Crystal structure of human branched-chain alpha-ketoacid dehydrogenase and the molecular basis of multienzyme complex deficiency in maple syrup urine disease (Q22253419)

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English	Crystal structure of human branched-chain alpha-ketoacid dehydrogenase and the molecular basis of multienzyme complex deficiency in maple syrup urine disease	scientific article

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scholarly article

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Crystal structure of human branched-chain alpha-ketoacid dehydrogenase and the molecular basis of multienzyme complex deficiency in maple syrup urine disease (English)

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crystal structure

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maple syrup urine disease

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inferred from title

author name string

A AEvarsson

1

author given names

A

author last names

AEvarsson

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28 March 2023

J L Chuang

2

author given names

J L

author last names

Chuang

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28 March 2023

R M Wynn

3

author given names

R M

author last names

Wynn

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28 March 2023

S Turley

4

author given names

S

author last names

Turley

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28 March 2023

D T Chuang

5

author given names

D T

author last names

Chuang

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28 March 2023

W G Hol

6

author given names

W G

author last names

Hol

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28 March 2023

language of work or name

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publication date

15 March 2000

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8

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277-91

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3

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Regulation of mammalian pyruvate and branched-chain alpha-keto acid dehydrogenase complexes by phosphorylation-dephosphorylation

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Atomic structure of the cubic core of the pyruvate dehydrogenase multienzyme complex

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Principles of quasi-equivalence and Euclidean geometry govern the assembly of cubic and dodecahedral cores of pyruvate dehydrogenase complexes

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Purification and partial characterization of branched-chain alpha-ketoacid dehydrogenase kinase from rat liver and rat heart

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Purification and properties of branched-chain alpha-keto acid dehydrogenase phosphatase from bovine kidney

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GroEL/GroES-dependent reconstitution of alpha2 beta2 tetramers of humanmitochondrial branched chain alpha-ketoacid decarboxylase. Obligatory interaction of chaperonins with an alpha beta dimeric intermediate

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Metabolism of the white blood cells in maple-syrup-urine disease

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Sixty years of thiamin diphosphate biochemistry.

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Roles of amino acid residues surrounding phosphorylation site 1 of branched-chain alpha-ketoacid dehydrogenase (BCKDH) in catalysis and phosphorylation site recognition by BCKDH kinase.

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

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A common structural motif in thiamin pyrophosphate-binding enzymes

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Impaired assembly of E1 decarboxylase of the branched-chain alpha-ketoacid dehydrogenase complex in type IA maple syrup urine disease

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A T-to-A substitution in the E1 alpha subunit gene of the branched-chain alpha-ketoacid dehydrogenase complex in two cell lines derived from Menonite maple syrup urine disease patients

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10.1016/S0969-2126(00)00105-2

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