Unique variants in the GJB2 gene

Information The variants shown are described using the NM_004004.5 transcript reference sequence.

132 entries on 2 pages. Showing entries 1 - 100.
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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 1 c.-216T>G r.(?) p.(=) - benign g.20767115A>C g.20192976A>C - - GJB2_000064 VKGL data sharing initiative Nederland - - - CLASSIFICATION record - - - - - VKGL-NL_Nijmegen
-?/. 1 1 c.-205T>G r.(?) p.(=) - likely benign g.20767104A>C g.20192965A>C - - GJB2_000063 VKGL data sharing initiative Nederland - - - CLASSIFICATION record - - - - - VKGL-NL_Nijmegen
-/. 1 1 c.-127G>C r.(?) p.(=) - benign g.20767026C>G g.20192887C>G - - GJB2_000062 VKGL data sharing initiative Nederland - - - CLASSIFICATION record - - - - - VKGL-NL_Nijmegen
-/., -?/. 2 1 c.-45C>A r.(?) p.(=) - benign, likely benign g.20766944G>T g.20192805G>T GJB2(NM_004004.6):c.-45C>A - GJB2_000048 VKGL data sharing initiative Nederland - - - CLASSIFICATION record - - - - - VKGL-NL_Rotterdam, VKGL-NL_Nijmegen
-?/. 1 1 c.-35C>T r.(?) p.(=) - likely benign g.20766934G>A g.20192795G>A - - GJB2_000061 VKGL data sharing initiative Nederland - - - CLASSIFICATION record - - - - - VKGL-NL_Nijmegen
+/., +?/. 9 1i c.-23+1G>A r.spl, r.spl? p.? - likely pathogenic, likely pathogenic (recessive), pathogenic, pathogenic (recessive) g.20766921C>T g.20192782C>T GJB2(NM_004004.6):c.-23+1G>A - GJB2_000011 12 heterozygous, no homozygous; Clinindb (India), ACMG PVS1, PP5, PM2, BP4, 1 more item PubMed: Baux 2017, Journal: Baux 2017, PubMed: Dalamon 2013, PubMed: Narang 2020, Journal: Narang 2020, 3 more items - rs80338940 CLASSIFICATION record, Germline, Germline/De novo (untested) - 1/476 cases, 12/2787 individuals - - - Johan den Dunnen, David Baux, Mieke Wesdorp, VKGL-NL_Rotterdam, VKGL-NL_Nijmegen, Viviana Karina Dalamón, Mohammed Faruq
-?/. 1 1i c.-23+12G>A r.(=) p.(=) - likely benign g.20766910C>T g.20192771C>T - - GJB2_000060 VKGL data sharing initiative Nederland - - - CLASSIFICATION record - - - - - VKGL-NL_Nijmegen
-/. 1 1 c.-22-12C>T r.(=) p.(=) - benign g.20763754G>A g.20189615G>A - - GJB2_000059 VKGL data sharing initiative Nederland - - - CLASSIFICATION record - - - - - VKGL-NL_Nijmegen
-?/. 2 1i c.-22-6T>C r.(=) p.(=) - likely benign g.20763748A>G g.20189609A>G GJB2(NM_004004.6):c.-22-6T>C - GJB2_000047 VKGL data sharing initiative Nederland - - - CLASSIFICATION record - - - - - VKGL-NL_Rotterdam, VKGL-NL_Nijmegen
+?/. 3 1, 2 c.-22-2A>C r.spl, r.spl? p.0?, p.? - likely pathogenic, pathogenic (recessive) g.20763744T>G g.20189605T>G GJB2(NM_004004.6):c.-22-2A>C - GJB2_000058 VKGL data sharing initiative Nederland - 551912 rs201895089 CLASSIFICATION record, Germline ? - - - - VKGL-NL_Leiden, VKGL-NL_Nijmegen, Viviana Karina Dalamón
-/. 1 - c.-15C>T r.(?) p.(=) - benign g.20763735G>A g.20189596G>A - - GJB2_000096 VKGL data sharing initiative Nederland - - - CLASSIFICATION record - - - - - VKGL-NL_Nijmegen
-/. 1 2 c.-6T>A r.(?) p.(=) - benign g.20763726A>T g.20189587A>T - - GJB2_000057 VKGL data sharing initiative Nederland - - - CLASSIFICATION record - - - - - VKGL-NL_Nijmegen
-?/. 1 - c.-1G>A r.(?) p.(=) - likely benign g.20763721C>T - GJB2(NM_004004.5):c.-1G>A (p.?) - GJB2_000131 VKGL data sharing initiative Nederland - - - CLASSIFICATION record - - - - - VKGL-NL_Leiden
