Unique variants in the SOX10 gene

Information The variants shown are described using the NM_006941.3 transcript reference sequence.

170 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 - c.-227C>T r.(?) p.(=) - VUS g.38380488G>A - SOX10(NM_006941.4):c.-227C>T - POLR2F_000030 VKGL data sharing initiative Nederland - - - CLASSIFICATION record - - - - - VKGL-NL_Leiden
-?/. 1 - c.-10_-4del r.(?) p.(=) - likely benign g.38379798_38379804del - SOX10(NM_006941.4):c.-10_-4delCGGGGGC - POLR2F_000005 VKGL data sharing initiative Nederland - - - CLASSIFICATION record - - - - - VKGL-NL_AMC
+/+, +?/+? 7 _1_4_ c.-278_*1183{0} r.0 p.0 - likely pathogenic (dominant), pathogenic (dominant) g.(?_38368319)_(38380539_?)del - 56.6 kb deletion of SOX10 upstream regulatory elements, 6 more items - SOX10_000063, SOX10_000064, SOX10_000067, SOX10_000068, SOX10_000069, SOX10_000071, SOX10_000072 whole gene deletion (1.3 to 1.6 Mb), whole gene deletion (213 to 222 kb), 3 more items PubMed: Bondurand 2007, PubMed: Bondurand 2012, PubMed: Siomou 2012 - - De novo - - - - - Veronique Pingault
+?/. 1 2 c.2T>C r.(?) p.(Met1?) - likely pathogenic g.38379790A>G g.37983783A>G - - SOX10_000121 VKGL data sharing initiative Nederland - - - CLASSIFICATION record - - - - - VKGL-NL_Nijmegen
+/+ 1 2 c.2T>G r.(?) p.0? - pathogenic (dominant) g.38379790A>C g.37983783A>C Met1? - SOX10_000076 A more distal initiation codon is used in vitro but the function of the mutant protein is lost PubMed: Pingault 2013 - - Germline - - - - - Veronique Pingault
?/. 1 2 c.11A>G r.(?) p.(Glu4Gly) - VUS g.38379781T>C g.37983774T>C SOX10(NM_006941.3):c.11A>G (p.(Glu4Gly)) - SOX10_000103 VKGL data sharing initiative Nederland - - - CLASSIFICATION record - - - - - VKGL-NL_Leiden
+?/. 2 - c.12_13delinsAT r.(?) p.(Gln5*) ACMG pathogenic (dominant) g.38379779_38379780delinsAT g.37983772_37983773delinsAT - - SOX10_000151 - PubMed: Batissoco 2021 ClinVar-SCV001821525.1 - Germline, Unknown yes - - - - Karina Lezirovitz Mandelbaum
-/. 2 2 c.18C>T r.(?) p.(Asp6=) - benign g.38379774G>A g.37983767G>A SOX10(NM_006941.4):c.18C>T (p.D6=) - SOX10_000102 VKGL data sharing initiative Nederland - - - CLASSIFICATION record - - - - - VKGL-NL_Nijmegen, VKGL-NL_AMC
+/. 1 - c.44_62del r.(?) p.(Val15Alafs*11) ACMG pathogenic (dominant) g.38379738_38379756del g.37983731_37983749del g.38379730_38379748del - SOX10_000150 - PubMed: Batissoco 2021 ClinVar-SCV001792238 - Unknown ? - - - - Karina Lezirovitz Mandelbaum
+/+ 1 2 c.50_73delinsGCCCGACGCTAGGGCCCTAG r.(?) p.(Ser17Cysfs*7) - pathogenic (dominant) g.38379719_38379742delinsCTAGGGCCCTAGCGTCGGGC g.37983712_37983735delinsCTAGGGCCCTAGCGTCGGGC c.50-73del24insGCCCGACGCTAGGGCCCTAG - SOX10_000001 - PubMed: Pingault 2010 - - Germline - - - - - Veronique Pingault
+/+ 1 2 c.111_220del r.(?) p.(Gly39Argfs*58) - pathogenic (dominant) g.38379572_38379681del g.37983566_37983675del 110_219del110 - SOX10_000044 - PubMed: Chen 2010 - - Germline - - - - - Veronique Pingault
