ACOX2

Chr 3p14.3

acyl-CoA oxidase 2

Aliases:: BRCACOX, BRCOX, THCCox
MANE:: ENST00000302819.10

OMIM: 601641 ↗NCBI: 8309 ↗Ensembl ↗

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Predicted protein structure

AlphaFold predicted structure

ACOX2 · Q99424

v62025-08-01

Predicted Aligned Error matrix for ACOX2 — diagonal shows residue index, colour shows pairwise prediction confidence (lower is better) — Predicted Aligned Error

Mean pLDDT

94.4/ 100

Very high

681 residues

Confidence breakdown

Very high(≥ 90)91%
Confident(70–90)6%
Low(50–70)2%
Very low(< 50)1%

Open interactive 3D viewer

AlphaFold (Jumper et al., 2021) · CC BY 4.0

Clinical relevance (Genomics England PanelApp)

Diagnostic Grade (Green)

Likely inborn error of metabolism
BIALLELIC, autosomal or pseudoautosomal
Intellectual disability
BIALLELIC, autosomal or pseudoautosomal

Disease associations (Open Targets)

Disorder of bile acid synthesis
0.70
acute tonsillitis
0.08
liver cancer
0.07
posterior cortical atrophy
0.06
gallbladder disease 1
0.06
Senior-Boichis syndrome
0.06
Congenital bile acid synthesis defect type 3
0.06
non-small cell lung carcinoma
0.05
progressive familial intrahepatic cholestasis type 2
0.05
cholestasis, progressive familial intrahepatic, 4
0.05

Score is the Open Targets composite evidence score (0-1). Higher = stronger gene-disease association.

Protein function (UniProt)

Peroxisomal acyl-coenzyme A oxidase 2

Involved in peroxisomal beta-oxidation of branched-chain fatty acids (BCFAs) and bile acids intermediates. Catalyzes the initial and rate-limiting dehydrogenation step that removes two hydrogen molecules and introduces a trans double bond between the alpha and beta carbons of the acyl CoA molecules while generating hydrogen peroxide as a by-product. Oxidizes its substrates in a stereospecific way and can only desaturate isomers carrying (2S)-methyl groups (By similarity) (PubMed:29287774). In brown adipose tissue peroxisomes, mediates beta-oxidation of monomethyl branched-chain fatty acids with iso configuration (mmBCFAs) as part of a substrate synthesis and oxidation cycle fueled by FASN-dependent de novo mmBCFA synthesis. The mmBCFA catabolism is coupled to AMPK activation and mitochondrial ATP production as a thermogenic adaptation mechanism to maintain the body temperature in cold environment (By similarity). Metabolizes multi-methyl-branched fatty acyl-CoA esters such as (2S)-pristanoyl-CoA, displaying redundancy with ACOX3 (PubMed:29287774). In the liver, mediates side-chain beta-oxidation of C27 bile acyl-CoA intermediates carrying a (25S)-methyl group to yield 24,25-trans unsaturated derivatives as part of peroxisomal bile acid synthesis pathway (PubMed:27884763, PubMed:29287774). Can oxidize straight-chain fatty acyl CoAs such as C10-CoA and C16-CoA with low efficiency (PubMed:29287774)