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Because patients with AOx deficiency suffer from more severe symptoms than those with X-ALD, accumulation of VLC-PUFA and/or reduction of DHA may be associated with the severity of peroxisomal diseases.
ACOX1 and GNPAT silencing up-regulated ceramide galactosyltransferase (UGT8) mRNA expression, and down-regulated UDP-glucoseceramide glucosyltransferase (UGCG).
Data show that human ACOX1b isoform is more effective than the ACOX1a isoform in reversing the Acox1 null phenotype in the mouse.
Mutational spectrum of peroxisomal acyl-coenzyme A oxidase deficiency.
report on two new patients with ACOX1 deficiency and mutational analyses
molecular characterization of the ACOX1 gene; identified one SNP and investigated its association in 7 Bos taurus populations; the A1865C SNP of ACOX1 is significantly associated with ultrasound backfat thickness and ultrasound marbling score in cattle
Our data support a link between oxidative stress and the deficiency of Abcd1 or Acox1 peroxisomal proteins.
progressive PPARalpha- and p8-mediated ER stress contribute to the hepatocarcinogenesis in ACOX1(-/-) mice
Loss of Fatty acyl-CoA oxidase leads to increased amounts of PPARalpha, peroxisomal bifunctional enzyme and thiolase in hepatocellular carcinoma
STAT5A binds to -1841 to -1825 of the murine AOX promoter.
The protein encoded by this gene is the first enzyme of the fatty acid beta-oxidation pathway, which catalyzes the desaturation of acyl-CoAs to 2-trans-enoyl-CoAs. It donates electrons directly to molecular oxygen, thereby producing hydrogen peroxide. Defects in this gene result in pseudoneonatal adrenoleukodystrophy, a disease that is characterized by accumulation of very long chain fatty acids. Alternatively spliced transcript variants encoding different isoforms have been identified.
acyl-Coenzyme A oxidase 1, palmitoyl
, peroxisomal acyl-coenzyme A oxidase 1
, palmitoyl-CoA oxidase
, Acyl-coenzyme A oxidase 1, peroxisomal
, acyl-CoA oxidase 1, palmitoyl
, peroxisomal acyl-coenzyme A oxidase 1-like
, acyl-CoA oxidase, straight-chain
, peroxisomal fatty acyl-CoA oxidase
, straight-chain acyl-CoA oxidase
, palmitoyl acyl-CoA oxidase 1
, peroxisomal fatty acyl-coenzyme A oxidase
, peroxisomal straight-chain Acyl-CoA oxidase
, palmitoyl-CoA oxidase 1