Assays of pyruvate dehydrogenase complex and pyruvate carboxylase activity

Methods in Molecular Biology

Volumn 837, Issue , 2012, Pages 93-119

  Kerr, Douglas ^a   Grahame, George ^a   Nakouzi, Ghunwa ^a  

^a UNIVERSITY HOSPITALS OF CLEVELAND   (United States)

Author keywords

citrate synthase; dihydrolipoamide dehydrogenase; pyruvate; pyruvate carboxylase; Pyruvate dehydrogenase complex; radiometric assays; spectrophotometric assays

Indexed keywords

CITRATE SYNTHASE; DIHYDROLIPOAMIDE DEHYDROGENASE; PYRUVATE CARBOXYLASE; PYRUVATE DEHYDROGENASE COMPLEX;

ADULT; ANIMAL; ARTICLE; ENZYME ASSAY; HUMAN; INFANT; METABOLISM; METHODOLOGY; PRESCHOOL CHILD;

ADULT; ANIMALS; CHILD, PRESCHOOL; CITRATE (SI)-SYNTHASE; DIHYDROLIPOAMIDE DEHYDROGENASE; ENZYME ASSAYS; HUMANS; INFANT; PYRUVATE CARBOXYLASE; PYRUVATE DEHYDROGENASE COMPLEX;

EID: 84856342302     PISSN: 10643745     EISSN: None     Source Type: Book Series    
DOI: 10.1007/978-1-61779-504-6_7     Document Type: Article

References (14)

1. Sheu, K. F. R., Hu, C. W. C., and Utter, M. F. (1981) Pyruvate dehydrogenase complex activity in normal and deficient fibroblasts. J. Clin. Invest. 67, 1463-1471. (Pubitemid 11099362)
2. Robinson, B. H., Taylor, J., and Sherwood, W. G. (1980) The genetic heterogeneity of lactic acidosis: occurrence of recognizable inborn errors of metabolism in pediatric population with lactic acidosis. Pediatr. Res. 14, 956-962. (Pubitemid 10035866)
3. Kerr, D. S., Ho, L., Berlin, C. M., Lanoue, K. F., et al. (1987) Systemic deficiency of the first component of the pyruvate dehydrogenase complex. Pediatr. Res. Pediatr. Res. 22, 312-318. (Pubitemid 17162795)
4. Lib, M., Rodriguez-Mari, A., Marusich, M. F., et al (2003) Immunocapture and microplatebased activity measurement of mammalian pyruvate dehydrogenase complex. Anal. Biochem. 314, 121-127. (Pubitemid 36299027)
5. Sterk, J. P., Stanley, W. C., Hoppel, C. L., et al. (2003) A radiochemical pyruvate dehydrogenase assay: activity in heart. Anal. Biochem. 313, 179-182. (Pubitemid 36268836)
6. Lib, M. Y., Brown, R. M., Brown, G. K., et al. (2002) Detection of pyruvate dehydrogenase E1 alpha-subunit deficiencies in females by immunohistochemical demonstration of mosaicism in cultured fibroblasts. J. Histochem. Cytochem. 50, 877-884. (Pubitemid 34774598)
7. Kerr, D. S. and Zinn, A. B. (2009) Disorders of Pyruvate Metabolism and the Tricarboxylic Acid Cycle. In: Sarafoglu, K. (ed) Essentials of Pediatric Endocrinology and Metabolism. McGraw-Hill.
8. Chuang, D. T., Niu, W. L., and Cox, R. P. (1981) Activities of branched-chain 2-oxo acid dehydrogenase and its components in skin fibroblasts from normal and classical-maple-syrupurine-disease subjects. Biochem. J. 200, 59-67. (Pubitemid 12174948)
9. Patel, M. S., Hong, Y. S., and Kerr, D. S. (2000) Genetic defects in E3 component of alpha-keto acid dehydrogenase complexes. Methods Enzymol. 324, 453-464.
10. Atkin, B. M., Utter, M. F., and Weinberg, M. B. (1979) Pyruvate carboxylase and phosphoenolpyruvate carboxykinase activity in leukocytes and fibroblasts from a patient with pyruvate carboxylase deficiency. Pediatr. Res. 13, 38-43. (Pubitemid 9051213)
11. Scrutton, M. C., Olmsted, M. R., and Utter, M. F. (1969) Pyruvate carboxyalse. Methods Enzymol. Volume 13, 235-249.
12. Srere, P. A. (1969) Citrate synthase: [EC 4.1.3.7. Citrate oxaloacetate-lyase (CoA-acetylating)]. Methods Enzymol. Volume 13, 3-11.
13. Lowry, O. H., Rosebrough, N. J., Farr, A. L., et al (1951) Protein measurement with the folin phenol reagent. J. Biol. Chem. 193, 265-275.
14. Maj, M. C., MacKay, N., Levandovskiy, V., et al. (2005) Pyruvate dehydrogenase phosphatase deficiency: identification of the first mutation in two brothers and restoration of activity by protein complementation. J. Clin. Endocrinol. Metab 90, 4101-4107. (Pubitemid 41058179)