+/+ 1 2 c.112_131del r.(?) p.(Gly38Glnfs*22) - pathogenic (dominant) g.38379661_38379680del g.37983656_37983675del c.112_131del20 (G38Qfs21X) - SOX10_000042 loss of transactivation capabilities PubMed: Sanchez-Meijas 2010 - - De novo - - - - - Veronique Pingault
+/+ 2 2 c.113del r.(?) p.(Gly38Alafs*71) - pathogenic, pathogenic (dominant) g.38379679del g.37983673del c.113delG - SOX10_000045 - MORL Deafness Variation Database, PubMed: Zhang 2012, PubMed: Chen 2010 - - SUMMARY record, Unknown - - - - - Global Variome, with Curator vacancy, Veronique Pingault
-?/., ?/. 2 2 c.114_116dup r.(?) p.(Gly39dup) - likely benign, VUS g.38379688_38379690dup g.37983681_37983683dup SOX10(NM_006941.3):c.114_116dupCGG (p.G39dup), SOX10(NM_006941.4):c.114_116dup (p.(Gly39dup)) - SOX10_000118 VKGL data sharing initiative Nederland - - - CLASSIFICATION record - - - - - VKGL-NL_Leiden, VKGL-NL_Rotterdam
+/+ 1 2 c.115G>T r.(?) p.(Gly39*) - pathogenic g.38379677C>A g.37983670C>A - - SOX10_000143 - MORL Deafness Variation Database, PubMed: Arimoto 2014 - - SUMMARY record - - - - - Global Variome, with Curator vacancy
+/. 1 - c.119C>A r.(?) p.(Ser40*) - pathogenic g.38379673G>T - - - SOX10_000148 - - - - Unknown - - - - - Gunnar Schmidt
-/., -?/. 3 2 c.122G>T r.(?) p.(Gly41Val) - benign, likely benign g.38379670C>A g.37983663C>A SOX10(NM_006941.3):c.122G>T (p.G41V, p.(Gly41Val)), SOX10(NM_006941.4):c.122G>T (p.G41V) - SOX10_000101 VKGL data sharing initiative Nederland - - - CLASSIFICATION record - - - - - VKGL-NL_Leiden, VKGL-NL_Rotterdam, VKGL-NL_AMC
+/+ 1 2 c.126_127delinsCT r.(?) p.(Arg43*) - pathogenic (dominant) g.38379665_38379666delinsAG g.37983658_37983659delinsAG R43X ; 126GC>CT - SOX10_000002 - PubMed: Pingault 2002 - - De novo - - - - - Veronique Pingault
+/+ 1 2 c.126_127delinsTT r.(?) p.(Arg43*) - pathogenic (dominant) g.38379665_38379666delinsAA g.37983658_37983659delinsAA c.126_127delGCinsTT - SOX10_000046 - PubMed: Chen 2010 - - Unknown - - - - - Veronique Pingault
-?/. 1 - c.131C>G r.(?) p.(Ala44Gly) - likely benign g.38379661G>C - SOX10(NM_006941.3):c.131C>G (p.A44G) - POLR2F_000022 VKGL data sharing initiative Nederland - - - CLASSIFICATION record - - - - - VKGL-NL_Utrecht
+/+, +/. 2 2 c.155del r.(?) p.(Gly52Alafs*57), p.(Gly52AlafsTer57) - pathogenic, pathogenic (dominant) g.38379637del, g.38379639del g.37983632del 153-155del; G52Afs56X, SOX10(NM_006941.3):c.155delG (p.G52Afs*57) - SOX10_000043 inherited from unaffected mother; loss of transactivation capabilities, 1 more item PubMed: Sanchez-Meijas 2010 - - CLASSIFICATION record, Germline - - - - - VKGL-NL_Rotterdam, Veronique Pingault
+/+ 1 2 c.165del r.(?) p.(Lys56Argfs*53) - pathogenic g.38379627del g.37983620del - - SOX10_000142 - MORL Deafness Variation Database, PubMed: Inoue 2002 - - SUMMARY record - - - - - Global Variome, with Curator vacancy