+/., +?/. 3 2 c.1A>G r.(?) p.(Met1?), p.? - likely pathogenic, pathogenic g.20763720T>C g.20189581T>C GJB2(NM_004004.6):c.1A>G (p.(Met1?)) - GJB2_000031 5 heterozygous, no homozygous; Clinindb (India), VKGL data sharing initiative Nederland PubMed: Baux 2017, Journal: Baux 2017, PubMed: Narang 2020, Journal: Narang 2020 - rs111033293 CLASSIFICATION record, Germline, Germline/De novo (untested) - 5/2793 individuals - - - David Baux, VKGL-NL_Leiden, Mohammed Faruq
-?/. 3 - c.11G>A r.(?) p.(Gly4Asp) - likely benign g.20763710C>T g.20189571C>T GJB2(NM_004004.6):c.11G>A (p.G4D) - GJB2_000046 VKGL data sharing initiative Nederland - - - CLASSIFICATION record - - - - - VKGL-NL_Rotterdam, VKGL-NL_Groningen, VKGL-NL_Nijmegen
+?/. 1 - c.16C>G r.(?) p.(Leu6Val) - likely pathogenic g.20763705G>C g.20189566G>C - - GJB2_000106 VKGL data sharing initiative Nederland - - - CLASSIFICATION record - - - - - VKGL-NL_Nijmegen
+?/., ?/. 2 2 c.23C>T r.(?) p.(Thr8Met), p.(Trp8Met) - pathogenic (recessive), VUS g.20763698G>A g.20189559G>A GJB2(NM_004004.5):c.23C>T (p.(Thr8Met)) - GJB2_000084 VKGL data sharing initiative Nederland PubMed: Dalamon 2013 - - CLASSIFICATION record, Germline - 1/476 cases - - - VKGL-NL_Leiden, Viviana Karina Dalamón
-?/. 2 2 c.24G>A r.(=), r.(?) p.(=), p.(Thr8=) - likely benign g.20763697C>T g.20189558C>T - - GJB2_000075 VKGL data sharing initiative Nederland PubMed: Dalamon 2013 - - CLASSIFICATION record, Germline - 1/74 cases - - - VKGL-NL_Nijmegen, Viviana Karina Dalamón
+/. 1 - c.29T>C r.(?) p.(Leu10Pro) - pathogenic (dominant) g.20763692A>G g.20189553A>G - - GJB2_000077 - PubMed: Dalamon 2013 - - Germline - - - - - Viviana Karina Dalamón
+/. 1 - c.31_68del r.(?) p.(Gly11Leufs*24) ACMG pathogenic g.20763653_20763690del g.20189514_20189551del - - GJB2_000055 1 more item - - rs397516873 Germline - - - - - Andreas Laner
+?/. 1 - c.32G>A r.(?) p.(Gly11Glu) ACMG likely pathogenic g.20763689C>T g.20189550C>T - - GJB2_000016 - PubMed: Trujillano 2017 - - De novo - - - - - Daniel Trujillano
+/+, +/., +?/+, +?/., ./. 130 2 c.35del r.(35del), r.(?), r.35del p.(Gly12Valfs*2), p.(Gly12ValfsTer2), p.Gly12Valfs*2 ACMG likely pathogenic, likely pathogenic (recessive), pathogenic, pathogenic (recessive) g.20763686del, g.20763691del g.20189552del 35delG, c.35delG, g.8429delG, 1 more item - GJB2_000001, GJB2_000007 ACMG PM2, PVS1, PP5, ACMG PVS1, PS3, PP3, PP5, ACMG PVS1, PS3, PP5, PP3, no variant 2nd chromosome, 2 more items {CV:94392}, PubMed: Batissoco 2021, PubMed: Baux 2017, Journal: Baux 2017, PubMed: Boucher 2020, 14 more items ClinVar-32043 rs1801002, rs80338939 CLASSIFICATION record, Germline, Germline/De novo (untested), Unknown ?, yes 1/476 cases, 2/476 cases, 26/476 cases, 3/476 cases, 4/476 cases - - - Johan den Dunnen, David Baux, Atteeq Rehman, Mieke Wesdorp, VKGL-NL_Leiden, VKGL-NL_Rotterdam, Jan Traeger-Synodinos, VKGL-NL_Groningen, Viviana Karina Dalamón, Anaïs Begemann, MobiDetails, Karina Lezirovitz Mandelbaum
+/. 1 - c.35delG r.(?) p.(Gly12Valfs*2) - pathogenic g.20763691del g.20189552del GJB2 c.35delG, p.(Gly12Valfs*2) - GJB2_000001 - PubMed: Niepokoj 2018 - - Germline yes - - - - LOVD
+/+ 1 2 c.35dup r.(?) p.(Val13Cysfs*35) - pathogenic g.20763691dup g.20189552dup 35insG - GJB2_000005 - {PMID:Estivill 1998:09482292:}, Journal: Estivill 1998 - - Germline yes - - - - Johan den Dunnen
+?/. 3 2 c.35G>T r.(?) p.(Gly12Val) - likely pathogenic, pathogenic (recessive) g.20763686C>A g.20189547C>A - - GJB2_000083 VKGL data sharing initiative Nederland PubMed: Dalamon 2013 - - CLASSIFICATION record, Germline - 1/476 cases - - - VKGL-NL_Nijmegen, Viviana Karina Dalamón
./. 1 - c.38_39del r.(?) p.(Val13Glufs*34) - likely pathogenic g.20763685_20763686del g.20189546_20189547del NM_004004.5(GJB2):c.38_39del p.(Val13Glufs*34) - GJB2_000017 variant could not be associated with disease phenotype PubMed: Vogelaar 2017, Journal: Vogelaar 2017 - - Germline - - - - - Marjolijn JL Ligtenberg
