Unique variants in the SIX1 gene

Information The variants shown are described using the NM_005982.3 transcript reference sequence.

44 entries on 1 page. Showing entries 1 - 44.
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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.? r.(?) p.(?) - likely pathogenic g.? g.? SIX1 arr[hg19] 14q22.2q23.2(54331256-63789238)x1 (including SIX1, OTX2) - SERPINA1_000009 no protein change given, heterozygous PubMed: Zanolli 2020 - - Unknown ? - - - - LOVD
?/. 1 - c.13C>G r.(?) p.(Pro5Ala) - VUS g.61115895G>C g.60649177G>C - - SIX1_000008 VKGL data sharing initiative Nederland - - - CLASSIFICATION record - - - - - VKGL-NL_Nijmegen
+?/. 2 - c.31C>T r.(?) p.(Gln11Ter) - likely pathogenic g.61115877G>A g.60649159G>A - - SIX1_000039 - PubMed: Calpena 2022 - - Germline - - - - - Johan den Dunnen
+?/. 1 - c.40G>C r.(?) p.(Val14Leu) - likely pathogenic g.61115868C>G g.60649150C>G - - SIX1_000038 - PubMed: Calpena 2022 - - De novo - - - - - Johan den Dunnen
+/+ 1 1 c.50T>A r.(?) p.(Val17Glu) - pathogenic g.61115858A>T g.60649140A>T - - SIX1_000027 - MORL Deafness Variation Database, PubMed: Smith 1993, PubMed: Kochhar 2008, PubMed: Patrick 2009 - - SUMMARY record - - - - - Global Variome, with Curator vacancy
-?/. 1 - c.58G>C r.(?) p.(Val20Leu) - likely benign g.61115850C>G g.60649132C>G - - SIX1_000007 VKGL data sharing initiative Nederland - - - CLASSIFICATION record - - - - - VKGL-NL_Nijmegen
+/., +?/. 2 - c.64C>T r.(?) p.(Gln22Ter) - likely pathogenic, pathogenic g.61115844G>A g.60649126G>A SIX1(NM_005982.3):c.64C>T (p.Q22*) - SIX1_000002 VKGL data sharing initiative Nederland PubMed: Calpena 2022 - - CLASSIFICATION record, Germline - - - - - Johan den Dunnen, VKGL-NL_Rotterdam
?/. 1 - c.154G>T r.(?) p.(Ala52Ser) - VUS g.61115754C>A g.60649036C>A SIX1(NM_005982.3):c.154G>T (p.A52S) - SIX1_000013 VKGL data sharing initiative Nederland - - - CLASSIFICATION record - - - - - VKGL-NL_Rotterdam
?/. 1 - c.155C>T r.(?) p.(Ala52Val) - VUS g.61115753G>A g.60649035G>A SIX1(NM_005982.3):c.155C>T (p.A52V) - SIX1_000012 VKGL data sharing initiative Nederland - - - CLASSIFICATION record - - - - - VKGL-NL_Rotterdam
-?/. 1 - c.162G>A r.(?) p.(Ala54=) - likely benign g.61115746C>T g.60649028C>T SIX1(NM_005982.3):c.162G>A (p.A54=) - SIX1_000011 VKGL data sharing initiative Nederland - - - CLASSIFICATION record - - - - - VKGL-NL_Rotterdam
-?/. 1 - c.180C>G r.(?) p.(Arg60=) - likely benign g.61115728G>C - SIX1(NM_005982.3):c.180C>G (p.R60=) - SIX1_000029 VKGL data sharing initiative Nederland - - - CLASSIFICATION record - - - - - VKGL-NL_Rotterdam
?/. 1 - c.197T>G r.(?) p.(Leu66Arg) - VUS g.61115711A>C - - - SIX1_000044 VKGL data sharing initiative Nederland - - - CLASSIFICATION record - - - - - VKGL-NL_Nijmegen
-?/. 1 - c.208C>T r.(?) p.(Leu70=) - likely benign g.61115700G>A - - - SIX1_000042 VKGL data sharing initiative Nederland - - - CLASSIFICATION record - - - - - VKGL-NL_Nijmegen
+/+ 1 1 c.218A>C r.(?) p.(His73Pro) - pathogenic g.61115690T>G g.60648972T>G - - SIX1_000026 - MORL Deafness Variation Database, PubMed: Kochhar 2008 - - SUMMARY record - - - - - Global Variome, with Curator vacancy
+/. 1 - c.273_274insC r.(?) p.(Tyr92Leufs*62) - pathogenic g.61115634_61115635insG g.60648916_60648917insG - - SIX1_000030 - PubMed: Heidet 2017 - - Germline - - - - - Johan den Dunnen