+/+ 1 2 c.169del r.(?) p.(Glu57Serfs*52) - pathogenic (dominant) g.38379623del g.37983617del nt168delG - SOX10_000003 - PubMed: Sham 2001 - - De novo - - - - - Veronique Pingault
+/+ 1 2 c.184G>T r.(?) p.(Glu62*) - pathogenic g.38379608C>A g.37983601C>A - - SOX10_000141 - MORL Deafness Variation Database, PubMed: Vaaralahti 2014 - - SUMMARY record - - - - - Global Variome, with Curator vacancy
+/+ 1 2_2i c.219_428+43del r.spl p.? - pathogenic (dominant) g.38379321_38379573del g.37983316_37983568del - - SOX10_000065 - PubMed: Bondurand 2007 - - Germline - - - - - Veronique Pingault
-?/. 1 2 c.231C>T r.(?) p.(Ser77=) - likely benign g.38379561G>A g.37983554G>A SOX10(NM_006941.3):c.231C>T (p.S77=) - SOX10_000100 VKGL data sharing initiative Nederland - - - CLASSIFICATION record - - - - - VKGL-NL_Rotterdam
-?/. 2 2 c.246C>G r.(?) p.(Gly82=) - likely benign g.38379546G>C g.37983539G>C SOX10(NM_006941.3):c.246C>G (p.G82=), SOX10(NM_006941.4):c.246C>G (p.(Gly82=)) - SOX10_000116 VKGL data sharing initiative Nederland - - - CLASSIFICATION record - - - - - VKGL-NL_Leiden, VKGL-NL_Rotterdam
+/+ 2 2 c.249C>A r.(?) p.(Tyr83*) - pathogenic, pathogenic (dominant) g.38379543G>T g.37983536G>T Y83X (C>A at 249) - SOX10_000004 - MORL Deafness Variation Database, PubMed: Xiong 2015, PubMed: Pingault 1998 - - De novo, SUMMARY record - - - - - Global Variome, with Curator vacancy, Veronique Pingault
+/+ 1 2 c.249C>G r.(?) p.(Tyr83*) - pathogenic g.38379543G>C g.37983536G>C - - SOX10_000140 - MORL Deafness Variation Database, PubMed: Pingault 1998 - - SUMMARY record - - - - - Global Variome, with Curator vacancy
-/., -?/. 2 2 c.249C>T r.(?) p.(Tyr83=) - benign, likely benign g.38379543G>A g.37983536G>A SOX10(NM_006941.3):c.249C>T (p.Y83=), SOX10(NM_006941.4):c.249C>T (p.Y83=) - SOX10_000115 VKGL data sharing initiative Nederland - - - CLASSIFICATION record - - - - - VKGL-NL_Utrecht, VKGL-NL_AMC
+/+ 2 2 c.254G>A r.(?) p.(Trp85*) - pathogenic, pathogenic (dominant) g.38379538C>T g.37983531C>T - - SOX10_000047 inherited from unaffected mother; a mosaicism is suspected. MORL Deafness Variation Database, PubMed: Xiong 2015, PubMed: Jiang 2011 - - Germline, SUMMARY record - - - - - Global Variome, with Curator vacancy, Veronique Pingault
?/. 1 - c.256A>G r.(?) p.(Thr86Ala) - VUS g.38379536T>C - SOX10(NM_006941.4):c.256A>G (p.(Thr86Ala)) - POLR2F_000021 VKGL data sharing initiative Nederland - - - CLASSIFICATION record - - - - - VKGL-NL_Leiden
+/+ 1 2 c.259_260del r.(?) p.(Leu87Glyfs*46) - pathogenic g.38379532_38379533del g.37983525_37983526del - - SOX10_000139 - MORL Deafness Variation Database, PubMed: Chen 2014 - - SUMMARY record - - - - - Global Variome, with Curator vacancy
+?/+? 1 2 c.271_275del r.(?) p.(Pro91Alafs*41) - likely pathogenic g.38379517_38379521del g.37983510_37983514del - - SOX10_000138 - MORL Deafness Variation Database, PubMed: Tang 2015 - - SUMMARY record - - - - - Global Variome, with Curator vacancy
