Multiple Carboxylase Deficiency

Known as: Carboxylase Deficiencies, Multiple, Carboxylase Deficiency, Multiple, Deficiencies, Combined Carboxylase 
A deficiency in the activities of biotin-dependent enzymes (propionyl-CoA carboxylase, methylcrotonyl-CoA carboxylase, and PYRUVATE CARBOXYLASE) due… (More)
National Institutes of Health

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BiotinBiotinidaseHolocarboxylase Synthetase DeficiencyIn Blood
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Papers overview

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Highly Cited
2003
Highly Cited
2003
Reduced histone biotinylation in multiple carboxylase deficiency patients: a nuclear role for holocarboxylase synthetase.
The attachment of biotin to apocarboxylases is catalyzed by holocarboxylase synthetase (HCS). An inherited deficiency of HCS… (More)
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1999
1999
Mechanism of biotin responsiveness in biotin-responsive multiple carboxylase deficiency.
Holocarboxylase synthetase (HCS) catalyses the biotinylation of the four biotin-dependent carboxylases found in humans. A… (More)
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Highly Cited
1998
Highly Cited
1998
Moderately High Temperatures Inhibit Ribulose-1,5-Bisphosphate Carboxylase/Oxygenase (Rubisco) Activase-Mediated Activation of Rubisco
We tested the hypothesis that light activation of ribulose-1,5-bisphosphate carboxylase/oxygenase (Rubisco) is inhibited by… (More)
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Review
1997
Review
1997
Multiple carboxylase deficiency: inherited and acquired disorders of biotin metabolism.
Acquired biotin deficiency and the two known congenital disorders of biotin metabolism, biotinidase and holocarboxylase… (More)
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Review
1988
Review
1988
Multiple carboxylase deficiency.
1. The multiple carboxylase deficiencies are inborn errors in the metabolism of biotin in which there is defective activity of… (More)
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Highly Cited
1985
Highly Cited
1985
Effect of temperature on the CO2/O2 specificity of ribulose-1,5-bisphosphate carboxylase/oxygenase and the rate of respiration in the light
Responses of the rate of net CO2 assimilation (A) to the intercellular partial pressure of CO2 (p i ) were measured on intact… (More)
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1981
1981
Two forms of biotin-responsive multiple carboxylase deficiency
Biotin-responsive multiple carboxylase deficiencies are classified into early and late forms. The early form showed higher… (More)
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1981
1981
Deficient acetyl CoA carboxylase activity in multiple carboxylase deficiency.
Multiple carboxylase deficiency has previously been characterized by deficient activity of three biotin-dependent enzymes… (More)
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Highly Cited
1981
Highly Cited
1981
Species variation in the specificity of ribulose biphosphate carboxylase/oxygenase
The balance between photosynthesis and photorespiration in many species, including most crop plants, is determined by the kinetic… (More)
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1981
1981
Mutant holocarboxylase synthetase: evidence for the enzyme defect in early infantile biotin-responsive multiple carboxylase deficiency.
Biotin-responsive multiple carboxylase deficiency is an inherited disorder of organic acid metabolism in man in which there are… (More)
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