(Q43708605)

English

Natural osmolyte trimethylamine N-oxide corrects assembly defects of mutant branched-chain alpha-ketoacid decarboxylase in maple syrup urine disease

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scholarly article

1 reference

Europe PubMed Central

26 November 2017

Natural osmolyte trimethylamine N-oxide corrects assembly defects of mutant branched-chain alpha-ketoacid decarboxylase in maple syrup urine disease (English)

1 reference

Europe PubMed Central

26 November 2017

author name string

Song JL

1

1 reference

Europe PubMed Central

26 November 2017

Chuang DT

2

1 reference

Europe PubMed Central

26 November 2017

language of work or name

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publication date

15 August 2001

1 reference

Europe PubMed Central

26 November 2017

Journal of Biological Chemistry

1 reference

Europe PubMed Central

26 November 2017

276

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Europe PubMed Central

26 November 2017

40241-40246

1 reference

Europe PubMed Central

26 November 2017

43

1 reference

Europe PubMed Central

26 November 2017

Thiamine-responsive maple-syrup-urine disease

1 reference

https://api.crossref.org/works/10.1074%2FJBC.M107242200

21 January 2018

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

1 reference

https://api.crossref.org/works/10.1074%2FJBC.M107242200

21 January 2018

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

1 reference

https://api.crossref.org/works/10.1074%2FJBC.M107242200

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

https://api.crossref.org/works/10.1074%2FJBC.M107242200

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.M107242200

21 January 2018

A naturally occurring protective system in urea-rich cells: mechanism of osmolyte protection of proteins against urea denaturation

1 reference

https://api.crossref.org/works/10.1074%2FJBC.M107242200

21 January 2018

Trimethylamine N-oxide-induced cooperative folding of an intrinsically unfolded transcription-activating fragment of human glucocorticoid receptor.

1 reference

https://api.crossref.org/works/10.1074%2FJBC.M107242200

21 January 2018

Osmolyte-driven contraction of a random coil protein

1 reference

https://api.crossref.org/works/10.1074%2FJBC.M107242200

21 January 2018

Chemical chaperones correct the mutant phenotype of the delta F508 cystic fibrosis transmembrane conductance regulator protein

1 reference

https://api.crossref.org/works/10.1074%2FJBC.M107242200

21 January 2018

Chemical chaperones mediate increased secretion of mutant alpha 1-antitrypsin (alpha 1-AT) Z: A potential pharmacological strategy for prevention of liver injury and emphysema in alpha 1-AT deficiency

1 reference

https://api.crossref.org/works/10.1074%2FJBC.M107242200

21 January 2018

Influence of molecular and chemical chaperones on protein folding

1 reference

https://api.crossref.org/works/10.1074%2FJBC.M107242200

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.M107242200

21 January 2018

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

1 reference

https://api.crossref.org/works/10.1074%2FJBC.M107242200

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.M107242200

21 January 2018

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

1 reference

https://api.crossref.org/works/10.1074%2FJBC.M107242200

21 January 2018

Time-dependent effects of trimethylamine-N-oxide/urea on lactate dehydrogenase activity: an unexplored dimension of the adaptation paradigm

1 reference

https://api.crossref.org/works/10.1074%2FJBC.M107242200

21 January 2018

Manipulating the amyloid-beta aggregation pathway with chemical chaperones.

1 reference

https://api.crossref.org/works/10.1074%2FJBC.M107242200

21 January 2018

Molecular Chaperones

1 reference

https://api.crossref.org/works/10.1074%2FJBC.M107242200

21 January 2018

Chaperonin-assisted folding of glutamine synthetase under nonpermissive conditions: off-pathway aggregation propensity does not determine the co-chaperonin requirement

1 reference

https://api.crossref.org/works/10.1074%2FJBC.M107242200

21 January 2018

Identifiers

10.1074/JBC.M107242200

1 reference

Europe PubMed Central

26 November 2017

release_b6yy7iznxndzpfeswh4ydo3ztu

1 reference

https://api.fatcat.wiki/v0/release/b6yy7iznxndzpfeswh4ydo3ztu

24 November 2022

based on heuristic

mapped directly with Wikidata item

1 reference

Europe PubMed Central

26 November 2017

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