-?/-? 1 2 c.274G>C r.(?) p.(Val92Leu) - likely benign g.38379518C>G g.37983511C>G - - SOX10_000005 1 more item PubMed: Bondurand 2007 - - Germline - - - - - Veronique Pingault
+/. 1 - c.314_315del r,(?) p.(Lys105ThrfsTer28) - pathogenic (dominant) g.38379477_38379478del g.37983470_37983471del - - SOX10_000163 combination of alleles not reported PubMed: Wu 2019 - - Germline - 1/1291 cases hearing loss - - - Johan den Dunnen
?/. 1 2 c.315G>C r.(?) p.(Lys105Asn) - VUS g.38379477C>G g.37983470C>G SOX10(NM_006941.3):c.315G>C (p.K105N) - SOX10_000114 VKGL data sharing initiative Nederland - - - CLASSIFICATION record - - - - - VKGL-NL_Rotterdam
+?/+? 1 2 c.316C>T r.(?) p.(Arg106Trp) - likely pathogenic (dominant) g.38379476G>A g.37983469G>A - - SOX10_000050 major functional effect in vitro PubMed: Chaoui 2011 - - Germline - - - - - Veronique Pingault
+?/. 1 - c.322A>G r.(?) p.(Met108Val) - likely pathogenic g.38379470T>C - - - POLR2F_000015 VKGL data sharing initiative Nederland - - - CLASSIFICATION record - - - - - VKGL-NL_Nijmegen
+?/+? 1 2 c.323T>C r.(?) p.(Met108Thr) - likely pathogenic (dominant) g.38379469A>G g.37983462A>G - - SOX10_000082 Loss of function in vitro PubMed: Pingault 2013 - - Germline - - - - - Veronique Pingault
+/. 1 - c.327C>G r.(?) p.(Asn109Lys) - pathogenic g.38379465G>C - SOX10(NM_006941.3):c.327C>G (p.N109K) - POLR2F_000007 VKGL data sharing initiative Nederland - - - CLASSIFICATION record - - - - - VKGL-NL_Rotterdam
+/+ 1 2 c.328_329del r.(?) p.(Ala110Leufs*23) - pathogenic (dominant) g.38379463_38379464del g.37983457_37983458del 327delCG - SOX10_000006 - PubMed: Pingault 2002 - - Germline - - - - - Veronique Pingault
+?/+? 1 2 c.331T>G r.(?) p.(Phe111Val) - likely pathogenic (dominant) g.38379461A>C g.37983454A>C - - SOX10_000077 Loss of function in vitro PubMed: Pingault 2013 - - De novo - - - - - Veronique Pingault
+?/. 1 2 c.333C>A r.(?) p.(Phe111Leu) - likely pathogenic g.38379459G>T - SOX10(NM_006941.3):c.333C>A (p.F111L) - SOX10_000128 VKGL data sharing initiative Nederland - - - CLASSIFICATION record - - - - - VKGL-NL_Rotterdam
+/. 1 - c.335T>C r.(?) p.(Met112Thr) - pathogenic g.38379457A>G - - - POLR2F_000027 VKGL data sharing initiative Nederland - - - CLASSIFICATION record - - - - - VKGL-NL_Nijmegen
+?/+?, +?/. 3 2 c.336G>A r.(?) p.(Met112Ile) ACMG likely pathogenic, likely pathogenic (dominant) g.38379456C>T g.37983449C>T - - SOX10_000051 mild functional effect in vitro PubMed: Chaoui 2011 - - De novo, Germline/De novo (untested) yes - entsj - - Veronique Pingault, Jian Song
+?/+? 1 2 c.336G>C r.(?) p.(Met112Ile) - likely pathogenic (dominant) g.38379456C>G g.37983449C>G - - SOX10_000052 mild functional effect in vitro PubMed: Chaoui 2011 - - Germline - - - - - Veronique Pingault
