Changes in the Carboxyl Terminus of the β Subunit of Human Propionyl-CoA Carboxylase Affect the Oligomer Assembly and Catalysis: Expression and Characterization of Seven Patient-Derived Mutant Forms of PCC in Escherichia coli

Propionyl-CoA carboxylase (PCC) catalyzes the biotin-dependent carboxylation of propionyl-CoA to d-methylmalonyl-CoA in the mitochondrial matrix. Human PCC is a dodecamer composed of pairs of nonidentical α and β subunits encoded by PCCA and PCCB genes, respectively. Deficiency of PCC results in pro...

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Bibliographic Details
Published in:	Molecular genetics and metabolism Vol. 71; no. 4; pp. 623 - 632
Main Authors:	Chloupková, Maja, Ravn, Kirstine, Schwartz, Marianne, Kraus, Jan P.
Format:	Journal Article
Language:	English
Published:	United States Elsevier Inc 01-12-2000
Subjects:	Amino Acid Sequence Base Sequence Blotting, Western Carboxy-Lyases - chemistry Carboxy-Lyases - genetics Carboxy-Lyases - metabolism Catalysis Chromatography, Gel Enzyme Stability Escherichia coli - genetics Exons - genetics Fibroblasts Genotype Humans Metabolism, Inborn Errors - enzymology Metabolism, Inborn Errors - genetics Methylmalonyl-CoA Decarboxylase mitochondria Mitochondria - enzymology Molecular Sequence Data Mutation - genetics mutations Oligodeoxyribonucleotides Phenotype Propionates - blood Propionates - metabolism propionic acidemia propionyl-CoA carboxylase protein degradation Protein Structure, Quaternary Protein Subunits Recombinant Proteins - chemistry Recombinant Proteins - metabolism propionic acidemia mutations propionyl-CoA carboxylase mitochondria protein degradation
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