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146 Possible Causes for 5 aminopentanamide, L lysine, acetyl CoA, catabolic, process, to, via

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  • Biotin Deficiency

    […] developed in an 11-month-old Japanese infant who had been diagnosed as a neonate with cow milk and soy bean allergy and fed with an amino acid formula and hypoallergenic rice processed[] We conclude that anticonvulsant treatment led to increased biotin catabolism and probably to reduced biotin status.[] 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[] For example, acetyl-CoA carboxylase catalyzes the rate-limiting step in fatty acid elongation.[] 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[] Also, try to avoid processed foods, since processing strips this and other vitamins out of the food you eat.[] Therefore, biotin is essential for amino acid catabolism, gluconeogenesis, and fatty acid metabolism.[]

    Missing: 5 aminopentanamide to
  • Cytosolic Acetoacetyl-CoA Thiolase Deficiency

    This process is experimental and the keywords may be updated as the learning algorithm improves. This is a preview of subscription content, log in to check access.[] Associations: Defects in ACAT1 are a cause of 3-ketothiolase deficiency (3KTD) [ MIM:203750 ], also known as alpha-methylacetoaceticaciduria. 3KTD is an inborn error of isoleucine catabolism[] Deficiency of lyase Deficiency of lysine-tRNA ligase Deficiency of lysolecithin acylmutase Deficiency of lysophospholipase Deficiency of lysozyme Deficiency of malate dehydrogenase[] CoA Thiolase, Acetoacetyl; C- Acetyltransferase, Acetyl- CoA; Acetyltransferase, Acetyl- CoA; Acetyl CoA C Acetyltransferase; Acetyl CoA Acetyltransferase Substance CAS Registry[] 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[] Sequence processing i : The displayed sequence is further processed into a mature form. This entry describes 2 isoforms i produced by alternative splicing .[] Prenatal Diagnosis / Carrier Detection Research Abstract Beta-ketothiolase deficiency (3KTD) is an autosomal recessive metabolic disease of organic acids involving isoleucine catabolic[]

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  • Acetyl-CoA Carboxylase Deficiency

    Two colleagues were particularly helpful ledge of genetic defects, biochemical abnormalities, and provided us with essential and unpublished f- and cellular processes underlying[] Provider 3- Methylcrotonyl - CoA Carboxylase Deficiency (3MMC or 3MCC) Description: 3- Methylcrotonyl - CoA Carboxylase Deficiency is an autosomal recessive disorder of leucine catabolism[] […] dipeptidase Deficiency of L-serine dehydratase Deficiency of lyase Deficiency of lysine racemase Deficiency of lysine-tRNA ligase Deficiency of lysolecithin acylmutase Deficiency[] An important gene associated with Acetyl-Coa Carboxylase Deficiency is ACACA (Acetyl-CoA Carboxylase Alpha).[] For example, AMPK activation increases (a) glucose uptake (via recruiting to the cell membrane or activating glucose transporters GLUT4 and GLUT1), (b) glycolysis (via phosphorylation[] : vitamin metabolic process; acetyl-CoA metabolic process; positive regulation of cellular metabolic process; lipid homeostasis; triacylglycerol biosynthetic process; carnitine[] Maintenance on a low-protein diet, to minimize the burden of leucine that must be catabolized, prevents the development of metabolic acidosis.[]

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  • Isovaleric Acidemia

    We have reported elsewhere nine point mutations in the IVD gene in fibroblasts of patients with IVA, which lead to abnormalities in IVD protein processing and activity.[] In approximately one-fifth of catabolic episodes, no trigger was apparent. Triggering factors of catabolic episodes in symptomatic isovaleric acidemia (IVA) .[] L-Methionine, L- Cystine, L Valine, L-Phenylalanine, L- Alanine, L-Aspartic acid, L-Tyrosine, L-Glutamic Acid, Glutamine, Lysine) Tri calcium phosphate, Magnesium oxide, Ferric[] The Ki of isovaleryl-CoA dehydrogenase by (methylenecyclopropyl)acetyl-CoA was approximately 2 microM.[] This suggests that detoxification via glucuronide conjugation plays an important role when the glycine conjugation system is saturated.[] These data suggest that binding of the variant protein to mitochondrial membrane receptors per se is hindered, resulting in the inefficient mitochondrial processing.[] Its pathophysiological process is mainly via the competitive inhibition of N-acetylglutamate synthetase.[]

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  • Glutaric Aciduria

    Proton magnetic resonance spectroscopy in the parietal white matter revealed a markedly increased choline/creatine ratio, suggesting a demyelinating process.[] Glutaric aciduria type I (GA-I; synonym, glutaric acidemia type I) is a rare inherited metabolic disease caused by deficiency of glutaryl-CoA dehydrogenase located in the catabolic[] The primary defect is the deficiency of Glutaryl-CoA dehydrogenase (EC number enzyme that is involved in the catabolic pathways of the amino acids l-lysine, l-hydroxylysine[] […] will then produce two molecules of acetyl-CoA and enter TCA Cycle 19 Lysine Degradation 20 Lysine Degradation 21 Tryptophan Degradation Eight mechanistic steps Eventually[] In conclusion, our results indicate a low but specific efflux transport for GA, 3-OH-GA, and MMA across pBCEC, an in vitro model of the BBB, via OAT1 and OAT3 but not across[] This might indicate increased resynthesis of dendritic processes secondary to preceding metabolic crises.[] Glutaric aciduria type I is caused by inherited deficiency of glutaryl-CoA dehydrogenase which is involved in the catabolic pathways of L-lysine, L-hydroxylysine and L-tryptophan[]