?/. 1 - c.337G>A r.(?) p.(Val113Met) - VUS g.38379455C>T - - - POLR2F_000020 VKGL data sharing initiative Nederland - - - CLASSIFICATION record - - - - - VKGL-NL_Nijmegen
+?/. 1 - c.341G>C r.(?) p.(Trp114Ser) - likely pathogenic (dominant) g.38379451C>G g.37983444C>G - - SOX10_000159 - PubMed: Poli 2024 - - Germline/De novo (untested) - - - - - Johan den Dunnen
+/. 1 2 c.346C>G r.(?) p.(Gln116Glu) - pathogenic g.38379446G>C g.37983439G>C - - SOX10_000111 VKGL data sharing initiative Nederland - - - CLASSIFICATION record - - - - - VKGL-NL_Nijmegen
+?/. 1 2 c.347A>T r.(?) p.(Gln116Leu) - likely pathogenic g.38379445T>A - - - SOX10_000127 VKGL data sharing initiative Nederland - - - CLASSIFICATION record - - - - - VKGL-NL_Nijmegen
+?/+? 1 2 c.391A>C r.(?) p.(Asn131His) - likely pathogenic (dominant) g.38379401T>G g.37983394T>G - - SOX10_000053 mild functional effect in vitro PubMed: Chaoui 2011 - - De novo - - - - - Veronique Pingault
+?/+? 1 2 c.398A>G r.(?) p.(Glu133Gly) - likely pathogenic (dominant) g.38379394T>C g.37983387T>C - - SOX10_000074 - PubMed: Elmaleh-Berges 2013 - - De novo - - - - - Veronique Pingault
+/+ 1 2 c.403A>G r.(?) p.(Ser135Gly) - pathogenic g.38379389T>C g.37983382T>C - - SOX10_000137 - MORL Deafness Variation Database, PubMed: Marcos 2014 - - SUMMARY record - - - - - Global Variome, with Curator vacancy
+/+, +/., +?/+? 3 2 c.404G>C r.(?) p.(Ser135Thr) - likely pathogenic (dominant), pathogenic g.38379388C>G g.37983381C>G S135T, SOX10(NM_006941.4):c.404G>C (p.S135T) - SOX10_000007 VKGL data sharing initiative Nederland MORL Deafness Variation Database, PubMed: Hennekam 1996, PubMed: Bondurand 1999 - - CLASSIFICATION record, SUMMARY record, Unknown - - - - - Global Variome, with Curator vacancy, VKGL-NL_Groningen, Veronique Pingault
+?/. 1 - c.415G>A r.(?) p.(Gly139Ser) - likely pathogenic g.38379377C>T - - - POLR2F_000017 VKGL data sharing initiative Nederland - - - CLASSIFICATION record - - - - - VKGL-NL_Nijmegen
+?/+? 1 2 c.424T>C r.(?) p.(Trp142Arg) - likely pathogenic (dominant) g.38379368A>G g.37983361A>G - - SOX10_000078 Loss of function in vitro PubMed: Pingault 2013 - - Unknown - - - - - Veronique Pingault
+/. 1 2 c.425G>A r.(?) p.(Trp142Ter) - pathogenic g.38379367C>T g.37983360C>T - - SOX10_000109 VKGL data sharing initiative Nederland - - - CLASSIFICATION record - - - - - VKGL-NL_Nijmegen
+/+ 2 2i c.428+2T>G r.spl, r.spl? p.? - pathogenic, pathogenic (dominant) g.38379362A>C g.37983355A>C 428+2T-G - SOX10_000008 - Touraine 1998 Am J Hum Genet 63:A174, 1 more item - - Germline, SUMMARY record - - - - - Global Variome, with Curator vacancy, Veronique Pingault
+/+ 1 2i_3i c.429-1112_697+396del r.? p.? - pathogenic (dominant) g.38373478_38375254del g.37977477_37979253del - - SOX10_000066 - PubMed: Bondurand 2007 - - Germline - - - - - Veronique Pingault
+/. 1 2i c.429-1G>A r.spl p.? - pathogenic g.38374143C>T g.37978136C>T - - SOX10_000145 - - - - De novo ? - - - - Seungmin Lee
