250G>A (p.(Val84Ile)) - GJB6_000040 VKGL data sharing initiative Nederland - - - CLASSIFICATION record - - - - - VKGL-NL_Leiden
?/. 1 - c.254C>T r.(?) p.(Ser85Phe) - VUS g.20797366G>A - GJB6(NM_006783.5):c.254C>T (p.S85F) - GJB6_000036 VKGL data sharing initiative Nederland - - - CLASSIFICATION record - - - - - VKGL-NL_Groningen
+/. 1 - c.263C>A r.(?) p.(Ala88Glu) - pathogenic g.20797357G>T g.20223218G>T - - GJB6_000023 VKGL data sharing initiative Nederland - - - CLASSIFICATION record - - - - - VKGL-NL_Nijmegen
+/. 5 3 c.263C>T r.(?) p.(Ala88Val) - pathogenic g.20797357G>A g.20223218G>A GJB6(NM_001110219.3):c.263C>T (p.A88V) - GJB6_000001 VKGL data sharing initiative Nederland PubMed: Lamartine 2000, OMIM:var0003, PubMed: Marakhonov 2012, {CV:SCV000026065} - rs28937872 CLASSIFICATION record, Germline yes - - - - Andrey Marakhonov, VKGL-NL_Groningen
?/. 1 - c.301G>A r.(?) p.(Glu101Lys) - VUS g.20797319C>T - GJB6(NM_006783.5):c.301G>A (p.E101K) - GJB6_000037 VKGL data sharing initiative Nederland - - - CLASSIFICATION record - - - - - VKGL-NL_Groningen
-?/. 1 - c.339T>A r.(?) p.(Asn113Lys) - likely benign g.20797281A>T g.20223142A>T GJB6(NM_001110219.3):c.339T>A (p.N113K) - GJB6_000026 VKGL data sharing initiative Nederland - - - CLASSIFICATION record - - - - - VKGL-NL_Rotterdam
-/- 1 3 c.396G>A r.(=) p.(L132=) - benign g.20797224C>T g.20223085C>T - - GJB6_000006 - PubMed: Gabriel 2001 - - Unknown - - - - - Connexin-deafness
-?/. 1 - c.405G>A r.(?) p.(Thr135=) - likely benign g.20797215C>T g.20223076C>T GJB6(NM_001110219.3):c.405G>A (p.T135=) - GJB6_000025 VKGL data sharing initiative Nederland - - - CLASSIFICATION record - - - - - VKGL-NL_Rotterdam
-?/- 1 3 c.415A>G r.(?) p.(Ser139Gly) - likely benign g.20797205T>C g.20223066T>C - - GJB6_000009 - PubMed: Kelley 1999 - - Unknown - - - - - Connexin-deafness
?/. 1 - c.425del r.(?) p.(Phe142Serfs*26) - VUS g.20797199del - GJB6(NM_001110219.3):c.425del (p.(Phe142Serfs*26)) - GJB6_000038 VKGL data sharing initiative Nederland - - - CLASSIFICATION record - - - - - VKGL-NL_Leiden
?/? 1 3 c.476A>G r.(?) p.(Asn159Ser) - VUS g.20797144T>C g.20223005T>C - - GJB6_000018 - - - rs35277762 Unknown - - - - - Johan den Dunnen
-/-, -?/. 2 3 c.489G>A r.(=), r.(?) p.(L163=), p.(Leu163=) - benign, likely benign g.20797131C>T g.20222992C>T GJB6(NM_001110219.3):c.489G>A (p.L163=), L163L - GJB6_000007 VKGL data sharing initiative Nederland PubMed: Kelley 1999 - rs35002004 CLASSIFICATION record, Unknown no - - - - Connexin-deafness, VKGL-NL_Rotterdam
-?/-, -?/. 3 3 c.595T>A r.(?) p.(Ser199Thr) - likely benign g.20797025A>T g.20222886A>T GJB6(NM_001110219.3):c.595T>A (p.S199T) - GJB6_000008 VKGL data sharing initiative Nederland PubMed: Kelley 1999 - - CLASSIFICATION record, Unknown - - - - - Connexin-deafness, VKGL-NL_Groningen, VKGL-NL_Nijmegen
-?/. 1 - c.607A>G r.(?) p.(Met203Val) - likely benign g.20797013T>C g.20222874T>C GJB6(NM_006783.4):c.607A>G (p.M203V) - GJB6_000031 VKGL data sharing initiative Nederland - - - CLASSIFICATION record - - - - - VKGL-NL_Rotterdam
?/. 1 - c.617A>C r.(?) p.(Asn206Thr) - VUS g.20797003T>G - GJB6(NM_006783.5):c.617A>C (p.N206T) - GJB6_000035 VKGL data sharing initiative Nederland - - - CLASSIFICATION record - - - - - VKGL-NL_Groningen
+?/. 1 - c.689del r.(?) p.(Asn230IlefsTer7) - likely pathogenic g.20796937del g.20222798del GJB6(NM_001110219.3):c.689delA (p.N230Ifs*7) - GJB6_000029 VKGL data sharing initiative Nederland - - - CLASSIFICATION record - - - - - VKGL-NL_Rotterdam
?/. 1 - c.689dup r.(?) p.(Asn230Lysfs*11) - VUS g.20796937dup - - - GJB6_000041 VKGL data sharing initiative Nederland - - - CLASSIFICATION record - - - - - VKGL-NL_Groningen
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