Annotation Detail

Information
Associated Genes
POLG POLGARF
Associated Variants
POLG p.Arg852Cys (p.R852C) ( ENST00000442287.6, ENST00000636937.2, ENST00000268124.11 )
POLG p.Arg852Cys (p.R852C) ( ENST00000268124.11, ENST00000442287.6, ENST00000636937.2 )
Associated Disease
Progressive external ophthalmoplegia with mitochondrial DNA deletions, autosomal recessive 1 mitochondrial DNA depletion syndrome 1 mitochondrial DNA depletion syndrome 4b Progressive sclerosing poliodystrophy Progressive external ophthalmoplegia with mitochondrial DNA deletions, autosomal dominant 1 sensory ataxic neuropathy, dysarthria, and ophthalmoparesis
Source Database
ClinVar
Description
NM_002693.3(POLG):c.2554C>T (p.Arg852Cys) AND multiple conditions
ClinVar Allele ID
202960
ClinVar RefSeq Alternation Syntax
NM_002693.3:c.2554C>T
ClinVar RefSeq Alternation Syntax
NM_001126131.2:c.2554C>T
Clinical Significance Description
Pathogenic
Clinical Significance Last Update
2018-10-31
Clinical Significance Review Status
criteria provided, single submitter
URL
https://www.ncbi.nlm.nih.gov/clinvar/RCV000762953
ClinVar Disease
Progressive sclerosing poliodystrophy
ClinVar Disease
Progressive external ophthalmoplegia with mitochondrial DNA deletions, autosomal dominant 1
ClinVar Disease
Sensory ataxic neuropathy, dysarthria, and ophthalmoparesis
ClinVar Disease
Mitochondrial DNA depletion syndrome 1
ClinVar Disease
Mitochondrial DNA depletion syndrome 4b
ClinVar Disease
Progressive external ophthalmoplegia with mitochondrial DNA deletions, autosomal recessive 1
Observed Origin Sample
unknown
Drugs