Abstract
We have biochemically characterized several parameters of propionyl CoA carboxylase (PCC) activity in fibroblast extracts from PCC-deficient patients belonging to the two minor genetic complementation groups, pcc B and pcc BC. Comparison of PCCs from these groups with those of the two major complementation groups, pcc A and pcc C, has demonstrated that PCCs from both the pcc B and pcc BC groups closely resemble each other as well as PCC from the pcc C group. These results further support the hypothesis that the pcc B and pcc BC lines are interallelic with respect to pcc C and consequently that the structural mutations in the PCCs from these groups involve the same subunit.
Original language | English (US) |
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Pages (from-to) | 703-707 |
Number of pages | 5 |
Journal | Biochemical Genetics |
Volume | 17 |
Issue number | 7-8 |
DOIs | |
State | Published - Aug 1979 |
Keywords
- genetic complementation
- propionic acidemia
- propionyl CoA carboxylase deficiency
ASJC Scopus subject areas
- Genetics
- Ecology, Evolution, Behavior and Systematics
- Molecular Biology
- Biochemistry