Overview

Variant ID 1517
Entrez Gene ID 5428
Gene POLG (GeneCards)
Location hg19 15:89866069-89866069
hg38 15:89322838-89322838
Disease Aplastic Anaemia (view all the variants in this disease)
Method HiSeq 2500
Mutation(HGVS format) NC_000015.9:g.89866069 C>A (Genome Assembly: hg19)

Other information

Exon or Intron Exon
Position in protein 777
Amino acid changes in protein G > V
Position in cDNA 2330
Changes in cDNA G > T
mRNA accession NM_001126131.1
mRNA length 4452
Reference length 102531392

Annotations and predictions

MAF in gnomAD genome (version 2.0.1) 0
EIGEN score 0.9249
CADD Raw score (version 1.3) 6.770822 (Deleterious)
FATHMM raw prediction score 0.9725 (Tolerated)
SIFT score 0.002 (Deleterious)
LRT score 0 (Deleterious)
MutationTaster score 1 (Deleterious)
MutatioinAssessor score 3.4 (Deleterious)
PROVEAN score -6.29 (Deleterious)
MetaSVM score 1.099 (Deleterious)
MetaLR score 0.959 (Deleterious)
MCAP score 0.534 (Deleterious)
FitCons score 0.672 (Highly Significant p < 0.003 )
Genomic Evolutionary Rate Profiling (GERP) score 5.44
PhyloP score based on multiple alignment of 100 vertebrates 7.542
PhastCons score based on multiple alignment of 100 vertebrates 1
SiPhy log transformed odds ratio on multiple alignment of 29 mammals 19.261
Deleterious probability by iFish2 0.9939 (Deleterious)
Deleterious probability by DeFine 0.9707 (Deleterious)
Entrez Gene ID 5428 (NCBI Gene)
Official Gene Symbol POLG (GeneCards)
Number of variants in POLG in this database 2 (view all the variants)
Full name DNA polymerase gamma, catalytic subunit
Band 15q26.1
Other IDs Vega: OTTHUMG00000149646
OMIM: 174763
HGNC: HGNC:9179
Ensembl: ENSG00000140521
Other names PEO, MDP1, SCAE, MIRAS, POLG1, POLGA, SANDO, MTDPS4A, MTDPS4B
Summary Mitochondrial DNA polymerase is heterotrimeric, consisting of a homodimer of accessory subunits plus a catalytic subunit. The protein encoded by this gene is the catalytic subunit of mitochondrial DNA polymerase. The encoded protein contains a polyglutamine tract near its N-terminus that may be polymorphic. Defects in this gene are a cause of progressive external ophthalmoplegia with mitochondrial DNA deletions 1 (PEOA1), sensory ataxic neuropathy dysarthria and ophthalmoparesis (SANDO), Alpers-Huttenlocher syndrome (AHS), and mitochondrial neurogastrointestinal encephalopathy syndrome (MNGIE). Two transcript variants encoding the same protein have been found for this gene. [provided by RefSeq, Jul 2008]

Individual #1

Individual ID 28699658.18 (view all the variants in this individual)
Pubmed ID 28699658
Whose mosaic mutation Patient  
Phenotype 3  
Disease Aplastic Anaemia (view all the variants in this disease)
OMIM ID 609135

Publication #1: 28699658

Pubmed ID 28699658
Title Telomere length and somatic mutations in correlation with response to immunosuppressive treatment in aplastic anaemia.
Journal British Journal of Haematology
Publication date 2017.08
Disease Aplastic Anaemia
Number of cases Male cases: 22; Female cases: 1; cases of unknown sex: 4;