| Literature DB >> 25730 |
G Morrow, B Revsin, R Clark, J Lebowitz, D T Whelan.
Abstract
Cultured fibroblasts from a patient with methylmalonic acidemia, clinically responsive to vitamin B-12, were studied in vitro. Kinetic analysis revealed abnormal binding of the coenzyme, 5'-deoxyadenosylcobalamin, for its methylmalonyl-CoA carbonylmutase apoenzyme, i.e., KM of 3.8 X 10(-5) M versus control KM of 1.5 X 10(-8) M. These data are interpreted as indicating a structural defect of the apoenzyme at the coenzyme binding site, and represent another variant of this genetic disorder.Entities:
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Year: 1978 PMID: 25730 DOI: 10.1016/0009-8981(78)90102-x
Source DB: PubMed Journal: Clin Chim Acta ISSN: 0009-8981 Impact factor: 3.786