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122 Possible Causes for 5 aminopentanamide, L lysine, acetyl CoA, degradation, to, via

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  • Cytosolic Acetoacetyl-CoA Thiolase Deficiency

    CoA Thiolase, Acetoacetyl; C- Acetyltransferase, Acetyl- CoA; Acetyltransferase, Acetyl- CoA; Acetyl CoA C Acetyltransferase; Acetyl CoA Acetyltransferase Substance CAS Registry[] Each thiolase catalyzes this reaction in both directions, but the equilibrium is far much favoring the degradative direction (K eq is 10 5 , favoring the degradation).[] Deficiency of lyase Deficiency of lysine-tRNA ligase Deficiency of lysolecithin acylmutase Deficiency of lysophospholipase Deficiency of lysozyme Deficiency of malate dehydrogenase[] To view the fulltext, please log in To view the pdf, please log in Buy FullText & PDF Unlimited re-access via MyKarger Unrestricted printing, no saving restrictions for personal[] Swiss-Prot for ACAT2 Gene Disorder Aliases PubMed IDs acat2 deficiency cytosolic acetoacetyl-coa thiolase deficiency - elite association - COSMIC cancer census association via[] Insulin inhibits Epinephrine activate (via cAMP) In what state is HS Lipase active? Why can't glycerol formed in adipose be used there?[]

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  • Acetyl-CoA Carboxylase Deficiency

    An important gene associated with Acetyl-Coa Carboxylase Deficiency is ACACA (Acetyl-CoA Carboxylase Alpha).[] […] and elongation of fatty acids in mammals 85 Chapter 5 Fatty acid oxidation and its regulation 113 Chapter 6 Fatty acid biosynthesis in higher plants 155 Chapter 7 Lipid degradation[] […] dipeptidase Deficiency of L-serine dehydratase Deficiency of lyase Deficiency of lysine racemase Deficiency of lysine-tRNA ligase Deficiency of lysolecithin acylmutase Deficiency[] By comparison, C4 carbon fixation via PEP carboxylase is more efficient. Humans express the following two propionyl-CoA carboxylase isozymes:[] As shown below, tiglyl CoA is an intermediate in the metabolism of isoleucine, and normally is metabolised onwards, via propionyl CoA to yield succinyl COA, an intermediate[] All thiolases, whether they are biosynthetic or degradative in vivo, preferentially catalyze the degradation of 3-ketoacyl-CoA to form acetyl-CoA and a shortened acyl-CoA[]

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  • Pipecolic Acidemia

    See also AASDHPPT PHYH References External links Definition in v t e Inborn error of amino acid metabolism ( E70–E72 , 270 ) K acetyl-CoA Lysine /straight[] Background: The essential amino acid lysine can be degraded via two pathways, the saccharopine pathway and the pipecolic acid (PA) pathway.[] Aminopentanamide C01028 N6-Hydroxy-L-lysine C01142 (3S)-3,6-Diaminohexanoate C01144 (S)-3-Hydroxybutanoyl-CoA C01149 4-Trimethylammoniobutanal C01181 4-Trimethylammoniobutanoate[] ., July 31, via Qnebeo AlU. 25. witb iron to order. U. S. steam transpor... ... forces here.[] Pristanic acid is a 2-methyl fatty acid that can be degraded by β-oxidation.[] […] associations of SNPs for AASDHPPT No data available for Polymorphic Variants from UniProtKB/Swiss-Prot for AASDHPPT Gene - elite association - COSMIC cancer census association via[]

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  • Pyruvate Carboxylase Deficiency

    This observation, coupled with the intermittent hypercholesterolemia and the increased tissue acetyl-CoA concentrations, suggests that pyruvate carboxylase is important in[] If the patient is in a catabolic state, proteins are degraded, resulting in the elevation of many amino acids and a nonspecific amino acid profile.[] Increased plasma L/P ratio Decreased plasma 3-OHB/AcAc ratio Ammonemia: 268 μmol/L Plasma amino acids: Alanine: 298 μmol/L Proline: 114 μmol/L Lysine: 136 μmol/L Glutamine[] The oxaloacetate thus produced is needed for the first step in cellular respiration via the Krebs' cycle, for gluconeogenesis and also for the effective removal of ammonia[] The reaction proceeds via the removal of a proton from pyruvate, by an as yet unidentified active site residue, to generate an enolate intermediate.[] […] pyruvate metabolism and gluconeogenesis Or: 2015/16 ICD-10-CM E74.8 Other specified disorders of carbohydrate metabolism Or: 2015/16 ICD-10-CM E77.1 Defects in glycoprotein degradation[]

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  • 2-Aminoadipic 2-Oxoadipic Aciduria

    In the matrix of mitochondria, 2-oxoadipate is decarboxylated to glutaryl-CoA by the 2-oxoadipate dehydrogenase complex and then converted to acetyl-CoA.; 2-Oxoadipic aciduria[] […] accumulation of deuterium-labeled 2-oxoadipate only in noncomplemented cells, demonstrating that DHTKD1 codes for the enzyme mediating the last unresolved step in the L-lysine-degradation[] Degradation of lysine through either pathway leads to the formation of α-aminoadipic semialdehyde, which is catabolized to acetyl-coenzyme A (CoA) and enters the tricarboxylic[] DHTKD1 is located one enzymatic step above glutaryl-CoA dehydrogenase in the lysine degradative pathway.[] Significant amounts of 3OHGA were found to be excreted via the intestinal tract.[] […] the enzyme mediating the last unresolved step in the L-lysine-degradation pathway.[]