?/. 1 - c.44A>C r.(?) p.(Lys15Thr) - VUS g.20763677T>G g.20189538T>G - - GJB2_000056 VKGL data sharing initiative Nederland - - - CLASSIFICATION record - - - - - VKGL-NL_Nijmegen
+?/. 1 2 c.56G>C r.(?) p.(Ser19Thr) - pathogenic (recessive) g.20763665C>G g.20189526C>G - - GJB2_000082 - PubMed: Dalamon 2013 - - Germline - 1/476 cases - - - Viviana Karina Dalamón
+/+, +/., +?/+ 44 2 c.71G>A r.(?) p.(Trp24*), p.(Trp24Ter) ACMG likely pathogenic, pathogenic, pathogenic (recessive) g.20763650C>T g.20189511C>T c.71G>A (p.Trp24*), GJB2(NM_004004.6):c.71G>A (p.W24*, p.(Trp24Ter)) - GJB2_000003 32 heterozygous, no homozygous; Clinindb (India), ACMG PVS1, PM1, PP3, PP5, 1 more item PubMed: Baux 2017, Journal: Baux 2017, PubMed: Falk 2012, Journal: Falk 2012, PubMed: Hull-2016, 6 more items - rs104894396 CLASSIFICATION record, Germline, Germline/De novo (untested) yes 32/2795 individuals - - - Johan den Dunnen, David Baux, Atteeq Rehman, Mieke Wesdorp, VKGL-NL_Leiden, VKGL-NL_Rotterdam, Jan Traeger-Synodinos, Anju Shukla, Mohammed Faruq
-/., -?/. 13 2 c.79G>A r.(?) p.(Val27Ile) - benign, likely benign g.20763642C>T g.20189503C>T g.8473G>A, GJB2(NM_004004.6):c.79G>A (p.V27I) - GJB2_000045 10 homozygous; Clinindb (India), 121 heterozygous; Clinindb (India), 2 more items PubMed: Dalamon 2013, PubMed: Narang 2020, Journal: Narang 2020 36279 rs2274084 CLASSIFICATION record, Germline, Germline/De novo (untested) ?, no 0.04538, 10/2795 individuals, 121/2795 individuals, 29/125 cases non-syndromic deafness, 2 more items - - - VKGL-NL_Rotterdam, VKGL-NL_Groningen, VKGL-NL_Nijmegen, Viviana Karina Dalamón, Mohammed Faruq, Guillermina García Sánchez
+?/. 1 2 c.79_82delinsAGA r.(?) p.(Val27Argfs*8) ACMG pathogenic g.20763639_20763642delinsTCT g.20189500_20189503delinsTCT - - GJB2_000116 - PubMed: Batissoco 2021 ClinVar-SCV001792212 - Germline ? - - - - Karina Lezirovitz Mandelbaum
-?/. 1 - c.84C>T r.(?) p.(Leu28=) - likely benign g.20763637G>A g.20189498G>A - - GJB2_000101 VKGL data sharing initiative Nederland - - - CLASSIFICATION record - - - - - VKGL-NL_Nijmegen
+?/. 1 - c.94C>G r.(?) p.(Arg32Gly) - likely pathogenic g.20763627G>C - - - GJB2_000117 - - - - CLASSIFICATION record - - - - - MobiDetails
+/. 13 - c.94C>T r.(?) p.(Arg32Cys) ACMG pathogenic (recessive) g.20763627G>A g.20189488G>A - - GJB2_000126 - PubMed: Dia 2022 - - Germline yes 2/148 controls - - - Yacouba Dia
+/., +?/. 3 2 c.95G>A r,(?), r.(?) p.(Arg32His) - likely pathogenic, pathogenic, pathogenic (recessive) g.20763626C>T g.20189487C>T - - GJB2_000032 3 heterozygous, no homozygous; Clinindb (India), combination of alleles not reported PubMed: Baux 2017, Journal: Baux 2017, PubMed: Narang 2020, Journal: Narang 2020, PubMed: Wu 2019 - rs111033190 Germline yes 1/1291 cases hearing loss, 3/2795 individuals - - - Johan den Dunnen, David Baux, Mohammed Faruq
+/., +?/. 21 2 c.101T>C r.(?) p.(Met34Thr) - likely pathogenic, pathogenic, pathogenic (recessive) g.20763620A>G g.20189481A>G GJB2(NM_004004.6):c.101T>C (p.M34T, p.(Met34Thr)) - GJB2_000012 VKGL data sharing initiative Nederland PubMed: Baux 2017, Journal: Baux 2017, PubMed: Boucher 2020, PubMed: Dalamon 2013, PubMed: Papuc 2019, 2 more items - rs35887622 CLASSIFICATION record, Germline, Germline/De novo (untested) yes 1/476 cases, 2/476 cases - - - Johan den Dunnen, David Baux, Mieke Wesdorp, VKGL-NL_Leiden, VKGL-NL_Rotterdam, VKGL-NL_Groningen, VKGL-NL_Nijmegen, Viviana Karina Dalamón, Anaïs Begemann, MobiDetails