+?/. 1 2i c.429-1G>C r.spl p.? ACMG likely pathogenic g.38374143C>G g.37978136C>G - - SOX10_000158 - - - - Germline/De novo (untested) - - - - - Dongye He
+?/+? 1 3 c.434T>C r.(?) p.(Leu145Pro) - likely pathogenic (dominant) g.38374137A>G g.37978130A>G - - SOX10_000054 major functional effect in vitro PubMed: Chaoui 2011 - - Germline - - - - - Veronique Pingault
+?/. 1 - c.448A>G r.(?) p.(Lys150Glu) ACMG pathogenic (dominant) g.38374123T>C - - - SOX10_000152 - PubMed: Batissoco 2021 - - De novo yes - - - - Karina Lezirovitz Mandelbaum
+?/+? 1 3 c.450G>C r.(?) p.(Lys150Asn) - likely pathogenic (dominant) g.38374121C>G g.37978114C>G - - SOX10_000055 1 more item PubMed: Chaoui 2011 - - De novo - - - - - Veronique Pingault
+?/+?, ?/. 2 3 c.451C>T r.(?) p.(Arg151Cys) - likely pathogenic (dominant), VUS g.38374120G>A g.37978113G>A SOX10(NM_006941.4):c.451C>T (p.R151C) - SOX10_000083 Loss of function in vitro, VKGL data sharing initiative Nederland PubMed: Pingault 2013 - - CLASSIFICATION record, Unknown - - - - - VKGL-NL_AMC, Veronique Pingault
?/. 1 3 c.452G>A r.(?) p.(Arg151His) - VUS g.38374119C>T g.37978112C>T - - SOX10_000108 VKGL data sharing initiative Nederland - - - CLASSIFICATION record - - - - - VKGL-NL_Nijmegen
+?/+? 1 3 c.470C>T r.(?) p.(Ala157Val) - likely pathogenic (dominant) g.38374101G>A g.37978094G>A - - SOX10_000009 - PubMed: Morin 2008 - - De novo - - - - - Veronique Pingault
+/. 1 3 c.472G>T r.(?) p.(Glu158Ter) - pathogenic g.38374099C>A - - - SOX10_000126 VKGL data sharing initiative Nederland - - - CLASSIFICATION record - - - - - VKGL-NL_Nijmegen
+?/. 1 3 c.475C>T r.(?) p.(Arg159Trp) - likely pathogenic g.38374096G>A - - - SOX10_000125 VKGL data sharing initiative Nederland - - - CLASSIFICATION record - - - - - VKGL-NL_Nijmegen
+/+ 1 3 c.477_482dup r.(?) p.(Leu160_Arg161dup) - pathogenic (dominant) g.38374089_38374094dup g.37978084_37978089dup 482ins6 (GCTCCG) - SOX10_000010 - PubMed: Pingault 1998 - - De novo - - - - - Veronique Pingault
+/+ 1 3 c.481C>T r.(?) p.(Arg161Cys) - pathogenic g.38374090G>A g.37978083G>A - - SOX10_000136 - MORL Deafness Variation Database, PubMed: Marcos 2014 - - SUMMARY record - - - - - Global Variome, with Curator vacancy
+/., +?/+?, +?/., ?/. 4 3 c.482G>A r.(?) p.(Arg161His) - likely pathogenic, likely pathogenic (dominant), pathogenic, VUS g.38374089C>T g.37978082C>T - - SOX10_000056 major functional effect in vitro, VKGL data sharing initiative Nederland PubMed: Chaoui 2011, PubMed: Roman 2020, Journal: Roman 2020, 1 more item - - CLASSIFICATION record, Germline - - - - - Johan den Dunnen, Mieke Wesdorp, VKGL-NL_Nijmegen, Veronique Pingault
+/+ 1 3 c.483_484insGCTCCT r.(?) p.(Arg161_Met162insAlaPro) - pathogenic g.38374088_38374089insGGAGCA g.37978081_37978082insGGAGCA - - SOX10_000135 - MORL Deafness Variation Database, PubMed: Pingault 1998 - - SUMMARY record - - - - - Global Variome, with Curator vacancy
