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Multiple carboxylase deficiency.
Int J Biochem 1988; 20(4):363-70IJ

Abstract

1. The multiple carboxylase deficiencies are inborn errors in the metabolism of biotin in which there is defective activity of propionyl CoA carboxylase, 3-methylcrotonyl CoA carboxylase and pyruvate carboxylase. 2. Two distinct disorders have been described. 3. In one the fundamental defect is in the enzyme holocarboxylase synthetase which catalyzes the molecular activation of the apocarboxylase proteins. 4. In the other the fundamental defect is in biotinidase which catalyzes the reutilization of biotin and may be involved in its digestion and intestinal absorption.

Authors+Show Affiliations

University of California San Diego, La Jolla 92093.

Pub Type(s)

Journal Article
Review

Language

eng

PubMed ID

3284772

Citation

Nyhan, W L.. "Multiple Carboxylase Deficiency." The International Journal of Biochemistry, vol. 20, no. 4, 1988, pp. 363-70.
Nyhan WL. Multiple carboxylase deficiency. Int J Biochem. 1988;20(4):363-70.
Nyhan, W. L. (1988). Multiple carboxylase deficiency. The International Journal of Biochemistry, 20(4), pp. 363-70.
Nyhan WL. Multiple Carboxylase Deficiency. Int J Biochem. 1988;20(4):363-70. PubMed PMID: 3284772.
* Article titles in AMA citation format should be in sentence-case
TY - JOUR T1 - Multiple carboxylase deficiency. A1 - Nyhan,W L, PY - 1988/1/1/pubmed PY - 1988/1/1/medline PY - 1988/1/1/entrez SP - 363 EP - 70 JF - The International journal of biochemistry JO - Int. J. Biochem. VL - 20 IS - 4 N2 - 1. The multiple carboxylase deficiencies are inborn errors in the metabolism of biotin in which there is defective activity of propionyl CoA carboxylase, 3-methylcrotonyl CoA carboxylase and pyruvate carboxylase. 2. Two distinct disorders have been described. 3. In one the fundamental defect is in the enzyme holocarboxylase synthetase which catalyzes the molecular activation of the apocarboxylase proteins. 4. In the other the fundamental defect is in biotinidase which catalyzes the reutilization of biotin and may be involved in its digestion and intestinal absorption. SN - 0020-711X UR - https://www.unboundmedicine.com/medline/citation/3284772/Multiple_carboxylase_deficiency_ DB - PRIME DP - Unbound Medicine ER -