+?/. 1 - c.102G>A r.(?) p.(Met34Ile) - likely pathogenic g.20763619C>T g.20189480C>T - - GJB2_000095 VKGL data sharing initiative Nederland - - - CLASSIFICATION record - - - - - VKGL-NL_Nijmegen
+/., ?/. 2 - c.107T>C r,(?), r.(?) p.(Leu36Pro) - pathogenic (recessive), VUS g.20763614A>G g.20189475A>G - - GJB2_000104 4 heterozygous, no homozygous; Clinindb (India), combination of alleles not reported PubMed: Narang 2020, Journal: Narang 2020, PubMed: Wu 2019 - rs587783644 Germline - 1/1291 cases hearing loss, 4/2795 individuals - - - Johan den Dunnen, Mohammed Faruq
+/., +?/., -?/. 29 2 c.109G>A r,(?), r.(?) p.(Val37Ile) - likely benign, likely pathogenic, likely pathogenic (recessive), pathogenic, pathogenic (recessive) g.20763612C>T g.20189473C>T GJB2(NM_004004.6):c.109G>A (p.V37I, p.(Val37Ile)), V37I - GJB2_000013 1 heterozygous, no homozygous; Clinindb (India), combination of alleles not reported, 2 more items PubMed: Baux 2017, Journal: Baux 2017, PubMed: Dalamon 2013, PubMed: del Castillo 2005, 9 more items - rs72474224 CLASSIFICATION record, Germline, Germline/De novo (untested) yes 1/2792 individuals, 1/476 cases, 1608/1291 cases hearing loss, frequency 0.096 - - - Global Variome, with Curator vacancy, Johan den Dunnen, David Baux, Mieke Wesdorp, VKGL-NL_Leiden, VKGL-NL_Rotterdam, VKGL-NL_Groningen, VKGL-NL_Nijmegen, VKGL-NL_VUmc, Viviana Karina Dalamón, Mohammed Faruq
+?/. 1 2 c.119C>A r.(?) p.(Ala40Glu) - likely pathogenic g.20763602G>T g.20189463G>T - - GJB2_000027 - PubMed: Baux 2017, Journal: Baux 2017 - rs111033296 Germline/De novo (untested) - - - - - David Baux
+?/. 1 - c.119C>G r.(?) p.(Ala40Gly) - likely pathogenic g.20763602G>C g.20189463G>C - - GJB2_000105 VKGL data sharing initiative Nederland - - - CLASSIFICATION record - - - - - VKGL-NL_Nijmegen
?/. 1 - c.126G>T r.(?) p.(Glu42Asp) - VUS g.20763595C>A g.20189456C>A - - GJB2_000094 VKGL data sharing initiative Nederland - - - CLASSIFICATION record - - - - - VKGL-NL_Nijmegen
?/. 1 2 c.128T>G r.(?) p.(Val43Gly) - VUS g.20763593A>C g.20189454A>C - - GJB2_000009 - PubMed: Sommen 2016, Journal: Sommen 2016 - - Germline - - - - - Manou Sommen
+/. 3 2 c.132G>A r.(?) p.(p.(Trp44Ter), p.(Trp44Ter) ACMG pathogenic, pathogenic (recessive) g.20763589C>T g.20189450C>T - - GJB2_000093 VKGL data sharing initiative Nederland PubMed: Dia 2022 - - CLASSIFICATION record, Germline yes - - - - VKGL-NL_Nijmegen, Yacouba Dia
?/. 1 - c.136G>T r.(?) p.(Asp46Tyr) - VUS g.20763585C>A - GJB2(NM_004004.6):c.136G>T (p.D46Y) - GJB2_000115 VKGL data sharing initiative Nederland - - - CLASSIFICATION record - - - - - VKGL-NL_Rotterdam
+/., +?/. 9 2 c.139G>T r.(?) p.(Glu47*), p.(Glu47Ter) - pathogenic, pathogenic (recessive) g.20763582C>A g.20189443C>A GJB2(NM_004004.6):c.139G>T (p.E47*) - GJB2_000028 VKGL data sharing initiative Nederland PubMed: Baux 2017, Journal: Baux 2017, PubMed: Dalamon 2013 - rs104894398 CLASSIFICATION record, Germline, Germline/De novo (untested) - 1/476 cases, 2/476 cases - - - David Baux, VKGL-NL_Rotterdam, VKGL-NL_VUmc, Viviana Karina Dalamón
+/. 3 - c.148G>A r.(?) p.(Asp50Asn) ACMG pathogenic, pathogenic (dominant) g.20763573C>T g.20189434C>T - - GJB2_000054 0/60 healthy controls from Cameroon, VKGL data sharing initiative Nederland PubMed: Wonkam 2013 - - CLASSIFICATION record, Germline/De novo (untested) ? - - - - VKGL-NL_Nijmegen, Yacouba Dia
-?/. 1 - c.156C>T r.(?) p.(Val52=) - likely benign g.20763565G>A g.20189426G>A - - GJB2_000100 VKGL data sharing initiative Nederland - - - CLASSIFICATION record - - - - - VKGL-NL_Nijmegen