+?/. 1 - c.492C>A r.(?) p.(His164Gln) - likely pathogenic g.38374079G>T - - - SOX10_000160 - - - - CLASSIFICATION record - - - - - MobiDetails
+?/. 2 3 c.506C>T r.(?) p.(Pro169Leu) ACMG likely pathogenic (dominant) g.38374065G>A g.37978058G>A - - SOX10_000161 - PubMed: Graziani 2024, PubMed: Pingault 2014 - - Germline yes - - - - Johan den Dunnen, Ludovico Graziani
+/+ 1 3 c.506del r.(?) p.(Pro169Argfs*117) - pathogenic (dominant) g.38374065del g.37978060del 506delC, p.Pro169fsX284 - SOX10_000011 - PubMed: Iso 2008 - - Germline - - - - - Veronique Pingault
+/+ 2 3 c.519C>G r.(?) p.(Tyr173*) - pathogenic, pathogenic (dominant) g.38374052G>C g.37978045G>C - - SOX10_000012 - MORL Deafness Variation Database, PubMed: Xiong 2015, PubMed: Pingault 2010 - - Germline, SUMMARY record - - - - - Global Variome, with Curator vacancy, Veronique Pingault
+/+, +?/+? 2 3 c.521A>C r.(?) p.(Gln174Pro) - likely pathogenic (dominant), pathogenic g.38374050T>G g.37978043T>G Q174P - SOX10_000013 - MORL Deafness Variation Database, PubMed: Chaoui 2011, PubMed: Barnett 2009 - - Germline, SUMMARY record - - - - - Global Variome, with Curator vacancy, Veronique Pingault
+?/+? 1 3 c.523C>G r.(?) p.(Pro175Ala) - likely pathogenic (dominant) g.38374048G>C g.37978041G>C - - SOX10_000057 major functional effect in vitro PubMed: Chaoui 2011 - - De novo - - - - - Veronique Pingault
+?/+? 1 3 c.524C>G r.(?) p.(Pro175Arg) - likely pathogenic (dominant) g.38374047G>C g.37978040G>C - - SOX10_000058 major functional effect in vitro PubMed: Chaoui 2011 - - De novo - - - - - Veronique Pingault
+?/+? 1 3 c.524C>T r.(?) p.(Pro175Leu) - likely pathogenic (dominant) g.38374047G>A g.37978040G>A - - SOX10_000059 major functional effect in vitro PubMed: Chaoui 2011 - - Germline - - - - - Veronique Pingault
+?/. 1 - c.532C>T r.(?) p.(Arg178Trp) - likely pathogenic g.38374039G>A - - - SOX10_000153 - - - - CLASSIFICATION record - - - - - MobiDetails
+/. 1 - c.544_557del r.(?) p.(Lys182Argfs*94) ACMG pathogenic g.38374017_38374030del g.37978010_37978023del - - SOX10_000155 - - - - De novo - - - - - Jian Song
-?/. 1 - c.550G>A r.(?) p.(Ala184Thr) - likely benign g.38374021C>T - SOX10(NM_006941.3):c.550G>A (p.(Ala184Thr)) - POLR2F_000026 VKGL data sharing initiative Nederland - - - CLASSIFICATION record - - - - - VKGL-NL_Leiden
+/. 1 - c.553C>T r.(?) p.(Gln185*) ACMG pathogenic g.38374018G>A g.37978011G>A - - SOX10_000154 F-1 - - - Germline/De novo (untested) yes - - - - Jian Song
+/+, +/. 3 3 c.565G>T r.(?) p.(Glu189*) - pathogenic, pathogenic (dominant) g.38374006C>A g.37977999C>A E189X (G>T at 565) - SOX10_000014 VKGL data sharing initiative Nederland MORL Deafness Variation Database, PubMed: Xiong 2015, PubMed: Pingault 1998 - - CLASSIFICATION record, De novo, SUMMARY record - - - - - Global Variome, with Curator vacancy, VKGL-NL_Nijmegen, Veronique Pingault
