Nothing Special   »   [go: up one dir, main page]

Wendel et al., 1975 - Google Patents

Maple syrup urine disease: analysis of branched chain ketoacid decarboxylation in cultured fibroblasts

Wendel et al., 1975

View PDF
Document ID
4501636378879267913
Author
Wendel U
Wentrup H
Rüdiger H
Publication year
Publication venue
Pediatric Research

External Links

Snippet

Extract: Kinetic data are presented for the decarboxylation of branched chain α-ketoacids (BCKA) by intact human fibroblasts. Cultured cells of normal individuals and nine patients with different clinical pictures of maple syrup urine disease (MSUD) are studied with both α …
Continue reading at www.nature.com (PDF) (other versions)

Classifications

    • AHUMAN NECESSITIES
    • A61MEDICAL OR VETERINARY SCIENCE; HYGIENE
    • A61KPREPARATIONS FOR MEDICAL, DENTAL, OR TOILET PURPOSES
    • A61K31/00Medicinal preparations containing organic active ingredients
    • A61K31/185Acids; Anhydrides, halides or salts thereof, e.g. sulfur acids, imidic, hydrazonic, hydroximic acids
    • A61K31/19Carboxylic acids, e.g. valproic acid
    • AHUMAN NECESSITIES
    • A61MEDICAL OR VETERINARY SCIENCE; HYGIENE
    • A61KPREPARATIONS FOR MEDICAL, DENTAL, OR TOILET PURPOSES
    • A61K31/00Medicinal preparations containing organic active ingredients
    • A61K31/33Heterocyclic compounds
    • A61K31/395Heterocyclic compounds having nitrogen as a ring hetero atom, e.g. guanethidine, rifamycins
    • A61K31/40Heterocyclic compounds having nitrogen as a ring hetero atom, e.g. guanethidine, rifamycins having five-membered rings with one nitrogen as the only ring hetero atom, e.g. sulpiride, succinimide, tolmetin, buflomedil
    • CCHEMISTRY; METALLURGY
    • C12BIOCHEMISTRY; BEER; SPIRITS; WINE; VINEGAR; MICROBIOLOGY; ENZYMOLOGY; MUTATION OR GENETIC ENGINEERING
    • C12QMEASURING OR TESTING PROCESSES INVOLVING ENZYMES OR MICRO-ORGANISMS; COMPOSITIONS OR TEST PAPERS THEREFOR; PROCESSES OF PREPARING SUCH COMPOSITIONS; CONDITION RESPONSIVE CONTROL IN MICROBIOLOGICAL OR ENZYMOLOGICAL PROCESSES
    • C12Q1/00Measuring or testing processes involving enzymes, nucleic acids or micro-organisms; Compositions therefor; Processes of preparing such compositions
    • C12Q1/26Measuring or testing processes involving enzymes, nucleic acids or micro-organisms; Compositions therefor; Processes of preparing such compositions involving oxidoreductase
    • CCHEMISTRY; METALLURGY
    • C12BIOCHEMISTRY; BEER; SPIRITS; WINE; VINEGAR; MICROBIOLOGY; ENZYMOLOGY; MUTATION OR GENETIC ENGINEERING
    • C12QMEASURING OR TESTING PROCESSES INVOLVING ENZYMES OR MICRO-ORGANISMS; COMPOSITIONS OR TEST PAPERS THEREFOR; PROCESSES OF PREPARING SUCH COMPOSITIONS; CONDITION RESPONSIVE CONTROL IN MICROBIOLOGICAL OR ENZYMOLOGICAL PROCESSES
    • C12Q1/00Measuring or testing processes involving enzymes, nucleic acids or micro-organisms; Compositions therefor; Processes of preparing such compositions
    • C12Q1/60Measuring or testing processes involving enzymes, nucleic acids or micro-organisms; Compositions therefor; Processes of preparing such compositions involving cholesterol
    • CCHEMISTRY; METALLURGY
    • C12BIOCHEMISTRY; BEER; SPIRITS; WINE; VINEGAR; MICROBIOLOGY; ENZYMOLOGY; MUTATION OR GENETIC ENGINEERING
    • C12QMEASURING OR TESTING PROCESSES INVOLVING ENZYMES OR MICRO-ORGANISMS; COMPOSITIONS OR TEST PAPERS THEREFOR; PROCESSES OF PREPARING SUCH COMPOSITIONS; CONDITION RESPONSIVE CONTROL IN MICROBIOLOGICAL OR ENZYMOLOGICAL PROCESSES
    • C12Q1/00Measuring or testing processes involving enzymes, nucleic acids or micro-organisms; Compositions therefor; Processes of preparing such compositions
    • C12Q1/68Measuring or testing processes involving enzymes, nucleic acids or micro-organisms; Compositions therefor; Processes of preparing such compositions involving nucleic acids
    • C12Q1/6876Hybridisation probes
    • C12Q1/6883Hybridisation probes for diseases caused by alterations of genetic material
    • AHUMAN NECESSITIES
    • A61MEDICAL OR VETERINARY SCIENCE; HYGIENE
    • A61KPREPARATIONS FOR MEDICAL, DENTAL, OR TOILET PURPOSES
    • A61K38/00Medicinal preparations containing peptides