+/+, +?/. 13 2 c.167del r.(?) p.(Leu56Argfs*26) - pathogenic, pathogenic (recessive) g.20763554del g.20189415del 167delT - GJB2_000006 - PubMed: Dalamon 2013, PubMed: Lerer 2001, Journal: Lerer 2001 - - Germline yes 1/476 cases, 2/476 cases, 4/476 cases - - - Johan den Dunnen, Viviana Karina Dalamón
+/. 2 2 c.169C>T r.(?) p.(Gln57*) - pathogenic g.20763552G>A g.20189413G>A - - GJB2_000029 - PubMed: Baux 2017, Journal: Baux 2017 - rs111033297 Germline, Germline/De novo (untested) yes - - - - David Baux
+/. 2 - c.176_191del r,(?), r.(?) p.(Gly59AlafsTer18) - pathogenic, pathogenic (recessive) g.20763531_20763546del g.20189392_20189407del - - GJB2_000108 combination of alleles not reported, VKGL data sharing initiative Nederland PubMed: Wu 2019 - - CLASSIFICATION record, Germline - 2/1291 cases hearing loss - - - Johan den Dunnen, VKGL-NL_Nijmegen
?/. 1 - c.179G>T r.(?) p.(Cys60Phe) - VUS g.20763542C>A - - - GJB2_000132 VKGL data sharing initiative Nederland - - - CLASSIFICATION record - - - - - VKGL-NL_Nijmegen
?/. 1 - c.200A>G r.(?) p.(His67Arg) - VUS g.20763521T>C - GJB2(NM_004004.6):c.200A>G (p.H67R) - GJB2_000125 VKGL data sharing initiative Nederland - - - CLASSIFICATION record - - - - - VKGL-NL_Groningen
-?/. 1 - c.213C>T r.(?) p.(Ile71=) - likely benign g.20763508G>A g.20189369G>A - - GJB2_000099 VKGL data sharing initiative Nederland - - - CLASSIFICATION record - - - - - VKGL-NL_Nijmegen
?/. 1 - c.217C>A r.(?) p.(His73Asn) - VUS g.20763504G>T - GJB2(NM_004004.6):c.217C>A (p.H73N) - GJB2_000119 VKGL data sharing initiative Nederland - - - CLASSIFICATION record - - - - - VKGL-NL_Rotterdam
+/. 3 2 c.223C>T r.(?) p.(Arg75Trp) - pathogenic, pathogenic (dominant), pathogenic (recessive) g.20763498G>A g.20189359G>A - - GJB2_000053 VKGL data sharing initiative Nederland PubMed: Dalamon 2013, PubMed: Richard 2019 - - CLASSIFICATION record, Germline - - - - - Johan den Dunnen, VKGL-NL_Nijmegen, Viviana Karina Dalamón
+/. 6 2 c.224G>A r.(?) p.(Arg75Gln) - pathogenic, pathogenic (dominant), pathogenic (recessive) g.20763497C>T g.20189358C>T - - GJB2_000078 2 heterozygous, no homozygous; Clinindb (India), VKGL data sharing initiative Nederland PubMed: Dalamon 2013, PubMed: Narang 2020, Journal: Narang 2020, PubMed: Richard 2019, PubMed: Wu 2013 - rs28931593 CLASSIFICATION record, Germline yes 2/2795 individuals - - - Johan den Dunnen, VKGL-NL_Nijmegen, Viviana Karina Dalamón, Mohammed Faruq
+/., +?/. 3 2 c.229T>C r.(?) p.(Trp77Arg) - pathogenic, pathogenic (recessive) g.20763492A>G g.20189353A>G GJB2(NM_004004.6):c.229T>C (p.W77R) - GJB2_000044 VKGL data sharing initiative Nederland PubMed: Dalamon 2013 - - CLASSIFICATION record, Germline - 1/476 cases - - - VKGL-NL_Rotterdam, VKGL-NL_Nijmegen, Viviana Karina Dalamón
+/., ?/+ 27 2 c.231G>A r.(?) p.(Trp77*), p.(Trp77Ter) - pathogenic, pathogenic (recessive), VUS g.20763490C>T g.20189351C>T GJB2(NM_004004.6):c.231G>A (p.W77*) - GJB2_000004 7 heterozygous, no homozygous; Clinindb (India), VKGL data sharing initiative Nederland PubMed: Kelsell 1997, PubMed: Naz 2017, PubMed: Narang 2020, Journal: Narang 2020, PubMed: Shaikh 2017, 1 more item - rs80338944 CLASSIFICATION record, Germline yes 7/2795 individuals - - - Johan den Dunnen, Atteeq Rehman, VKGL-NL_Rotterdam, VKGL-NL_Nijmegen, Mohammed Faruq
+/. 1 2 c.232dup r.(?) p.(Ala78Glyfs*24) - pathogenic (recessive) g.20763491dup g.20189352dup - - GJB2_000074 - PubMed: Dalamon 2013 553568 - Germline yes - - - - Viviana Karina Dalamón
+/. 1 2 c.232G>T r.(?) p.(Ala78Ser) - pathogenic (recessive) g.20763489C>A g.20189350C>A - - GJB2_000071 - PubMed: Dalamon 2013 - - Germline yes singleton - - - Viviana Karina Dalamón
