• Specifically, it is involved in the first step of a process called the peroxisomal fatty acid beta-oxidation pathway. (medlineplus.gov)
  • 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. (wikipedia.org)
  • Catalase, fatty-acyl-coenzyme-A-oxidase (FAO), and total peroxisomal beta oxidation activities were determined. (cdc.gov)
  • Dicamba treatment at 1% increased FAO activity in male rats, peroxisomal beta oxidation, lauric-acid-hydroxylase activity, and CYP4A reactive protein in female and male rats, and yielded an 80 kilodalton protein as indicated by gel electrophoresis. (cdc.gov)
  • peroxisomal fatty acid beta-oxidation. (swisspalm.org)
  • Involved in the initial and rate-limiting step of peroxisomal beta-oxidation of straight-chain saturated and unsaturated very-long-chain fatty acids (PubMed:7876265, PubMed:15060085, PubMed:17458872, PubMed:17603022, PubMed:32169171, PubMed:33234382). (swisspalm.org)
  • b Identification of putative peroxisomal enzymes and enyzmes of mitochondrial beta-oxidation. (biomedcentral.com)
  • Another major function of the peroxisomal beta-oxidation system is related to the biosynthesis of polyunsaturated fatty acid (C22:6w3). (medscape.com)
  • Futile cycling of intermediates of fatty acid biosynthesis toward peroxisomal beta-oxidation in Saccharomyces cerevisiae. (unil.ch)
  • Peroxisomal acyl-CoA oxidase deficiency is a disorder that causes deterioration of nervous system functions (neurodegeneration) beginning in infancy. (medlineplus.gov)
  • Newborns with peroxisomal acyl-CoA oxidase deficiency have weak muscle tone (hypotonia) and seizures. (medlineplus.gov)
  • Most babies with peroxisomal acyl-CoA oxidase deficiency learn to walk and begin speaking, but they experience a gradual loss of these skills (developmental regression), usually beginning between the ages of 1 and 3. (medlineplus.gov)
  • Most children with peroxisomal acyl-CoA oxidase deficiency do not survive past early childhood. (medlineplus.gov)
  • Peroxisomal acyl-CoA oxidase deficiency is a rare disorder. (medlineplus.gov)
  • Peroxisomal acyl-CoA oxidase deficiency is caused by mutations in the ACOX1 gene, which provides instructions for making an enzyme called peroxisomal straight-chain acyl-CoA oxidase. (medlineplus.gov)
  • It is unclear exactly how VLCFA accumulation leads to the specific features of peroxisomal acyl-CoA oxidase deficiency. (medlineplus.gov)
  • Leukodystrophy is likely involved in the development of the neurological abnormalities that occur in peroxisomal acyl-CoA oxidase deficiency. (medlineplus.gov)
  • Cytochrome-c Oxidase Deficiency" is a descriptor in the National Library of Medicine's controlled vocabulary thesaurus, MeSH (Medical Subject Headings) . (uams.edu)
  • This graph shows the total number of publications written about "Cytochrome-c Oxidase Deficiency" by people in UAMS Profiles by year, and whether "Cytochrome-c Oxidase Deficiency" was a major or minor topic of these publications. (uams.edu)
  • Below are the most recent publications written about "Cytochrome-c Oxidase Deficiency" by people in Profiles over the past ten years. (uams.edu)
  • Sudden death in medium chain acyl-coenzyme a dehydrogenase deficiency (MCADD) despite newborn screening. (musc.edu)
  • Catalyzes the desaturation of fatty acyl-CoAs such as palmitoyl-CoA (hexadecanoyl-CoA) to 2-trans-enoyl-CoAs ((2E)-enoyl-CoAs) such as (2E)-hexadecenoyl-CoA, and donates electrons directly to molecular oxygen (O(2)), thereby producing hydrogen peroxide (H(2)O(2)) (PubMed:7876265, PubMed:17458872, PubMed:17603022). (swisspalm.org)
  • The NAD+ reduction was measured spectrophotometrically at 340 nm for 5 min by addition of 0.01 mM palmitoyl-CoA to the assay mixture containing 47 mM Tris·HCl (pH 8.0) 0.2 mM NAD+ 1 mM dithiothreitol 0.0075% (wt/vol) bovine serum albumin 0.01% (wt/vol) Triton X-100 0.1 mM coenzyme A 0.01 mM flavin adenine dinucleotide and 1 mM KCN. (cancerhugs.com)
  • Peroxisomes of plant leaves contain catalaze together with the enzymes of glycolate pathway, as glycolate oxidase, glutamate glyoxylate, serine-glyoxylate and aspirate-a-ketoglutarate aminotransferases, hydroxyl pyruvate reductase and malic dehydrogenase. (exama2z.in)