?/. 1 - c.277G>A r.(?) p.(Val93Met) - VUS g.61115631C>T - SIX1(NM_005982.3):c.277G>A (p.V93M) - SIX1_000033 VKGL data sharing initiative Nederland - - - CLASSIFICATION record - - - - - VKGL-NL_Rotterdam
+?/. 1 - c.316G>A r.(?) p.(Val106Met) - likely pathogenic g.61115592C>T - - - SIX1_000034 VKGL data sharing initiative Nederland - - - CLASSIFICATION record - - - - - VKGL-NL_Nijmegen
+/+ 1 1 c.317T>G r.(?) p.(Val106Gly) - pathogenic g.61115591A>C g.60648873A>C - - SIX1_000025 - MORL Deafness Variation Database, PubMed: Smith 1993, PubMed: Kochhar 2008, PubMed: Patrick 2009 - - SUMMARY record - - - - - Global Variome, with Curator vacancy
+/+ 1 1 c.328C>G r.(?) p.(Arg110Gly) - pathogenic g.61115580G>C g.60648862G>C - - SIX1_000023 - MORL Deafness Variation Database, PubMed: Iglesias 2014 - - SUMMARY record - - - - - Global Variome, with Curator vacancy
+/+, +/., +?/. 3 1 c.328C>T r.(?) p.(Arg110Trp) - likely pathogenic, pathogenic g.61115580G>A g.60648862G>A - - SIX1_000024 - MORL Deafness Variation Database, PubMed: Smith 1993, PubMed: Ruf 2004, PubMed: Patrick 2009, 1 more item - rs80356459 De novo, SUMMARY record, Unknown - - - - - Global Variome, with Curator vacancy, Johan den Dunnen, MobiDetails
+/+ 1 1 c.329G>A r.(?) p.(Arg110Gln) - pathogenic g.61115579C>T g.60648861C>T - - SIX1_000022 - MORL Deafness Variation Database, PubMed: Kochhar 2008 - - SUMMARY record - - - - - Global Variome, with Curator vacancy
+?/. 1 - c.334C>G r.(?) p.(Arg112Gly) - likely pathogenic g.61115574G>C g.60648856G>C - - SIX1_000005 VKGL data sharing initiative Nederland - - - CLASSIFICATION record - - - - - VKGL-NL_Nijmegen
+/+ 1 1 c.334C>T r.(?) p.(Arg112Cys) - pathogenic g.61115574G>A g.60648856G>A - - SIX1_000021 - MORL Deafness Variation Database, PubMed: Smith 1993, PubMed: Kochhar 2008, PubMed: Patrick 2009 - - SUMMARY record - - - - - Global Variome, with Curator vacancy
+?/. 1 - c.353C>T r.(?) p.(Pro118Leu) - likely pathogenic g.61115555G>A - - - SIX1_000045 VKGL data sharing initiative Nederland - - - CLASSIFICATION record - - - - - VKGL-NL_Nijmegen
+/+ 1 1 c.364T>A r.(?) p.(Trp122Arg) - pathogenic g.61115544A>T g.60648826A>T - - SIX1_000020 - MORL Deafness Variation Database, PubMed: Smith 1993, PubMed: Sanggaard 2007 - - SUMMARY record - - - - - Global Variome, with Curator vacancy
?/. 1 - c.366G>C r.(?) p.(Trp122Cys) - VUS g.61115542C>G - - - SIX1_000032 VKGL data sharing initiative Nederland - - - CLASSIFICATION record - - - - - VKGL-NL_Nijmegen
+?/. 1 - c.368A>G r.(?) p.(Asp123Gly) - likely pathogenic g.61115540T>C - - - SIX1_000043 VKGL data sharing initiative Nederland - - - CLASSIFICATION record - - - - - VKGL-NL_Nijmegen
+/+ 1 1 c.373G>A r.(?) p.(Glu125Lys) - pathogenic g.61115535C>T g.60648817C>T - - SIX1_000019 - MORL Deafness Variation Database, PubMed: Shearer 1993, PubMed: Mosrati 2011 - - SUMMARY record - - - - - Global Variome, with Curator vacancy
+?/. 1 - c.373G>T r.(?) p.(Glu125Ter) - likely pathogenic g.61115535C>A g.60648817C>A - - SIX1_000037 - PubMed: Calpena 2022 - - Germline - - - - - Johan den Dunnen
+?/. 1 - c.374A>T r.(?) p.(Glu125Val) - likely pathogenic g.61115534T>A g.60648816T>A - - SIX1_000004 VKGL data sharing initiative Nederland - - - CLASSIFICATION record - - - - - VKGL-NL_Nijmegen