+/., +?/. 15 11, 9 c.235del r,(?), r.(?) p.(Leu79Cysfs*3), p.(Leu79CysfsTer3) ACMG likely pathogenic, likely pathogenic (recessive), pathogenic, pathogenic (recessive) g.20763486del, g.20763488del g.20189349del 233delC, 235delC, c.235delC, 1 more item - GJB2_000052 combination of alleles not reported, rs80338943, VKGL data sharing initiative Nederland PubMed: Choi 2013, PubMed: Sang 2019, PubMed: Shaikh 2017, PubMed: Wu 2019, PubMed: Xie 2022, 1 more item - - CLASSIFICATION record, Germline - 215/1291 cases hearing loss - - - Johan den Dunnen, VKGL-NL_Leiden, VKGL-NL_Groningen
+/. 1 - c.238C>A r.(?) p.(Gln80Lys) - pathogenic g.20763483G>T g.20189344G>T - - GJB2_000051 VKGL data sharing initiative Nederland - - - CLASSIFICATION record - - - - - VKGL-NL_Nijmegen
+?/. 1 - c.239A>C r.(?) p.(Gln80Pro) - likely pathogenic g.20763482T>G - - - GJB2_000110 - - - rs727504302 CLASSIFICATION record - - - - - MobiDetails
+?/. 1 - c.241C>G r.(?) p.(Leu81Val) - likely pathogenic g.20763480G>C - - - GJB2_000109 - - - rs145216882 CLASSIFICATION record - - - - - MobiDetails
+?/. 2 2 c.246C>G r.(?) p.(Ile82Met) - likely pathogenic, pathogenic (recessive) g.20763475G>C g.20189336G>C - - GJB2_000081 VKGL data sharing initiative Nederland PubMed: Dalamon 2013 - - CLASSIFICATION record, Germline - 1/476 cases - - - VKGL-NL_Nijmegen, Viviana Karina Dalamón
-?/. 3 2 c.249C>G r.(?) p.(Phe83Leu) - likely benign g.20763472G>C g.20189333G>C GJB2(NM_004004.6):c.249C>G (p.F83L) - GJB2_000043 VKGL data sharing initiative Nederland PubMed: Dalamon 2013 - - CLASSIFICATION record, Germline - 1/74 cases - - - VKGL-NL_Rotterdam, VKGL-NL_Nijmegen, Viviana Karina Dalamón
+/., +?/. 3 2 c.250G>C r.(?) p.(Val84Leu) - likely pathogenic, pathogenic, pathogenic (recessive) g.20763471C>G g.20189332C>G - - GJB2_000014 VKGL data sharing initiative Nederland PubMed: Dalamon 2013, PubMed: Zazo Seco 2017, Journal: Zazo Seco 2017 - - CLASSIFICATION record, Germline - 1/476 cases - - - Mieke Wesdorp, VKGL-NL_Nijmegen, Viviana Karina Dalamón
?/. 1 - c.250G>T r.(?) p.(Val84Leu) - VUS g.20763471C>A - - - GJB2_000135 VKGL data sharing initiative Nederland - - - CLASSIFICATION record - - - - - VKGL-NL_Nijmegen
?/. 1 - c.257C>T r.(?) p.(Thr86Met) - VUS g.20763464G>A - - - GJB2_000127 VKGL data sharing initiative Nederland - - - CLASSIFICATION record - - - - - VKGL-NL_Nijmegen
+/. 1 - c.263C>T r,(?) p.(Ala88Val) - pathogenic (dominant) g.20763458G>A g.20189319G>A - - GJB2_000134 combination of alleles not reported PubMed: Wu 2019 - - Germline - 1/1291 cases hearing loss - - - Johan den Dunnen
+?/. 1 2 c.269dup r.(?) p.(Val91Serfs*11) - pathogenic (recessive) g.20763452dup g.20189313dup 269dupT - GJB2_000080 - PubMed: Dalamon 2013 - - Germline - 1/476 cases - - - Viviana Karina Dalamón
+/., +?/. 9 2 c.269T>C r.(?) p.(Leu90Pro) - likely pathogenic, pathogenic, pathogenic (recessive) g.20763452A>G g.20189313A>G GJB2(NM_004004.6):c.269T>C (p.L90P, p.(Leu90Pro)) - GJB2_000015 VKGL data sharing initiative Nederland PubMed: Baux 2017, Journal: Baux 2017, PubMed: Dalamon 2013, 1 more item - rs80338945 CLASSIFICATION record, Germline, Germline/De novo (untested) yes 1/476 cases - - - David Baux, Mieke Wesdorp, VKGL-NL_Leiden, VKGL-NL_Rotterdam, VKGL-NL_Groningen, VKGL-NL_Nijmegen, Viviana Karina Dalamón
?/. 1 - c.280C>G r.(?) p.(His94Asp) - VUS g.20763441G>C - - - GJB2_000124 VKGL data sharing initiative Nederland - - - CLASSIFICATION record - - - - - VKGL-NL_Nijmegen