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  • Pipecolic Acidemia

    Quickly grasp the fundamentals you need to know through a more precise, streamlined format, reorganized by systems and disease processes, as well as "Teaching Boxes" that[] Packaging 1 g in glass bottle 100, 250 mg in glass insert Biochem/physiol Actions L-Pipecolic acid is a lysine metabolite; defect in its catabolism is involved in hyperpipecolic[] C01186 (3S,5S)-3,5-Diaminohexanoate C01211 Procollagen 5-hydroxy-L-lysine C01259 (3S)-3-Hydroxy-N6,N6,N6-trimethyl-L-lysine C01672 Cadaverine C02188 Protein lysine C02727[] 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[] ., July 31, via Qnebeo AlU. 25. witb iron to order. U. S. steam transpor... ... forces here.[] […] intake or from mammalian or intestinal bacterial enzyme metabolism Recent studies suggest that plasma pipecolic acid, particularly the D-isomer, originates mainly from the catabolism[] Background: The essential amino acid lysine can be degraded via two pathways, the saccharopine pathway and the pipecolic acid (PA) pathway.[]

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  • Lactobacillus Bulgaricus

    KEYWORDS: Lactobacillus bulgaricus extract; freeze–thawing process; poly (vinyl alcohol); poly (vinyl alcohol)κ-Carrageenan[] These results suggest that lactose catabolism is not under catabolic repression by glucose in L. bulgaricus .[] ATP-binding subunit ClpX ( clpX ), chromosomal replication initiation protein ( dnaA ), CTP synthetase ( pyrG ), chaperonin GroEL ( groEL ), UDP-N-acetylmuramoylalanyl-D-glutamate-L-lysine[] Acetyl-CoA may subsequently be used for the production of saturated fatty acids.[] . & Gerhardt P. 1981 Dialysis continuous process for ammonium-lactate fermentation: simulated and experimental dialysated-feed immobilized cell systems.[] Ardo Y (2006) Flavour formation by amino acid catabolism. Biotechnol Adv 24: 238–242. View Article Google Scholar 36.[] During the freeze-drying process, the wall materials alone provided little protection against cell mortality.[]

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  • Lactococcus Lactis

    Under these conditions, almost 10% of the 900 samples processed yielded viable phages.[] Changes in aroma profile resembled some of the biochemical changes occurring during cheese ripening and reflected amino acid catabolism, metabolism of fatty acids and conversion[] For the other genes, which play minor roles in the thermal tolerance, llmg_2477 and llmg_2541 encodes lysine specific permease and cation transporting ATPase, respectively[] Under aerobic conditions, the PDH complex is the only pathway leading to the formation of acetyl-CoA from pyruvate, and as acetyl-CoA is an essential metabolite in many processes[] The pH of culture was controlled at 6.0 for 6–24 h via the addition of NaOH (10 M).[] Pagina 241 - The work on peptidases and amino acid catabolism conducted in our laboratory was supported in part by the College of Agricultural and Life Sciences at the University[] […] selected for use in fermentations based on their metabolic stability, their resistance to bacteriophage, and their ability to produce unique compounds often from amino acid catabolism[]

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  • Propionic Acidemia

    This is one step in the process of converting certain amino acids and fats into sugar for energy.[] Four acetyl CoA precursors increased during clinical episodes: glutaric acid, a catabolic intermediate of lysine; 3-hydroxyisovaleric acid and 3-methylglutaconic acid, catabolic[] Homocitrullinuria, presence of epsilon-N-acetyl-l-lysine in urine, and absence of saccharopine indicate deficiency of the saccharopine pathway of lysine degradation.[] Continuous venovenous hemodialysis was performed successfully for both infants via extracorporeal membrane oxygenation pump, and both rapidly improved.[] […] propionic acid, propionylcarnitine and methylcitrate on the KvLQT1/KCNE1 channel complex cause long QT syndrome in patients with propionic acidemia by inhibition of K flow via[] Propionic acidemia is an autosomal recessive metabolic disorder where the body is unable to process certain parts of proteins and lipids.[] Reversal of catabolism by stopping protein intake and administering non-protein calories in the form of intravenous fluids is the mainstay of treatment of a crisis.[]

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  • Hyperammonemia

    Hyperammonemia in neuroendocrine tumor would represent a potentially reversible but ongoing process associated with an indolent malignancy.[] […] multifactorial including limited capacity of liver detoxification function due to congenital porto-caval shunt, liver ischemia, excessive protein intake and increased protein catabolic[] Increased urinary excretion of lysine is seen in LPI, whereas in HHH syndrome, plasma ornithine level is elevated along with increased urinary homocitrulline.[] KIC loading also increased levels of several leucine-related acyl-CoAs and reduced acetyl-CoA levels.[] The case represents the first report of a successful closure of splenorenal shunt via percutaneous embolization of the splenic vein with an amplatzer plug using a common femoral[] Abstract Ammonia is constantly produced as a metabolic waste from amino acid catabolism in mammals.[] SUMMARY: Signaling via myostatin and eIF2α phosphorylation causes decreases in protein synthesis and mTORC1 activity with a parallel mitochondrial dysfunction and increased[]

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