+/+, +/. 4 1 c.386A>G r.(?) p.(Tyr129Cys) - pathogenic, pathogenic (dominant) g.61115522T>C g.60648804T>C SIX1(NM_005982.3):c.386A>G (p.Y129C) - SIX1_000010 VKGL data sharing initiative Nederland PubMed: Krug 2011, 1 more item - - CLASSIFICATION record, Germline, SUMMARY record - - - - - Global Variome, with Curator vacancy, Johan den Dunnen, VKGL-NL_Rotterdam, VKGL-NL_Nijmegen
+/+, +?/. 2 1 c.397_399del r.(?) p.(Glu133del) - likely pathogenic, pathogenic g.61115511_61115513del g.60648793_60648795del - - SIX1_000014 VKGL data sharing initiative Nederland 1 more item - - CLASSIFICATION record, SUMMARY record - - - - - Global Variome, with Curator vacancy, VKGL-NL_Nijmegen
+?/. 1 - c.452C>T r.(?) p.(Pro151Leu) - likely pathogenic g.61115456G>A g.60648738G>A - - SIX1_000036 - PubMed: Calpena 2022 - - Germline - - - - - Johan den Dunnen
?/. 1 - c.507C>T r.(?) p.(Ser169=) - VUS g.61115401G>A g.60648683G>A - - SIX1_000003 VKGL data sharing initiative Nederland - - - CLASSIFICATION record - - - - - VKGL-NL_Nijmegen
+?/. 1 - c.513G>A r.(?) p.(Trp171Ter) - likely pathogenic g.61115395C>T g.60648677C>T - - SIX1_000035 - PubMed: Calpena 2022 - - Germline - - - - - Johan den Dunnen
?/. 1 - c.518A>C r.(?) p.(Lys173Thr) - VUS g.61115390T>G - SIX1(NM_005982.3):c.518A>C (p.K173T) - SIX1_000028 VKGL data sharing initiative Nederland - - - CLASSIFICATION record - - - - - VKGL-NL_Rotterdam
+?/. 1 - c.523C>T r.(?) p.(Arg175Trp) - likely pathogenic g.61115385G>A - - - SIX1_000046 VKGL data sharing initiative Nederland - - - CLASSIFICATION record - - - - - VKGL-NL_Nijmegen
?/. 1 - c.533G>A r.(?) p.(Arg178Lys) - VUS g.61115375C>T - - - SIX1_000041 VKGL data sharing initiative Nederland - - - CLASSIFICATION record - - - - - VKGL-NL_Nijmegen
+?/+? 1 1i c.560+1G>C r.spl? p.? - likely pathogenic g.61115347C>G g.60648629C>G - - SIX1_000018 - MORL Deafness Variation Database, PubMed: Smith 1993 - - SUMMARY record - - - - - Global Variome, with Curator vacancy
+?/. 1 1i c.560+3A>T r.spl? p.? - likely pathogenic g.61115345T>A g.60648627T>A - - SIX1_000017 - PubMed: Krug 2011 - - Germline/De novo (untested) - - - - - Global Variome, with Curator vacancy
-?/. 4 - c.578A>T r.(?) p.(Asn193Ile) - likely benign g.61113278T>A g.60646560T>A SIX1(NM_005982.3):c.578A>T (p.N193I, p.(Asn193Ile)), SIX1(NM_005982.4):c.578A>T (p.N193I) - SIX1_000001 VKGL data sharing initiative Nederland - - - CLASSIFICATION record - - - - - VKGL-NL_Leiden, VKGL-NL_Rotterdam, VKGL-NL_Utrecht, VKGL-NL_Nijmegen
-?/. 1 - c.615T>G r.(?) p.(Pro205=) - likely benign g.61113241A>C - SIX1(NM_005982.4):c.615T>G (p.P205=) - SIX1_000040 VKGL data sharing initiative Nederland - - - CLASSIFICATION record - - - - - VKGL-NL_Utrecht
?/+, ?/. 2 2 c.679G>T r.(?) p.(Asp227Tyr) - likely benign, VUS g.61113177C>A g.60646459C>A - - SIX1_000016 VKGL data sharing initiative Nederland PubMed: Weber 2006 - - CLASSIFICATION record, Germline - - - - - Global Variome, with Curator vacancy, VKGL-NL_Nijmegen
?/. 1 2 c.746C>T r.(?) p.(Pro249Leu) - likely benign g.61113110G>A g.60646392G>A - - SIX1_000015 - PubMed: Krug 2011 - - Germline/De novo (untested) - - - - - Global Variome, with Curator vacancy
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