GenTIGSA Gene Database on Rare Genetic Disorders

Variants in ClinVar from 539 of 916 Rare Genetic Disorders of GenTIGS

Deficiency of acetyl-CoA acetyltransferase

An  Autosomal recessive  mode(s) within the Metabolic disorders  category

Pathogenic 1
Pathogenic/Likely pathogenic 3
Uncertain significance 1

Variant name Variant type GRCH38 location Germline classification Molecular consequence dbSNP_ID Submitter
NM_000019.4(ACAT1):c.731-2A>G Single nucleotide variant Chr11:108141603 Pathogenic Splice acceptor variant rs752216720 .Kasturba Medical College, Manipal, Kasturba Medical College, Manipal, Manipal Academy of Higher Education, Manipal, India
NM_000019.4(ACAT1):c.238+2T>A Single nucleotide variant Chr11:108133939 Pathogenic/Likely pathogenic Splice donor variant|intron variant rs2135334862 .Kasturba Medical College, Manipal, Kasturba Medical College, Manipal, Manipal Academy of Higher Education, Manipal, India
NM_000019.4(ACAT1):c.1124A>G (p.Asn375Ser) Single nucleotide variant Chr11:108146320 Pathogenic/Likely pathogenic Missense variant rs373771053 .Kasturba Medical College, Manipal, Kasturba Medical College, Manipal, Manipal Academy of Higher Education, Manipal, India
NM_000019.4(ACAT1):c.578T>G (p.Met193Arg) Single nucleotide variant Chr11:108139040 Pathogenic/Likely pathogenic Missense variant rs541517496 .Lifecell International Pvt. Ltd
.Neuberg Centre For Genomic Medicine, NCGM
NM_000019.4(ACAT1):c.1199A>G (p.His400Arg) Single nucleotide variant Chr11:108147305 Uncertain significance Missense variant rs761086326 .Foundation for Research in Genetics and Endocrinology, FRIGE's Institute of Human Genetics

Indian Institutes & Variant Submission Overview
Chromosome-wise Variant Distribution