hypophosphatemic rickets (저인산 혈성 구루병, 저인산염 혈성 구루병
| alpha-ketoadipate dehydrogenase | <enzyme> X-ketoadipic acid + nad + CoA yields glutaryl-CoA + co(2) + NADH + h Registry number: EC 1.2.1.- (26 Jun 1999) |
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| alpha-ketoarginine decarboxylase | <enzyme> Alpha-ketoarginine gives gamma-guanidinobutyraldehyde Registry number: EC 4.1.1.- Synonym: alpha keto-arginine decarboxylase (26 Jun 1999) |
| alpha-ketobutyrate dehydrogenase | <enzyme> Rat liver enzyme oxidatively decarboxylates alpha-ketobutyrate to carbon dioxide and propionyl CoA; requires nad, fad and thiamine pyrophosphate Registry number: EC 1.2.1.- (26 Jun 1999) |
| alpha-ketodecarboxylase | Formerly, the enzyme system converting pyruvate (a 2-oxoacid) to acetyl-CoA and CO2, with reduction of NAD+ to NADH and the participation of lipoamide and thiamin pyrophosphate; now known to involve at least three enzymes in succession: pyruvate dehydrogenase, dihydrolipoamide acetyltransferase, and dihydrolipoamide dehydrogenase. Compare: pyruvate dehydrogenase (lipoamide). (05 Mar 2000) |
| alpha-ketoglutaramic acid | H2N-COCH2CH2COCOOH;a metabolite of glutamine formed by the action of glutamine aminotransferase; elevated in certain cases of hepatocoma. Synonym: 2-oxoglutaric acid. (05 Mar 2000) |
| alpha-ketoglutarate | A salt or ester of alpha-ketoglutaric acid. Alpha-ketoglutarate dehydrogenase, an enzyme that catalyses the oxidative decarboxylation of 2-ketoglutaric acid to succinyldihydrolipoate; the succinyl group is later transferred to CoA and the reduced lipoate is oxidised by NAD+; a complex that is a part of the tricarboxylic acid cycle. Synonym: 2-oxoglutarate dehydrogenase, alpha-ketoglutarate dehydrogenase complex. (05 Mar 2000) |
| alpha-ketoglutarate dehydrogenase complex | alpha-ketoglutarate dehydrogenase |
| alpha-ketoglutarate permease | <chemical> From E coli; 432-amino acid protein; amino acid sequence given in first source Synonym: kGTP gene product, wita protein, alpha-ketoglutarate transporter (26 Jun 1999) |
| alpha-ketoisocaproate oxygenase | <enzyme> From rat liver cytosol; oxidatively decarboxylates and hydroxylates alpha-ketoisocaporate to form beta-hydroxyisovalerate; requires fe(2+) and thiol; activated by ascorbate Registry number: EC 1.13.11.- Synonym: 2-ketoisocaproate oxygenase, alpha-ketoisocaproate dioxygenase (26 Jun 1999) |
| alpha-ketosuccinamic acid | NH2-CO-CH2-CO-COOH;the transamination product of asparagine; acted upon by ω-amidase. (05 Mar 2000) |
| alpha-lactyl-thiamin pyrophosphate | An intermediate formed in the oxidative decarboxylation of pyruvate. Compare: pyruvate dehydrogenase (lipoamide). Synonym: alpha-lactyl-thiamin pyrophosphate. (05 Mar 2000) |
| alpha-l-iduronidase | <enzyme> An enzyme that hydrolyzes terminal desulfated alpha-l-iduronic acid residues of dermatan sulfate and of heparan sulfate; a deficiency of this enzyme is associated with Hurler syndrome and Scheie syndrome. (05 Mar 2000) |
| alpha-linolenic acid | <chemical> (z,z,z)-9,12,15-octadecatrienoic acid. A fatty acid that is found in plants and involved in the formation of prostaglandins. Chemical name: 9,12,15-Octadecatrienoic acid, (Z,Z,Z)- (12 Dec 1998) |
| alpha-macroglobulins | Glycoproteins with a molecular weight of approximately 620,000 to 680,000. Precipitation by electrophoresis is in the alpha region. They include alpha 1-macroglobulins and alpha 2-macroglobulins. These proteins exhibit trypsin-, chymotrypsin-, thrombin-, and plasmin-binding activity and function as hormonal transporters. (12 Dec 1998) |
| alpha-mannosidase | <enzyme> Catalyses hydrolysis of terminal, non-reducing alpha-d-mannose residues in alpha-d-mannosides Registry number: EC 3.2.1.24 Synonym: alpha-d-mannoside mannohydrolase, alpha-d-mannosidase, neutral alpha-mannosidase, alpha mannosidase a, alpha mannosidase b, lysosomal alpha-mannosidase, laman (26 Jun 1999) |