(Q40793105)

English

Biochemical basis of type IB (E1beta ) mutations in maple syrup urine disease. A prevalent allele in patients from the Druze kindred in Israel

scientific article published on 11 July 2001

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scholarly article

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Europe PubMed Central

PubMed publication ID

23 September 2017

Biochemical basis of type IB (E1beta ) mutations in maple syrup urine disease. A prevalent allele in patients from the Druze kindred in Israel (English)

1 reference

Europe PubMed Central

PubMed publication ID

23 September 2017

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author name string

Wynn RM

1

1 reference

Europe PubMed Central

PubMed publication ID

23 September 2017

Chuang JL

2

1 reference

Europe PubMed Central

PubMed publication ID

23 September 2017

Sansaricq C

3

1 reference

Europe PubMed Central

PubMed publication ID

23 September 2017

Mandel H

4

1 reference

Europe PubMed Central

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23 September 2017

Chuang DT

5

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Europe PubMed Central

PubMed publication ID

23 September 2017

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publication date

11 July 2001

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Europe PubMed Central

PubMed publication ID

23 September 2017

number of pages

7

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Journal of Biological Chemistry

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Europe PubMed Central

PubMed publication ID

23 September 2017

276

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Europe PubMed Central

PubMed publication ID

23 September 2017

39

1 reference

Europe PubMed Central

PubMed publication ID

23 September 2017

36550-36556

1 reference

Europe PubMed Central

PubMed publication ID

23 September 2017

Thiamine-responsive maple-syrup-urine disease

1 reference

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21 January 2018

Purification and characterization of branched chain alpha-keto acid dehydrogenase complex of bovine kidney

1 reference

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21 January 2018

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

1 reference

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21 January 2018

Maple syrup urine disease in Mennonites. Evidence that the Y393N mutation in E1 alpha impedes assembly of the E1 component of branched-chain alpha-keto acid dehydrogenase complex

1 reference

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21 January 2018

Release of infectious Epstein-Barr virus by transformed marmoset leukocytes

1 reference

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21 January 2018

Molecular cloning of the mature E1b-beta subunit of human branched-chain alpha-keto acid dehydrogenase complex

1 reference

https://api.crossref.org/works/10.1074%2FJBC.M105862200

21 January 2018

Molecular and biochemical basis of intermediate maple syrup urine disease. Occurrence of homozygous G245R and F364C mutations at the E1 alpha locus of Hispanic-Mexican patients

1 reference

https://api.crossref.org/works/10.1074%2FJBC.M105862200

21 January 2018

Role of the pleiotropic effects of plasminogen deficiency in infection experiments with plasminogen-deficient mice

1 reference

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21 January 2018

Cleavage of Structural Proteins during the Assembly of the Head of Bacteriophage T4

1 reference

https://api.crossref.org/works/10.1074%2FJBC.M105862200

21 January 2018

Roles of active site and novel K+ ion-binding site residues in human mitochondrial branched-chain alpha-ketoacid decarboxylase/dehydrogenase

1 reference

https://api.crossref.org/works/10.1074%2FJBC.M105862200

21 January 2018

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

1 reference

https://api.crossref.org/works/10.1074%2FJBC.M105862200

21 January 2018

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

1 reference

https://api.crossref.org/works/10.1074%2FJBC.M105862200

21 January 2018

A Defect in Branched-Chain Amino Acid Metabolism in a Patient with Congenital Lactic Acidosis due to Dihydrolipoyl Dehydrogenase Deficiency

1 reference

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21 January 2018

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

1 reference

https://api.crossref.org/works/10.1074%2FJBC.M105862200

21 January 2018

Diagnosis and mutational analysis of maple syrup urine disease using cell cultures

1 reference

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21 January 2018

Localization and suppression of a kinetic defect in cystic fibrosis transmembrane conductance regulator folding

1 reference

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21 January 2018

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

1 reference

https://api.crossref.org/works/10.1074%2FJBC.M105862200

21 January 2018

GroEL/GroES promote dissociation/reassociation cycles of a heterodimeric intermediate during alpha(2)beta(2) protein assembly. Iterative annealing at the quaternary structure level

1 reference

https://api.crossref.org/works/10.1074%2FJBC.M105862200

21 January 2018

Interactions of GroEL/GroES with a heterodimeric intermediate during alpha 2beta 2 assembly of mitochondrial branched-chain alpha-ketoacid dehydrogenase. cis capping of the native-like 86-kDa intermediate by GroES

1 reference

https://api.crossref.org/works/10.1074%2FJBC.M105862200

21 January 2018

Identifiers

10.1074/JBC.M105862200

1 reference

Europe PubMed Central

PubMed publication ID

23 September 2017

PubMed publication ID

1 reference

Europe PubMed Central

PubMed publication ID

23 September 2017

ResearchGate publication ID

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