Literature DB >> 5051750

Defective decarboxylase in branched chain ketoacid oxidase multienzyme complex in classic type of maple syrup urine disease.

H W Rüdiger, U Langenbeck, M Schulze-Schencking, H W Goedde.   

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Year:  1972        PMID: 5051750     DOI: 10.1007/bf00290167

Source DB:  PubMed          Journal:  Humangenetik        ISSN: 0018-7348


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  11 in total

1.  [STUDIES ON THE SUBSTRATE SPECIFICITY OF LIPOIC ACID DEHYDROGENASE. SYNTHESES IN THE LIPOIC ACID SERIES. III].

Authors:  H W GOEDDE; P GRAFEN; U SCHMIDT
Journal:  Biochem Z       Date:  1963-09-19

2.  Maple syrup urine disease.

Authors:  J DANCIS; M LEVITZ; S MILLER; R G WESTALL
Journal:  Br Med J       Date:  1959-01-10

3.  [Oxidation of alpha-keto acids and some aldehydes by pyruvate decarboxylase from yeast].

Authors:  H HOLZER; H W GOEDDE
Journal:  Biochem Z       Date:  1957

4.  A new syndrome: progressive familial infantile cerebral dysfunction associated with an unusual urinary substance.

Authors:  J H MENKES; P L HURST; J M CRAIG
Journal:  Pediatrics       Date:  1954-11       Impact factor: 7.124

5.  Multiple exchange transfusions as treatment during the acute period in maple syrup urine disease.

Authors:  L Schuchmann; I Witt; P Schulz; H Schumacher; H Rüdiger
Journal:  Helv Paediatr Acta       Date:  1972-09

6.  Thin-layer chromatographic separation of 14C-labelled branched-chain alpha-keto acids and hydroxamic acids derived from the corresponding enzymatically formed acyl-CoA compounds.

Authors:  H W Rüdiger; M Nikisch; H W Goedde
Journal:  J Chromatogr       Date:  1971-10

7.  A new variant of maple syrup urine disease (branched chain ketoaciduria). Clinical and biochemical evaluation.

Authors:  J D Schulman; T J Lustberg; J L Kennedy; M Museles; J E Seegmiller
Journal:  Am J Med       Date:  1970-07       Impact factor: 4.965

8.  [Biochemical studies on inherited disorders of amino acid metabolism in pediatrics].

Authors:  I Antener
Journal:  Z Klin Chem Klin Biochem       Date:  1970-09-08

9.  Evaluation of a heterozygote test for maple syrup urine disease in leucocytes and cultured fibroblasts.

Authors:  U Langenbeck; H W Rüdiger; M Schulze-Schencking; W Keller; D Brackertz; H W Goedde
Journal:  Humangenetik       Date:  1971

10.  Intermittent branched-chain ketonuria. Variant of maple-syrup-urine disease.

Authors:  J Dancis; J Hutzler; T Rokkones
Journal:  N Engl J Med       Date:  1967-01-12       Impact factor: 91.245

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  1 in total

1.  Maple syrup urine disease: branched-chain amino acid concentrations and metabolism in cultured human lymphoblasts.

Authors:  S D Skaper; D P Molden; J E Seegmiller
Journal:  Biochem Genet       Date:  1976-08       Impact factor: 1.890

  1 in total

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