+?/. 3 2 c.283G>A r.(?) p.(Val95Met) - likely pathogenic, pathogenic (recessive) g.20763438C>T g.20189299C>T - - GJB2_000042 VKGL data sharing initiative Nederland PubMed: Dalamon 2013 - - CLASSIFICATION record, Germline - 1/476 cases - - - VKGL-NL_Nijmegen, Viviana Karina Dalamón
+/. 1 2 c.290dup r.(?) p.(Tyr97*) - pathogenic g.20763431dup g.20189292dup 290dupA - GJB2_000030 - PubMed: Baux 2017, Journal: Baux 2017 - rs786204491 Germline yes - - - - David Baux
+?/. 1 - c.299A>C r.(?) p.(His100Pro) - likely pathogenic g.20763422T>G g.20189283T>G - - GJB2_000050 VKGL data sharing initiative Nederland - - - CLASSIFICATION record - - - - - VKGL-NL_Nijmegen
+/., +?/. 5 - c.299_300del r,(?), r.(?) p.(His100Argfs*14), p.(His100ArgfsTer14) - likely pathogenic (recessive), pathogenic, pathogenic (recessive) g.20763421_20763422del g.20189282_20189283del 299_300delAT, c.299_300del - GJB2_000107 combination of alleles not reported, rs111033204, VKGL data sharing initiative Nederland PubMed: Choi 2013, PubMed: Wu 2019 - - CLASSIFICATION record, Germline ? 31/1291 cases hearing loss - - - Johan den Dunnen, VKGL-NL_Nijmegen, Seungmin Lee
+/. 1 - c.310_323del r.(?) p.(Arg104Glyfs*6) - pathogenic (recessive) g.20763398_20763411del g.20189259_20189272del 310del14 (Lys105Argfs*2) - GJB2_000113 - PubMed: Shaikh 2017 - - Germline - - - - - LOVD
+/., +?/. 6 2 c.313_326del r.(?) p.(Lys105Glyfs*5), p.(Lys105GlyfsTer5) - pathogenic, pathogenic (recessive) g.20763397_20763410del g.20189258_20189271del 313_326del14, GJB2(NM_004004.6):c.313_326delAAGTTCATCAAGGG (p.K105Gfs*5) - GJB2_000026 2 heterozygous, no homozygous; Clinindb (India), VKGL data sharing initiative Nederland PubMed: Baux 2017, Journal: Baux 2017, PubMed: Dalamon 2013, PubMed: Narang 2020, Journal: Narang 2020 - rs111033253 CLASSIFICATION record, Germline yes 1/476 cases, 2/2794 individuals - - - David Baux, VKGL-NL_Rotterdam, VKGL-NL_Nijmegen, Viviana Karina Dalamón, Mohammed Faruq
+/. 2 2 c.334_335del r.(?) p.(Lys112Glufs*2) - pathogenic g.20763387_20763388del g.20189248_20189249del 334_335delAA - GJB2_000019 - PubMed: Baux 2017, Journal: Baux 2017 - rs756484720 Germline, Germline/De novo (untested) yes - - - - David Baux
-/. 3 - c.341A>G r.(?) p.(Glu114Gly) - benign g.20763380T>C g.20189241T>C GJB2(NM_004004.6):c.341A>G (p.E114G) - GJB2_000041 VKGL data sharing initiative Nederland - - - CLASSIFICATION record - - - - - VKGL-NL_Rotterdam, VKGL-NL_Groningen, VKGL-NL_Nijmegen
+/. 1 2 c.353_356dup r.(?) p.(Glu119Aspfs*92) - pathogenic g.20763366_20763369dup g.20189227_20189230dup - - GJB2_000018 - PubMed: Baux 2017, Journal: Baux 2017 - - Germline yes - - - - David Baux
?/. 2 - c.355G>A r.(?) p.(Glu119Lys) - VUS g.20763366C>T - GJB2(NM_004004.6):c.355G>A (p.E119K) - GJB2_000040 VKGL data sharing initiative Nederland - - - CLASSIFICATION record - - - - - VKGL-NL_Groningen, VKGL-NL_Nijmegen
+/. 1 - c.355G>T r.(?) p.(Glu119Ter) ACMG pathogenic (recessive) g.20763366C>A g.20189227C>A - - GJB2_000128 - PubMed: Richard 2019 - - Germline - - - - - Johan den Dunnen
+/., +?/. 8 2 c.358_360del r.(?) p.(Glu120del) - likely pathogenic, pathogenic, pathogenic (recessive) g.20763364_20763366del g.20189225_20189227del 358_360delGAG, 1 more item - GJB2_000020 VKGL data sharing initiative Nederland PubMed: Baux 2017, Journal: Baux 2017, PubMed: Dalamon 2013, PubMed: Marlin 2005, PubMed: Naz 2017 - rs80338947 CLASSIFICATION record, Germline, Germline/De novo (untested) yes 1/476 cases - - - Johan den Dunnen, David Baux, VKGL-NL_Leiden, VKGL-NL_Rotterdam, VKGL-NL_Nijmegen, Viviana Karina Dalamón
