Annotation Detail

Information
Associated Genes
POLG POLGARF
Associated Variants
POLG p.Thr251Ile (p.T251I) ( ENST00000636937.2, ENST00000268124.11, ENST00000442287.6 )
POLG p.Thr251Ile (p.T251I) ( ENST00000268124.11, ENST00000442287.6, ENST00000636937.2 )
Associated Disease
mitochondrial DNA depletion syndrome 4b Progressive external ophthalmoplegia with mitochondrial DNA deletions, autosomal recessive 1 Progressive sclerosing poliodystrophy sensory ataxic neuropathy, dysarthria, and ophthalmoparesis
Source Database
ClinVar
Description
NM_002693.3(POLG):c.752C>T (p.Thr251Ile) AND multiple conditions
URL
https://www.ncbi.nlm.nih.gov/clinvar/RCV003458331
ClinVar Allele ID
28542
ClinVar RefSeq Alternation Syntax
NM_001126131.2:c.752C>T
ClinVar RefSeq Alternation Syntax
NM_002693.3:c.752C>T
Clinical Significance Description
not provided
Clinical Significance Review Status
no assertion provided
ClinVar Disease
Progressive sclerosing poliodystrophy
ClinVar Disease
Sensory ataxic neuropathy, dysarthria, and ophthalmoparesis
ClinVar Disease
Mitochondrial DNA depletion syndrome 4b
ClinVar Disease
Progressive external ophthalmoplegia with mitochondrial DNA deletions, autosomal recessive 1
Observed Origin Sample
unknown
Observed Origin Sample
paternal
Drugs