Similar Documents

Publication Publication Date Title
Lieber et al. Ethanol oxidation by hepatic microsomes: adaptive increase after ethanol feeding
Stanley et al. Medium-chain acyl-CoA dehydrogenase deficiency in children with non-ketotic hypoglycemia and low carnitine levels
Vaziri et al. Effect of chronic experimental renal insufficiency on urate metabolism.
Yudkoff et al. Brain glutamate metabolism: neuronal-astroglial relationships
Frohlich et al. Effect of fasting on free and esterified carnitine levels in human serum and urine: correlation with serum levels of free fatty acids and β-hydroxybutyrate
Elder et al. The primary enzyme defect in hereditary coproporphyria
Brivet et al. Defects in activation and transport of fatty acids
Robinson et al. Pyruvate dehydrogenase phosphatase deficiency: a cause of congenital chronic lactic acidosis in infancy
Kerr et al. A deficiency of both subunits of pyruvate dehydrogenase which is not expressed in fibroblasts
Kohlschütter et al. A familial progressive neurodegenerative disease with 2-oxoglutaric aciduria
KR20020093906A (en) Compositions of Biochemical Compounds Involved in Bioenergy Metabolism of Cells and Method of Use
Smelt et al. Very long chain acyl‐coenzyme A dehydrogenase deficiency with adult onset
LA DU The enzymatic deficiency in tyrosinemia
Valle et al. Type II hyperprolinemia. Delta1-pyrroline-5-carboxylic acid dehydrogenase deficiency in cultured skin fibroblasts and circulating lymphocytes.
Wendel et al. Maple syrup urine disease: analysis of branched chain ketoacid decarboxylation in cultured fibroblasts
Christensen et al. Studies on glutaryl-CoA dehydrogenase in leucocytes, fibroblasts and amniotic fluid cells. The normal enzyme and the mutant form in patients with glutaric aciduria
Thoene et al. Neonatal citrullinemia: Treatment with keto-analogues of essential amino acids
Sabbagh et al. 3-Mercaptopropionic acid, a potent inhibitor of fatty acid oxidation in rat heart mitochondria.
Brown et al. The biochemistry of Reye's syndrome
Lieber Alcohol and malnutrition in the pathogenesis of liver disease
Chuang et al. Activities of branched-chain 2-oxo acid dehydrogenase and its components in skin fibroblasts from normal and classical-maple-syrup-urine-disease subjects
Carroll et al. Carnitine “deficiency” lack of response to carnitine therapy
Robinson Lacticacidemia: biochemical, clinical, and genetic considerations
Christensen et al. Glutaric aciduria type II: evidence for a defect related to the electron transfer flavoprotein or its dehydrogenase
Bonne et al. El pyruvate dehydrogenase deficiency in a child with motor neuropathy