Results 191 to 200 of about 23,834 (239)
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Pyruvate carboxylase deficiency
Journal of Inherited Metabolic Disease, 1984AbstractThe causes of congenital lactic acidaemia are outlined. Isolated pyruvate carboxylase deficiency is reviewed in detail with a report of a recent case and a discussion of the biochemical consequences. Other causes of defective pyruvate carboxylation are described, particularly the combined carboxylase defects.
Janet-H. Stirk +4 more
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Substrate activation of pyruvate carboxylase by pyruvate
Biochemical and Biophysical Research Communications, 1969Abstract A kinetic analysis of pyruvate carboxylase isolated from sheep liver and using pyruvate as the variable substrate revealed non-Michaelis Menten kinetic. Double reciprocal plots were biphasic and R S values of 222 were obtained. Hill plots prepared from the initial velocity data showed that at low pyruvate concentrations, the slope of the ...
J. Nielsen, D.B. Keech, Helen Taylor
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Disorders of pyruvate carboxylase and the pyruvate dehydrogenase complex [PDF]
Journal of Inherited Metabolic Disease, 1996 SummaryThe most common defect associated with deficiency of the pyruvate dehydrogenase (PDH) complex occurs in the E1 component, specifically due to mutations in the X‐linked E1α gene. Clinical sequelae of these mutations, which range from severe neonatal lactic acidosis to carbohydrate‐sensitive ataxia, can be different in males and females depending ...
Mingfu Ling +3 more
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Pyruvate Carboxylase Deficiency [PDF]
, 1995 Pyruvate carboxylase (PC) deficiency is a rare disorder with autosomal recessive inheritance. Generally, two clinical and biochemical phenotypes of isolated PC deficiency can be distinguished. The so-called French phenotype presents in the neonatal period with severe lactic acidemia and is clinically characterized by failure to thrive, anorexia ...
Marjo S. van der Knaap, Jacob Valk
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Pyruvate carboxylase in genetic obesity
American Journal of Physiology-Endocrinology and Metabolism, 1992Immunoblotting and protein microsequencing were used to identify several adipocyte proteins expressed in an obesity-related fashion in the Zucker rat. One of these was a 116-kDa particulate protein (p116). The p116 levels in adipocytes from 5- to 7-wk-old obese Zucker rats were two- to fivefold higher on a per milligram of protein basis than levels in ...
K. M. McCall +7 more
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Decarboxylation of oxalacetate by pyruvate carboxylase
Biochemistry, 1986The decarboxylation of oxalacetate by pyruvate carboxylase in the absence of ADP and Pi is stimulated 400-fold by the presence of oxamate, which is an inhibitory analogue of pyruvate. The observation of substrate inhibition when either oxamate or oxalacetate is varied at a fixed concentration of the other indicates that both molecules bind at the same ...
Paul V. Attwood, W. W. Cleland
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Pyruvate carboxylase: Affinity labelling of the pyruvate binding site
Biochemical and Biophysical Research Communications, 1975Abstract The active-site-directed reagent, bromopyruvate has been used to covalently label the pyruvate binding site of pyruvate carboxylase (E.C.6.4.1.1.) isolated from sheep liver. Oxalo-acetate proved to be the most effective reaction component in protecting the enzyme against inactivation; pyruvate was less effective although its efficiency was ...
John C. Wallace +2 more
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Regulation of pyruvate carboxylase activity by pyruvate in mice liver
International Journal of Biochemistry, 1979Abstract 1. 1. In order to study in vivo the possible regulatory role of pyruvate concentrations on pyruvate carboxylase activity. fed or 24 hr-fasted mice received various overloads of unlabeled pyruvate. 2. 2. The effect of these overloads on the incorporation of [2- 14 C]pyruvate into blood glucose was studied. 3. 3.
P. Favarger, Simonne Rous, Sylvette Bas
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On the Intracellular Location of Pyruvate Carboxylase
1971Summary The concept of the exclusive location of pyruvate carboxylase in mitochondria is based upon the analysis of a subcellular fraction representing a mixture of fragments, and on the exclusion of a cytoplasmic location of this enzyme. Preferential extraction of pyruvate carboxylase – as compared to glutamate dehydrogenase – with isotonic ...
H Brod +3 more
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Pyruvate Carboxylase and Pyruvate Dehydrogenase Deficiency [PDF]
, 2014 Pyruvate carboxylase and pyruvate dehydrogenase deficiency are the most common disorders in pyruvate metabolism and almost always affect the central nervous system. The severity and the clinical phenotypes vary, with a range from overwhelming neonatal lactic acidosis and early death to milder presentations.
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