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24 Possible Causes for 2 ketoisocaproate, Arginine

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  • Hyperammonemia

    Carboxylation of [2-(14)C] pyruvate diminished following incubation of HLLKO hepatocytes with the leucine metabolite 2-ketoisocaproate (KIC).[ncbi.nlm.nih.gov] KEYWORDS: Ammonia; Arginine; Citrulline; Hyperammonemia; Liver; Ornithine; Urea cycle[ncbi.nlm.nih.gov] Herein, we report a case of a late-onset OTC deficient patient who was successfully treated with arginine, benzoate and hemodialysis.[ncbi.nlm.nih.gov]

  • Transient Hyperammonemia of the Newborn

    No specific disease is known at this level. (2) The second step is the conversion of 2-ketoisocaproic acid into isovaleryl coenzyme A by the branched-chain alpha-ketoacid[mrineonatalbrain.com] The most common treatments are dialysis (both peritoneal and hemodialysis), sodium benzoate, and arginine.[en.wikipedia.org] The role of arginine supplementation in UCDs is two fold.[themedicalbiochemistrypage.org]

  • Cardiomyopathy - Hypotonia - Lactic Acidosis Syndrome

    No specific disease is known at this level. (2) The second step is the conversion of 2-ketoisocaproic acid into isovaleryl coenzyme A by the branched-chain alpha-ketoacid[mrineonatalbrain.com] The insertion of an arginine in the hydrophobic sequence of ATPase 6 probably interferes with the hydrogen ion channel formed by subunits 6 and 9 of the ATPase, thus causing[catalog.coriell.org] […] wks (AR) Urea cycle defect: hyperammonemia, minimal metabolic acidosis (unlike sepsis), respiratory alkalosis, no LFT dysfunction, low citrulline, high orotic acid, low arginine[quizlet.com]

  • Acetyl-CoA Carboxylase Deficiency

    No specific disease is known at this level. (2) The second step is the conversion of 2-ketoisocaproic acid into isovaleryl coenzyme A by the branched-chain alpha-ketoacid[mrineonatalbrain.com] : 10 mg; Arginine: 10 mg; Ornithin: 10 mg; Ornithine: 10 mg; Glutathion: 20 mg.[google.ch] (R) Aspartat-Aminotransferase Asparagin (N) Asparaginsäure (D) Adenosintriphosphat Adenosintriphosphatase Arginin-Vasopressin Da DHF DNA DNase Dopa Dopamin ECM EDRF EDTA[docplayer.net]

  • Pyroglutamate Hydrolase Deficiency

    2-Ketoisocaproate Lactic acidosis; ketosis 2-Ketoisovalerate Lactic acidosis; ketosis 2-Methyl-acetoacetate (30) 2-Methylglutaconate 3-Hydroxy-2-ethylyglutarate 3-Hydroxy[thefreelibrary.com] .- Synonym: ADP-ribosyl histone splitting enzyme, ADP-ribosyl protein lyase (26 Jun 1999) ADPribosylarginine hydrolase Acts on adpribose arginine to yield adpribose and arginine[kmle.co.kr] Copper (see Copper , [[Copper]]) Gentamicin (see Gentamicin , [[Gentamicin]]) Ifosfamide (Ifex) (see Ifosfamide , [[Ifosfamide]]): produces both distal and proximal RTA L-Arginine[mdnxs.com]

  • Combined Oxidative Phosphorylation Defect Type 14

    No specific disease is known at this level. (2) The second step is the conversion of 2-ketoisocaproic acid into isovaleryl coenzyme A by the branched-chain alpha-ketoacid[mrineonatalbrain.com] While NOS oxidizes arginine to citrulline and nitric oxide (NO), arginase hydrolyzes arginine into ornithine and urea.[frontiersin.org] Arginine and citrulline for the treatment of MELAS syndrome. J Inborn Errors Metab Screen . 2017 Jan. 5: [Medline] .[emedicine.medscape.com]

  • Dysmyelination with Jaundice

    No specific disease is known at this level. (2) The second step is the conversion of 2-ketoisocaproic acid into isovaleryl coenzyme A by the branched-chain alpha-ketoacid[mrineonatalbrain.com] Acute treatment options include sodium benzoate, sodium phenylacetate, and arginine. Certain persons may benefit from liver transplantation.[aafp.org] NSIAD) is an SIADH-like clinical and laboratory picture seen in male infants who present with neurologic symptoms secondary to hyponatremia but who have undetectable plasma arginine[emedicine.medscape.com]

  • Early-Onset X-Linked Isolated Optic Atrophy

    No specific disease is known at this level. (2) The second step is the conversion of 2-ketoisocaproic acid into isovaleryl coenzyme A by the branched-chain alpha-ketoacid[mrineonatalbrain.com] (complex) arginine N-linked (GlcNAc...) (complex) asparagine N-linked (GlcNAc...) (high mannose) arginine N-linked (GlcNAc...)[uniprot.org] ketoisocaproic acid by the branched-chain amino acid transaminase.[mrineonatalbrain.com]

  • Streptozocin

    The insulin secretory response to other nutrient secretagogues, such as 2-ketoisocaproic acid and leucine, or non-nutrient secretagogues, such as the sulfonylurea drug tolbutamide[doi.org] The baseline insulin and glucagon concentrations were enhanced in a separate series of experiments by the addition of arginine (5 mM) to the perfusate, and while the insulin[ncbi.nlm.nih.gov] […] mean glucose disposal rate (Kg) remained within the normal range, but dropped from 2.0 /- 0.2% /- SE) to 1.2 /- 0.1%/min (P less than 0.01), the acute insulin response to arginine[ncbi.nlm.nih.gov]

  • Isovaleric Acidemia

    The same is true for [U- 13 C]-Lys feeding in the case of α-ketovalerate but not α-ketoisocaproate.[plantcell.org] The amino acid substitution of a glycine to arginine resulted in a markedly reduced steady-state level of the IVD protein, which explains the nearly complete lack of IVD enzyme[ncbi.nlm.nih.gov] Ingredients: Maltodextrin, Vegetable oils, Corn starch, Sugar, Amino acids (L-Tyrosine, L-Proline, L-Isoleucine, L-Arginine, L- Threonine, L-Serine, Glycine, L-Histidine,[pristineorganics.com]

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