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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 (show GNPAT Proteins) silencing up-regulated ceramide galactosyltransferase (show GGTA1 Proteins) (UGT8 (show UGT8 Proteins)) mRNA expression, and down-regulated UDP-glucoseceramide glucosyltransferase (UGCG (show UGCG Proteins)).
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 (show SOAT2 Proteins) 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 (show ABCD1 Proteins) or Acox1 peroxisomal proteins.
progressive PPARalpha (show PPARA Proteins)- and p8-mediated ER stress contribute to the hepatocarcinogenesis in ACOX1(-/-) mice
Loss of Fatty acyl-CoA (show GNPAT Proteins) oxidase leads to increased amounts of PPARalpha (show PPARA Proteins), peroxisomal bifunctional enzyme (show EHHADH Proteins) and thiolase (show HADHB Proteins) in hepatocellular carcinoma
STAT5A (show STAT5A Proteins) 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