(Q28286648)

English

Altered phosphorylation state of branched-chain 2-oxo acid dehydrogenase in a branched-chain acyltransferase deficient human fibroblast cell line

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Altered phosphorylation state of branched-chain 2-oxo acid dehydrogenase in a branched-chain acyltransferase deficient human fibroblast cell line (English)

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phosphorylation

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inferred from title

author name string

R S Eisenstein

1

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G Hoganson

2

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R H Miller

3

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A E Harper

4

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language of work or name

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publication date

1991

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Journal of Inherited Metabolic Disease

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14

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1

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37-44

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Activities of branched-chain 2-oxo acid dehydrogenase and its components in skin fibroblasts from normal and classical-maple-syrup-urine-disease subjects

1 reference

https://api.crossref.org/works/10.1007%2FBF01804386

21 January 2018

Resolution and reconstitution of bovine kidney branched-chain 2-oxo acid dehydrogenase complex

1 reference

https://api.crossref.org/works/10.1007%2FBF01804386

21 January 2018

The 2-oxo acid dehydrogenase complexes: recent advances

1 reference

https://api.crossref.org/works/10.1007%2FBF01804386

21 January 2018

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

1 reference

https://pubmed.ncbi.nlm.nih.gov/1861457

12 December 2020

based on heuristic

inferred from PubMed ID database lookup

Detection of heterozygotes in maple-syrup-urine disease: measurements of branched-chain alpha-ketoacid dehydrogenase and its components in cell cultures.

1 reference

https://pubmed.ncbi.nlm.nih.gov/1861457

12 December 2020

based on heuristic

inferred from PubMed ID database lookup

1 reference

https://pubmed.ncbi.nlm.nih.gov/1861457

12 December 2020

based on heuristic

inferred from PubMed ID database lookup

Perinatal diagnosis of the inborn errors of metabolism

1 reference

https://pubmed.ncbi.nlm.nih.gov/1861457

12 December 2020

based on heuristic

inferred from PubMed ID database lookup

Structure-function relationships in dihydrolipoamide acyltransferases

1 reference

https://pubmed.ncbi.nlm.nih.gov/1861457

12 December 2020

based on heuristic

inferred from PubMed ID database lookup

Regulation of branched-chain alpha-ketoacid dehydrogenase kinase

1 reference

https://pubmed.ncbi.nlm.nih.gov/1861457

12 December 2020

based on heuristic

inferred from PubMed ID database lookup

Inhibition of branched chain alpha-ketoacid dehydrogenase kinase activity by alpha-chloroisocaproate

1 reference

https://pubmed.ncbi.nlm.nih.gov/1861457

12 December 2020

based on heuristic

inferred from PubMed ID database lookup

Branched-chain ketoacid dehydrogenase activity and growth of normal and mutant human fibroblasts: the effect of branched-chain amino acid concentration in culture medium

1 reference

https://pubmed.ncbi.nlm.nih.gov/1861457

12 December 2020

based on heuristic

inferred from PubMed ID database lookup

Maple syrup urine disease: branched-chain keto acid decarboxylation in fibroblasts as measured with amino acids and keto acids

1 reference

https://pubmed.ncbi.nlm.nih.gov/1861457

12 December 2020

based on heuristic

inferred from PubMed ID database lookup

Effects of dietary protein intake on branched-chain keto acid dehydrogenase activity of the rat. Immunochemical analysis of the enzyme complex

1 reference

https://pubmed.ncbi.nlm.nih.gov/1861457

12 December 2020

based on heuristic

inferred from PubMed ID database lookup

Absence of branched chain acyl-transferase as a cause of maple syrup urine disease

1 reference

https://pubmed.ncbi.nlm.nih.gov/1861457

12 December 2020

based on heuristic

inferred from PubMed ID database lookup

Molecular phenotypes in cultured maple syrup urine disease cells. Complete E1 alpha cDNA sequence and mRNA and subunit contents of the human branched chain alpha-keto acid dehydrogenase complex

1 reference

https://pubmed.ncbi.nlm.nih.gov/1861457

12 December 2020

based on heuristic

inferred from PubMed ID database lookup

Phylogenetic comparisons of the branched-chain alpha-ketoacid dehydrogenase complex

1 reference

https://pubmed.ncbi.nlm.nih.gov/1861457

12 December 2020

based on heuristic

inferred from PubMed ID database lookup

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

1 reference

https://pubmed.ncbi.nlm.nih.gov/1861457

12 December 2020

based on heuristic

inferred from PubMed ID database lookup

Oxidative phosphorylation in mitochondria isolated from human fibroblasts

1 reference

https://pubmed.ncbi.nlm.nih.gov/1861457

12 December 2020

based on heuristic

inferred from PubMed ID database lookup

Classical maple syrup urine disease: cofactor resistance.

1 reference

https://pubmed.ncbi.nlm.nih.gov/1861457

12 December 2020

based on heuristic

inferred from PubMed ID database lookup

Isolation of rabbit liver branched chain alpha-ketoacid dehydrogenase and regulation by phosphorylation

1 reference

https://pubmed.ncbi.nlm.nih.gov/1861457

12 December 2020

based on heuristic

inferred from PubMed ID database lookup

Conservation of primary structure in the lipoyl-bearing and dihydrolipoyl dehydrogenase binding domains of mammalian branched-chain alpha-keto acid dehydrogenase complex: molecular cloning of human and bovine transacylase (E2) cDNAs

1 reference

https://pubmed.ncbi.nlm.nih.gov/1861457

12 December 2020

based on heuristic

inferred from PubMed ID database lookup

A rapid method for assay of branched-chain keto acid decarboxylation in cultured cells and its application to prenatal diagnosis of maple syrup urine disease

1 reference

https://pubmed.ncbi.nlm.nih.gov/1861457

12 December 2020

based on heuristic

inferred from PubMed ID database lookup

Activation of branched-chain alpha-ketoacid dehydrogenase complex by alpha-chloroisocaproate in normal and enzyme-deficient fibroblasts

1 reference

https://pubmed.ncbi.nlm.nih.gov/1861457

12 December 2020

based on heuristic

inferred from PubMed ID database lookup

Identifiers

10.1007/BF01804386

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