-?/. 2 - c.574G>A r.(?) p.(Gly192Ser) - likely benign g.38373997C>T - SOX10(NM_006941.4):c.574G>A (p.G192S, p.(Gly192Ser)) - POLR2F_000025 VKGL data sharing initiative Nederland - - - CLASSIFICATION record - - - - - VKGL-NL_Leiden, VKGL-NL_AMC
?/. 1 - c.601G>C r.(?) p.(Ala201Pro) - VUS g.38373970C>G - - - POLR2F_000024 VKGL data sharing initiative Nederland - - - CLASSIFICATION record - - - - - VKGL-NL_Nijmegen
+/. 1 - c.610C>T r.(?) p.(Gln204*) - pathogenic g.38373961G>A - SOX10(NM_006941.3):c.610C>T (p.Q204*) - POLR2F_000004 VKGL data sharing initiative Nederland - - - CLASSIFICATION record - - - - - VKGL-NL_Rotterdam
+/+ 1 3 c.621C>A r.(?) p.(Tyr207*) - pathogenic g.38373950G>T g.37977943G>T - - SOX10_000134 - MORL Deafness Variation Database, PubMed: Chen 2015 - - SUMMARY record - - - - - Global Variome, with Curator vacancy
+/+ 2 3 c.621C>G r.(?) p.(Tyr207*) - pathogenic, pathogenic (dominant) g.38373950G>C g.37977943G>C Y207X - SOX10_000015 - MORL Deafness Variation Database, PubMed: Xiong 2015, PubMed: Southard-Smith 1999 - - De novo, SUMMARY record - - - - - Global Variome, with Curator vacancy, Veronique Pingault
+/+ 1 3 c.644_648del r.(?) p.(Arg215Profs*64) - pathogenic (dominant) g.38373923_38373927del g.37977917_37977921del c.644_648delGGCAC - SOX10_000016 - PubMed: Pingault 2010 - - De novo - - - - - Veronique Pingault
-?/. 1 - c.660C>G r.(?) p.(Gly220=) - likely benign g.38373911G>C - SOX10(NM_006941.4):c.660C>G (p.G220=) - POLR2F_000014 VKGL data sharing initiative Nederland - - - CLASSIFICATION record - - - - - VKGL-NL_AMC
+/+ 1 3 c.671C>G r.(?) p.(Ser224*) - pathogenic (dominant) g.38373900G>C g.37977893G>C Stop at codon 224 - SOX10_000070 - PubMed: Parthev 2012 - - Unknown - - - - - Veronique Pingault
-?/. 1 - c.684C>T r.(?) p.(Pro228=) - likely benign g.38373887G>A - SOX10(NM_006941.4):c.684C>T (p.P228=) - POLR2F_000003 VKGL data sharing initiative Nederland - - - CLASSIFICATION record - - - - - VKGL-NL_AMC
+/+ 1 3i_4 c.698-740_1085delinsCCT r.? p.? - pathogenic (dominant) g.38369818_38370945delinsAGG g.37973811_37974938delinsAGG 697-740_1085delinsCCT - SOX10_000062 - PubMed: Bondurand 2007 - - De novo - - - - - Veronique Pingault
-/. 1 4i c.698-58_698-48del r.(=) p.(=) - benign g.38370253_38370263del g.37974246_37974256del 698-48_698-58del - POLR2F_000023 - PubMed: Pingault 2014 - - Germline no - - - - Johan den Dunnen
+/. 1 - c.698-52_698-42del r.(=) p.(=) - pathogenic g.38370253_38370263del - SOX10(NM_006941.3):c.698-52_698-42delCTCTAACCTGC (p.?) - POLR2F_000023 VKGL data sharing initiative Nederland - - - CLASSIFICATION record - - - - - VKGL-NL_Leiden
-?/. 1 3i c.698-19_698-18dup r.(=) p.(=) - likely benign g.38370233_38370234dup g.37974226_37974227dup SOX10(NM_006941.4):c.698-19_698-18dupCT - SOX10_000112 VKGL data sharing initiative Nederland - - - CLASSIFICATION record - - - - - VKGL-NL_AMC
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