Immunological studies of propionyl CoA carboxylase in livers and fibroblasts of patients with propionic acidemia

Chaterine McKeon, Robert Z. Eanes, R. Ray Fall, Diane M. Tasset, Barry Wolf*

*Corresponding author for this work

Research output: Contribution to journalArticle

3 Citations (Scopus)

Abstract

Antiserum prepared against homogeneous pig heart propionyl CoA carboxylase cross-reacted with human propionyl CoA carboxylase, and was used to demonstrate the presence of immunological cross-reacting material in extracts from the livers of three patients and from fibroblasts of four patients with propionic acidemia representing three major propionyl CoA carboxylasedeficient genetic complementation groups, pcc A, pcc C and bio. Since the quantity of cross-reacting material in the propionyl CoA carboxylase-deficient livers and enzyme-deficient fibroblast cell lines was comparable to that in normal tissues while showing less than five percent of the normal enzyme activity, these patients must synthesize normal or near-normal quantities of an enzymatically inactive propionyl CoA carboxylase protein. In addition, no appreciable change in the amount of cross-reacting material was found in the biotin-responsive bio fibroblasts after incubation with supplemental biotin despite a sixteenfold increase in enzyme activity suggesting that the defect in the bio mutant involves the activation rather than the synthesis of a pre-existing normal apoenzyme.

Original languageEnglish (US)
Pages (from-to)217-223
Number of pages7
JournalClinica Chimica Acta
Volume101
Issue number2-3
DOIs
StatePublished - Feb 28 1980

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Methylmalonyl-CoA Decarboxylase
Propionic Acidemia
Fibroblasts
Liver
Enzyme activity
Biotin
Enzymes
Apoenzymes
Liver Extracts
Immune Sera
Swine
Chemical activation
Cells
Tissue
Cell Line
Defects
Proteins

ASJC Scopus subject areas

  • Biochemistry
  • Clinical Biochemistry
  • Biochemistry, medical

Cite this

McKeon, Chaterine ; Eanes, Robert Z. ; Fall, R. Ray ; Tasset, Diane M. ; Wolf, Barry. / Immunological studies of propionyl CoA carboxylase in livers and fibroblasts of patients with propionic acidemia. In: Clinica Chimica Acta. 1980 ; Vol. 101, No. 2-3. pp. 217-223.
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abstract = "Antiserum prepared against homogeneous pig heart propionyl CoA carboxylase cross-reacted with human propionyl CoA carboxylase, and was used to demonstrate the presence of immunological cross-reacting material in extracts from the livers of three patients and from fibroblasts of four patients with propionic acidemia representing three major propionyl CoA carboxylasedeficient genetic complementation groups, pcc A, pcc C and bio. Since the quantity of cross-reacting material in the propionyl CoA carboxylase-deficient livers and enzyme-deficient fibroblast cell lines was comparable to that in normal tissues while showing less than five percent of the normal enzyme activity, these patients must synthesize normal or near-normal quantities of an enzymatically inactive propionyl CoA carboxylase protein. In addition, no appreciable change in the amount of cross-reacting material was found in the biotin-responsive bio fibroblasts after incubation with supplemental biotin despite a sixteenfold increase in enzyme activity suggesting that the defect in the bio mutant involves the activation rather than the synthesis of a pre-existing normal apoenzyme.",
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Immunological studies of propionyl CoA carboxylase in livers and fibroblasts of patients with propionic acidemia. / McKeon, Chaterine; Eanes, Robert Z.; Fall, R. Ray; Tasset, Diane M.; Wolf, Barry.

In: Clinica Chimica Acta, Vol. 101, No. 2-3, 28.02.1980, p. 217-223.

Research output: Contribution to journalArticle

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