Article
Characterization of four variant forms of human propionyl-CoA carboxylase expressed in Escherichia coli.
The Journal of biological chemistry - 29 Jul 2005
Jiang Hua, Rao K Sudhindra, Yee Vivien C, Kraus Jan P
Abstract excerpt
Propionyl-CoA carboxylase (PCC) is a biotin-dependent mitochondrial enzyme that catalyzes the conversion of propionyl-CoA to D-methylmalonyl-CoA. PCC consists of two heterologous subunits, alpha PCC and beta PCC, which are encoded by the nuclear PCCA and PCCB genes, respectively. Deficiency of PCC results in a metabolic disorder, propionic acidemia, which is sufficiently severe to cause neonatal death. We have...
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