  • The effects of dietary dicamba (1918009) on the induction of hepatic peroxisomal and microsomal enzymes were investigated in rats. (cdc.gov)
  • Hepatic peroxisomal fatty acid β-oxidation was suppressed by ethanol in associated with inhibition of acyl-coenzyme A oxidase 1. (cancerhugs.com)
  • The initial description of the peroxisome (originally termed the microbody) appeared in 1954 in a doctoral thesis about mouse kidneys, almost 10 years after the first case description of peroxisomal disease was published. (medscape.com)
  • In photorespiration, glycolic acid (glycolate), a two-carbon product of photosynthesis is released from chloroplasts and oxidized into and H 2 O 2 by a peroxisomal enzyme called glycolic acid oxidase. (exama2z.in)
  • Acyl carrier protein-like family protein. (go.jp)
  • protein_coding" "PTI_03G03170.1","No alias","Phaeodactylum tricornutum","Enoyl-(Acyl carrier protein) reductase [Interproscan]. (ntu.edu.sg)
  • The expression of regulatory molecules, TGF-β1/2, phospho-Akt (Ser473), PPARα, sterol regulatory element-binding protein 1 (SREBP-1), fatty acid synthase (FASN), hormone-sensitive lipase (HSL), and acyl dehydrogenases was analyzed in virus-infected hepatocytes. (academic-accelerator.com)
  • It has also been shown that PPARs can induce transcription of acyl coenzyme A oxidase and cytochrome P450 A6 (CYP450 A6) through interaction with specific response elements. (thermofisher.com)
  • Isoform 1 shows highest activity against medium-chain fatty acyl-CoAs and activity decreases with increasing chain length. (swisspalm.org)
  • Isoform 2 is active against a much broader range of substrates and shows activity towards very long-chain acyl-CoAs. (swisspalm.org)
  • ACOX1 gene mutations prevent the peroxisomal straight-chain acyl-CoA oxidase enzyme from breaking down VLCFAs efficiently. (medlineplus.gov)
  • Peroxisomal acyl-coenzyme A oxidase 1 is an enzyme that in humans is encoded by the ACOX1 gene. (wikipedia.org)
  • The membrane was probed with polyclonal antibody against acyl-coenzyme A oxidase 1 (ACOX1 Proteintech Chicago IL). (cancerhugs.com)
  • This was accompanied by an impairment of mitochondrial function with reduced expression of carnitine palmitoyltransferase I and an induction of acyl-coenzyme A oxidase-1 (ACOX1), oxidative stress and extracellular signal-regulated kinase (ERK) activation. (bmj.com)
  • Catalyzes the desaturation of acyl-CoAs to 2-trans-enoyl-CoAs. (swisspalm.org)
  • Peroxisomal acyl coenzyme A oxidase 2 converts 3a,7a-dihydroxy-5b-cholestanoyl-CoA into 3a,7a-dihydoxy-5b-cholest-24-enoyl-CoA. (smpdb.ca)
  • With the help of water, peroxisomal multifunctional enzyme type 2 turns 3a,7a-dihydoxy-5b-cholest-24-enoyl-CoA into 3a,7a,24-trihydoxy-5b-cholestanoyl-CoA. (smpdb.ca)
  • The coenzyme of various aerobic dehydrogenases, e.g. (rcsb.org)
  • Then, bile acyl-CoA synthetase teams up with 3a,7a-dihydroxycoprostanic acid to create 3a,7a-dihydroxy-5b-cholestanoyl-CoA. (smpdb.ca)
  • Zellweger described the first case of a peroxisomal disorder in the 1940s. (medscape.com)
  • The authors conclude that dicamba at a 1% dietary level yielded weak or moderate increases in peroxisomal enzymes, and in CYP4A content and activity. (cdc.gov)
  • NADPH oxidase 4 (NOX4), a source of cellular superoxide anion, has multiple biological functions that may be of importance in inflammation, and in the pathogenesis of human metabolic diseases, including diabetes. (docksci.com)
  • This compound then uses peroxisomal multifunctional enzyme type 2 to create chenodeoxycholoyl-CoA. (smpdb.ca)
  • Shows highest activity against medium-chain fatty acyl-CoAs. (swisspalm.org)
  • Similar to Acyl-coenzyme A oxidase 1, peroxisomal (EC 1.3.3.6) (AOX 1) (Long- chain acyl-CoA oxidase) (AtCX1). (go.jp)
  • Rosiglitazone elevated plasma adiponectin level and normalized peroxisomal fatty acid β-oxidation rate. (cancerhugs.com)
  • Furthermore, irinotecan-treated patients revealed higher hepatic ERK activity, expression of pro-inflammatory genes and markers indicative for a shift to peroxisomal fatty acid oxidation and an impaired autophagic flux. (bmj.com)