Regulation of the branched-chain alpha-ketoacid dehydrogenase and elucidation of a molecular basis for maple syrup urine disease (Q24329186)

cellular amino acid catabolic process

GOA release 2020-03-11

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maple syrup urine disease

GOA release 2020-03-11

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R A Harris

1

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B Zhang

2

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G W Goodwin

3

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M J Kuntz

4

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Y Shimomura

5

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P Rougraff

6

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P Dexter

7

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Y Zhao

8

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R Gibson

9

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D W Crabb

Advances in Enzyme Regulation

10

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language of work or name

English

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publication date

1990

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published in

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volume

30

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page(s)

245-63

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cites work

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Enzymatic determination of the branched-chain alpha-keto acids

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Stabilization of mammalian liver branched-chain alpha-ketoacid dehydrogenase by thiamin pyrophosphate

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Regulation of branched-chain alpha-ketoacid dehydrogenase kinase

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Clofibric acid, phenylpyruvate, and dichloroacetate inhibition of branched-chain alpha-ketoacid dehydrogenase kinase in vitro and in perfused rat heart

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Physiological covalent regulation of rat liver branched-chain alpha-ketoacid dehydrogenase.

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Phosphorylation sites and inactivation of branched-chain alpha-ketoacid dehydrogenase isolated from rat heart, bovine kidney, and rabbit liver, kidney, heart, brain, and skeletal muscle.

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Activation of branched-chain alpha-ketoacid dehydrogenase in isolated hepatocytes by branched-chain alpha-ketoacids

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Monovalent cations and inorganic phosphate alter branched-chain alpha-ketoacid dehydrogenase-kinase activity and inhibitor sensitivity

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Effects of L-leucine and alpha-ketoisocaproic acid upon insulin secretion and metabolism of isolated pancreatic islets

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Inactivation of purified ox kidney branched-chain 2-oxoacid dehydrogenase complex by phosphorylation

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Regulation of branched-chain alpha-ketoacid dehydrogenase complex by covalent modification

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Thiamine-responsive maple-syrup-urine disease

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Maple syrup urine disease in calves: a clinical, pathological and biochemical study

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Evidence for both a regulatory mutation and a structural mutation in a family with maple syrup urine disease

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Identifiers

DOI

1 reference

Consolidated OpenCitations Corpus – April 2017

4245084

4245084

1 reference

Consolidated OpenCitations Corpus – April 2017

Consolidated OpenCitations Corpus – April 2017

1 reference