Overview

Variant ID 29771
Entrez Gene ID 5019
Gene OXCT1 (GeneCards)
Location hg19 5:41850175-41850175
hg38 5:41850073-41850073
Disease Autism Spectrum Disorders (view all the variants in this disease)
Method NextSeq500
Mutation(HGVS format) NC_000005.9:g.41850175 T>C (Genome Assembly: hg19)

Other information

Exon or Intron Exon
Position in protein NA
Amino acid changes in protein NA > NA
Position in cDNA NA
Changes in cDNA NA > NA
mRNA accession NA
mRNA length NA
Reference length 180915260

Annotations and predictions

MAF in gnomAD genome (version 2.0.1) 0
EIGEN score 0.9357
CADD Raw score (version 1.3) 5.604958 (Deleterious)
FATHMM raw prediction score 0.99655 (Tolerated)
SIFT score 0.003 (Deleterious)
LRT score 0.001 (Deleterious)
MutationTaster score 1 (Deleterious)
MutatioinAssessor score 3.81 (Deleterious)
PROVEAN score -8 (Deleterious)
MetaSVM score 0.803 (Deleterious)
MetaLR score 0.771 (Deleterious)
MCAP score 0.447 (Deleterious)
FitCons score 0.719 (Highly Significant p < 0.003 )
Genomic Evolutionary Rate Profiling (GERP) score 5.12
PhyloP score based on multiple alignment of 100 vertebrates 7.718
PhastCons score based on multiple alignment of 100 vertebrates 1
SiPhy log transformed odds ratio on multiple alignment of 29 mammals 15.093
Deleterious probability by iFish2 0.9974 (Deleterious)
Deleterious probability by DeFine 0.9491 (Deleterious)
Entrez Gene ID 5019 (NCBI Gene)
Official Gene Symbol OXCT1 (GeneCards)
Number of variants in OXCT1 in this database 1 (view all the variants)
Full name 3-oxoacid CoA-transferase 1
Band 5p13.1
Other IDs Vega: OTTHUMG00000094783
OMIM: 601424
HGNC: HGNC:8527
Ensembl: ENSG00000083720
Other names OXCT, SCOT
Summary This gene encodes a member of the 3-oxoacid CoA-transferase gene family. The encoded protein is a homodimeric mitochondrial matrix enzyme that plays a central role in extrahepatic ketone body catabolism by catalyzing the reversible transfer of coenzyme A from succinyl-CoA to acetoacetate. Mutations in this gene are associated with succinyl CoA:3-oxoacid CoA transferase deficiency. [provided by RefSeq, Jul 2008]

Individual #1

Individual ID 28867142.35 (view all the variants in this individual)
Pubmed ID 28867142
Whose mosaic mutation Male Patient  
Origin of mosaic mutation in patients de novo
Phenotype 3  
Disease Autism Spectrum Disorders (view all the variants in this disease)
OMIM ID 209850

Publication #1: 28867142

Pubmed ID 28867142
Title Exonic Mosaic Mutations Contribute Risk for Autism Spectrum Disorder
Journal American Journal of Human Genetics
Publication date 2017.08
Disease Autism Spectrum Disorders
Incidence 0.01
Number of cases cases of unknown sex: 247;