Annotation Detail

Information
Associated Genes
POLG FANCI POLGARF
Associated Variants
POLG p.Glu1143Gly (p.E1143G) ( ENST00000442287.6, ENST00000636937.2, ENST00000268124.11 )
POLG p.Glu1143Gly (p.E1143G) ( ENST00000268124.11, ENST00000442287.6, ENST00000636937.2 )
Associated Disease
Progressive external ophthalmoplegia with mitochondrial DNA deletions, autosomal dominant 1 mitochondrial DNA depletion syndrome 4b mitochondrial DNA depletion syndrome 1 sensory ataxic neuropathy, dysarthria, and ophthalmoparesis Progressive sclerosing poliodystrophy Progressive external ophthalmoplegia with mitochondrial DNA deletions, autosomal recessive 1
Source Database
ClinVar
Description
NM_002693.3(POLG):c.3428A>G (p.Glu1143Gly) AND multiple conditions
ClinVar Allele ID
34164
ClinVar RefSeq Alternation Syntax
NM_001126131.2:c.3428A>G
ClinVar RefSeq Alternation Syntax
NM_002693.3:c.3428A>G
Clinical Significance Description
Benign
Clinical Significance Last Update
2021-08-03
Clinical Significance Review Status
criteria provided, single submitter
URL
https://www.ncbi.nlm.nih.gov/clinvar/RCV000755650
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