GenTIGSA Gene Database on Rare Genetic Disorders

Isovaleryl-CoA dehydrogenase (IVD) Associated conditions:  1. Isovaleryl-CoA dehydrogenase deficiency 
mRNA Information: Transcript Variants and Protein Isoforms
SNo. Status mRNA accession Protein accession Genomic DNA accession (Chromosome) mRNA location on genomic DNA Assembly UniProtkb ID
1 REVIEWED NM_001159508.3 NP_001152980.2 NC_000015.10 (40405794..40435946) Reference GRCh38.p14 Primary Assembly P26440
2 REVIEWED NM_001354597.3 NP_001341526.1 NC_000015.10 (40405794..40435946) Reference GRCh38.p14 Primary Assembly P26440
3 REVIEWED NM_001354598.3 NP_001341527.2 NC_000015.10 (40405794..40435946) Reference GRCh38.p14 Primary Assembly P26440
4 REVIEWED NM_001354599.3 NP_001341528.2 NC_000015.10 (40405794..40435946) Reference GRCh38.p14 Primary Assembly P26440
5 REVIEWED NM_001354600.3 NP_001341529.2 NC_000015.10 (40405794..40435946) Reference GRCh38.p14 Primary Assembly P26440
6 REVIEWED NM_001354601.3 NP_001341530.2 NC_000015.10 (40405794..40435946) Reference GRCh38.p14 Primary Assembly P26440
7 REVIEWED NM_002225.5 NP_002216.3 NC_000015.10 (40405794..40435946) Reference GRCh38.p14 Primary Assembly P26440
8 REVIEWED NR_148925.2 - NC_000015.10 (40405794..40435946) Reference GRCh38.p14 Primary Assembly P26440
9 REVIEWED XR_007064441.1 - NC_000015.10 (40405794..40435946) Reference GRCh38.p14 Primary Assembly P26440
10 REVIEWED XR_007064442.1 - NC_000015.10 (40405794..40435946) Reference GRCh38.p14 Primary Assembly P26440

Transcript variants arise from alternative splicing during gene expression, producing multiple mRNA transcripts from a single gene, each encoding distinct protein isoforms.

PubMed Links for Transcript Variants (Isoforms)
Link 157 reference articles

Variant Information Isovaleryl-CoA dehydrogenase (IVDMutation Visualization Dashboard: Pathogenic variant distribution chart
ClinVar variants:Revealing mutagenic patterns

GO Term of Isovaleryl-CoA dehydrogenase (IVD)
SNo. Reported in speceis Evidence Qualifier GO term Category Pubmed Link
1Homo sapiensIEAenablesbutyryl-CoA dehydrogenase activityFunction
2Homo sapiensIPIenablesprotein bindingFunction32296183 
3Homo sapiensIDAlocated_innucleoplasmComponent
4Homo sapiensIBAis_active_inmitochondrionComponent21873635 
5Homo sapiensIDAlocated_inmitochondrionComponent
6Homo sapiensISSlocated_inmitochondrial matrixComponent
7Homo sapiensTASlocated_inmitochondrial matrixComponent
8Homo sapiensIBAinvolved_inleucine catabolic processProcess21873635 
9Homo sapiensIDAinvolved_inleucine catabolic processProcess7640268 
10Homo sapiensIEAinvolved_inleucine catabolic processProcess
11Homo sapiensISSinvolved_inleucine catabolic processProcess
12Homo sapiensIBAenablesisovaleryl-CoA dehydrogenase activityFunction21873635 
13Homo sapiensIDAenablesisovaleryl-CoA dehydrogenase activityFunction3597357 7640268 
14Homo sapiensISSenablesisovaleryl-CoA dehydrogenase activityFunction
15Homo sapiensIDAinvolved_inbranched-chain amino acid catabolic processProcess7640268 
16Homo sapiensIDAinvolved_infatty acid beta-oxidation using acyl-CoA dehydrogenaseProcess3597357 
17Homo sapiensISSenablesidentical protein bindingFunction
18Homo sapiensIEAenablesflavin adenine dinucleotide bindingFunction