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  • Beta-Ureidopropionase Deficiency

    acetyl-CoA acyltransferase Deficiency of acetyl-CoA deacylase Deficiency of acetyl-CoA hydrolase Deficiency of acetylesterase Deficiency of acetylserotonin methyltransferase[] Clinical Chemistry, Emma Children's Hospital, The Netherlands. [email protected] Abstract beta-Ureidopropionase deficiency is an inborn error of the pyrimidine degradation[] Abstract The pyrimidine bases uracil and thymine are degraded via the consecutive action of three enzymes to beta-alanine and beta-aminoisobutyric acid, respectively.[] Thymidylate Synthesis (starting with CDP) 1) CDP to dCDP via ribonucleotide reductase 2) dCDP to dCTP via kinase 3) dCTP to dUTP via deaminase 4) dUTP to dUMP via dUTPase[] Pyrimidine bases and degradation products in body fluids As part of a selective screening program for inborn errors, the pyrimidine bases, uracil and thymine, and their degradation[] .- ) Other disorders of amino-acid metabolism Approximate Synonyms Deficiency of acetyl-coa acyltransferase Deficiency, beta ketothiolase Disorder of straight chain amino[]

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  • Biotin Deficiency

    For example, acetyl-CoA carboxylase catalyzes the rate-limiting step in fatty acid elongation.[] Experts believe this because smoking accelerates the degradation of many nutrients, including B-vitamins such as biotin, particularly in women.[] Am J Physiol 263:G81–G86 PubMed Google Scholar Said HM, Thuy LP, Sweetman L et al (1993) Transport of the biotin dietary derivative biocytin (N-biotinyl-L-lysine) in rat small[] If you have access to journal via a society or associations, read the instructions below Members of _ can log in with their society credentials below Username (required) Password[] In the lysosomes, various proteolytic enzymes degrade the holocarboxylase to form biocytin, which, in turn, is hydrolyzed by the enzyme biotinidase to form biotin and lysine[] Fetal hepatic acetyl-CoA carboxylase, pyruvate carboxylase, propionyl-CoA carboxylase and beta-methylcrotonyl-CoA carboxylase abundances determined by Western blotting decreased[]

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  • Treponema Denticola

    KEGG Modules Pathway module Energy metabolism Carbon fixation M00579 Phosphate acetyltransferase-acetate kinase pathway, acetyl-CoA acetate [PATH: tde01200 tde01100 tde01120[] Upon heating at 100 degrees C, the polypeptide was dissociated into three components (apparent molecular masses, 80, 48, and 45 kDa) that did not individually bind or degrade[] Purified GGT was inactivated by TLCK (Nalpha-p-tosyl-L-lysine chloromethyl ketone) and proteinase K treatment.[] In this study, we demonstrate that T. denticola induces innate immune responses via the utilization of Toll-like receptor 2 (TLR2) but not TLR4.[] The fixed cells were collected by centrifugation, washed with the same buffer, and deposited on a poly( l -lysine)- treated slide.[] T. denticola reduced inflammatory cytokines, including interleukin-1beta (IL-1beta), IL-6, and tumor necrosis factor alpha, in peripheral blood mononuclear cells through degradation[]

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  • 2-Methylbutyryl-CoA Dehydrogenase Deficiency

    External links Short descriptions of genetic disorders from v Inborn error of amino acid metabolism ( E70–E72 , 270 ) K acetyl-CoA Lysine /straight chain Glutaric[] 2-methylbutyryl-CoA dehydrogenase deficiency or short/branched chain acyl-CoA dehydrogenase deficiency (SBCADD) is caused by a defect in the degradation pathway of the amino[] […] dipeptidase • Deficiency of L-serine dehydratase • Deficiency of lyase • Deficiency of lysine racemase • Deficiency of lysine-tRNA ligase • Deficiency of lysolecithin acylmutase[] It is most often ascertained via newborn screening and is usually clinically asymptomatic, although some patients have been reported to have delayed development and neurologic[] acetyl-CoA acyltransferase Deficiency of acetyl-CoA deacylase Deficiency of acetyl-CoA hydrolase Deficiency of acetylesterase Deficiency of acetylserotonin methyltransferase[] Spinal Muscular Atrophy (SMA): SMA status is assessed via copy number analysis using the data generated via the NGS panel described above.[]

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  • Carnitine

    In addition to increasing the rate of fatty acid transport into mitochondria, levocarnitine reduces the intramitochondrial ratio of acetyl-CoA to free CoA, thus stimulating[] Before HIIT, muscle phosphocreatine (PCr) degradation (P 2max , Watt max , and work output were similarly increased in CON and CARN, by 9, 15, and 23% (P 2017 John Wiley &[] Acetyl L-Carnitine is an acetylated form of L-Carnitine, a nitrogen-containing compound derived from L-Lysine.[] Since L-carnitine (LC) has an indispensable role in lipid metabolism via its involvement in the β-oxidation of long-chain fatty acids and it has antioxidant properties as[] Found naturally in the body, acetyl-l-carnitine supports the availability of acetyl-CoA, an important energy generating metabolite.[] Based on current animal studies, a role of l-carnitine in the prevention of age-associated muscle protein degradation and regulation of mitochondrial homeostasis is suggested[]

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