Characterization of four variant forms of human propionyl-CoA carboxylase expressed in Escherichia coli.

@article{Jiang2005CharacterizationOF,
  title={Characterization of four variant forms of human propionyl-CoA carboxylase expressed in Escherichia coli.},
  author={Hua Jiang and Kandibanda S Rao and Vivien C. Yee and Jan P Kraus},
  journal={The Journal of biological chemistry},
  year={2005},
  volume={280 30},
  pages={27719-27}
}
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 purified three PCCs containing pathogenic mutations in the beta subunit (R165W, E168K… CONTINUE READING
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