Annotation Detail

Information
Associated Genes
KCNQ1
Associated Variants
KCNQ1 p.Arg583Cys (p.R583C) ( ENST00000155840.12, ENST00000526095.2, ENST00000496887.7, ENST00000713725.1, ENST00000646564.2, ENST00000335475.6 )
KCNQ1 p.Arg583Cys (p.R583C) ( ENST00000155840.12, ENST00000335475.6, ENST00000496887.7, ENST00000526095.2, ENST00000646564.2, ENST00000713725.1 )
Associated Disease
Jervell and Lange-Nielsen syndrome 1 Beckwith-Wiedemann syndrome long QT syndrome 1 Atrial fibrillation, familial, 3 Short QT syndrome type 2
Source Database
ClinVar
Description
NM_000218.3(KCNQ1):c.1747C>T (p.Arg583Cys) AND multiple conditions
ClinVar Allele ID
18181
ClinVar RefSeq Alternation Syntax
NM_001406839.1:c.259C>T
ClinVar RefSeq Alternation Syntax
NM_181798.2:c.1366C>T
ClinVar RefSeq Alternation Syntax
NM_001406838.1:c.1207C>T
ClinVar RefSeq Alternation Syntax
NM_001406837.1:c.1477C>T
ClinVar RefSeq Alternation Syntax
NM_001406836.1:c.1651C>T
ClinVar RefSeq Alternation Syntax
NM_000218.3:c.1747C>T
Clinical Significance Description
Likely pathogenic
Clinical Significance Last Update
2018-10-31
Clinical Significance Review Status
criteria provided, single submitter
URL
https://www.ncbi.nlm.nih.gov/clinvar/RCV000762837
ClinVar Disease
Beckwith-Wiedemann syndrome
ClinVar Disease
Long QT syndrome 1
ClinVar Disease
Jervell and Lange-Nielsen syndrome 1
ClinVar Disease
Atrial fibrillation, familial, 3
ClinVar Disease
Short QT syndrome type 2
Observed Origin Sample
unknown
Drugs