+/. 1 - c.370C>T r.(?) p.(Gln124*) - pathogenic g.20763351G>A g.20189212G>A - - GJB2_000103 3 heterozygous, no homozygous; Clinindb (India) PubMed: Narang 2020, Journal: Narang 2020 - rs397516874 Germline - 3/2795 individuals - - - Mohammed Faruq
-/., -?/., ?/. 4 2 c.380G>A r.(?) p.(Arg127His) - benign, likely benign, VUS g.20763341C>T g.20189202C>T GJB2(NM_004004.6):c.380G>A (p.R127H) - GJB2_000002 VKGL data sharing initiative Nederland PubMed: Dalamon 2013 - - CLASSIFICATION record, Germline - 1/74 cases - - - Yu Sun, VKGL-NL_Rotterdam, VKGL-NL_Nijmegen, Viviana Karina Dalamón
?/. 1 - c.382A>G r.(?) p.(Ile128Val) - VUS g.20763339T>C - GJB2(NM_004004.5):c.382A>G (p.(Ile128Val)) - GJB2_000130 VKGL data sharing initiative Nederland - - - CLASSIFICATION record - - - - - VKGL-NL_Leiden
-?/. 1 2 c.384C>T r.(=) p.(=) - likely benign g.20763337G>A g.20189198G>A - - GJB2_000076 - PubMed: Dalamon 2013 - - Germline - 1/74 cases - - - Viviana Karina Dalamón
?/. 1 - c.389G>C r.(?) p.(Gly130Ala) - VUS g.20763332C>G - - - GJB2_000123 VKGL data sharing initiative Nederland - - - CLASSIFICATION record - - - - - VKGL-NL_Nijmegen
?/. 1 - c.389G>T r.(?) p.(Gly130Val) - VUS g.20763332C>A g.20189193C>A - - GJB2_000070 VKGL data sharing initiative Nederland - - - CLASSIFICATION record - - - - - VKGL-NL_Nijmegen
+/., ?/. 3 2 c.400T>C r.(?) p.(Trp134Arg) - pathogenic, VUS g.20763321A>G g.20189182A>G - - GJB2_000008 VKGL data sharing initiative Nederland PubMed: Behar 2014, Journal: Behar 2014 - - CLASSIFICATION record, Germline yes - - - - Johan den Dunnen, Zippi Brownstein, VKGL-NL_Nijmegen
+?/., ?/. 4 - c.416G>A r.(?) p.(Ser139Asn) - likely pathogenic, VUS g.20763305C>T g.20189166C>T GJB2(NM_004004.5):c.416G>A (p.(Ser139Asn)), GJB2(NM_004004.6):c.416G>A (p.S139N) - GJB2_000039 VKGL data sharing initiative Nederland - - - CLASSIFICATION record - - - - - VKGL-NL_Leiden, VKGL-NL_Rotterdam, VKGL-NL_Groningen, VKGL-NL_Nijmegen
?/. 1 - c.425T>C r.(?) p.(Phe142Ser) - VUS g.20763296A>G g.20189157A>G - - GJB2_000092 VKGL data sharing initiative Nederland - - - CLASSIFICATION record - - - - - VKGL-NL_Nijmegen
+/., +?/. 15 2 c.427C>T r,(?), r.(?) p.(Arg143Trp) ACMG pathogenic, pathogenic (recessive) g.20189155G>A, g.20763294G>A g.20189155G>A GJB2(NM_004004.6):c.427C>T (p.R143W) - GJB2_000038 combination of alleles not reported, VKGL data sharing initiative Nederland PubMed: Dalamon 2013, PubMed: Dia 2022, PubMed: Wu 2019 17009 - CLASSIFICATION record, Germline yes 1/476 cases, 2/476 cases, 4/148 controls, 6/1291 cases hearing loss - - - Johan den Dunnen, VKGL-NL_Rotterdam, VKGL-NL_Nijmegen, Viviana Karina Dalamón, Yacouba Dia
+/. 2 - c.428G>A r,(?), r.(?) p.(Arg143Gln) ACMG pathogenic (dominant), pathogenic (recessive) g.20763293C>T g.20189154C>T - - GJB2_000091 combination of alleles not reported PubMed: Sang 2019, PubMed: Wu 2019 - - Germline - 1/1291 cases hearing loss - - - Johan den Dunnen
+?/. 1 2 c.428G>T r.(?) p.(Arg143Leu) - likely pathogenic g.20763293C>A g.20189154C>A - - GJB2_000025 - PubMed: Baux 2017, Journal: Baux 2017 - rs104894401 Germline yes - - - - David Baux
+/., +?/. 3 2 c.439G>A r.(?) p.(Glu147Lys) ACMG likely pathogenic, pathogenic (recessive) g.20763282C>T g.20189143C>T - - GJB2_000024 VKGL data sharing initiative Nederland PubMed: Baux 2017, Journal: Baux 2017, PubMed: Sang 2019 - rs1801002 CLASSIFICATION record, Germline yes - - - - Johan den Dunnen, David Baux, VKGL-NL_Nijmegen
+/. 1 - c.449del r.(?) p.(Phe150Serfs*18) - pathogenic g.20763273del - - - GJB2_000122 VKGL data sharing initiative Nederland - - - CLASSIFICATION record - - - - - VKGL-NL_Nijmegen
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