(Q36296830)

English

Thiamin-responsive maple-syrup-urine disease: decreased affinity of the mutant branched-chain alpha-keto acid dehydrogenase for alpha-ketoisovalerate and thiamin pyrophosphate

scientific article

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scholarly article

1 reference

Europe PubMed Central

PMC publication ID

13 August 2017

Thiamin-responsive maple-syrup-urine disease: decreased affinity of the mutant branched-chain alpha-keto acid dehydrogenase for alpha-ketoisovalerate and thiamin pyrophosphate (English)

1 reference

Europe PubMed Central

PMC publication ID

13 August 2017

author name string

D T Chuang

1

1 reference

Europe PubMed Central

PMC publication ID

13 August 2017

L S Ku

2

1 reference

Europe PubMed Central

PMC publication ID

13 August 2017

R P Cox

3

1 reference

Europe PubMed Central

PMC publication ID

13 August 2017

language of work or name

0 references

publication date

1 May 1982

1 reference

Europe PubMed Central

PMC publication ID

13 August 2017

number of pages

5

1 reference

based on heuristic

inferred from page(s)

Proceedings of the National Academy of Sciences of the United States of America

1 reference

Europe PubMed Central

PMC publication ID

13 August 2017

79

1 reference

Europe PubMed Central

PMC publication ID

13 August 2017

10

1 reference

Europe PubMed Central

PMC publication ID

13 August 2017

3300-3304

1 reference

Europe PubMed Central

PMC publication ID

13 August 2017

Protein measurement with the Folin phenol reagent

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=346403

30 September 2017

Amino acid metabolism in mammalian cell cultures

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=346403

30 September 2017

Affinity of cystathionine beta-synthase for pyridoxal 5'-phosphate in cultured cells. A mechanism for pyridoxine-responsive homocystinuria

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=346403

30 September 2017

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://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=346403

30 September 2017

Pyruvate dehydrogenase complex activity in normal and deficient fibroblasts

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=346403

30 September 2017

The mitochondrial pyruvate carrier. Kinetics and specificity for substrates and inhibitors

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=346403

30 September 2017

Abnormality of a thiamine-requiring enzyme in patients with Wernicke-Korsakoff syndrome

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=346403

30 September 2017

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

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=346403

30 September 2017

Purification and characterization of branched chain alpha-ketoacid dehydrogenase from bovine liver mitochondria

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=346403

30 September 2017

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

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=346403

30 September 2017

Thiamine dependency in a patient with congenital lacticacidaemia due to pyruvate dehydrogenase deficiency

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=346403

30 September 2017

Thiamine-responsive maple-syrup-urine disease

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=346403

29 June 2018

Maple syrup urine disease variant--amino acid pattern and problems of treatment during acute attacks

1 reference

https://pubmed.ncbi.nlm.nih.gov/6954481

12 December 2020

based on heuristic

inferred from PubMed ID database lookup

Enzyme activity in classical and variant forms of maple syrup urine disease

1 reference

https://pubmed.ncbi.nlm.nih.gov/6954481

12 December 2020

based on heuristic

inferred from PubMed ID database lookup

Maple syrup urine disease. Thiamin responsive branched chain aminoaciduria: a unique case in an adult: (case report)

1 reference

https://pubmed.ncbi.nlm.nih.gov/6954481

12 December 2020

based on heuristic

inferred from PubMed ID database lookup

In Vivo and in Vitro Response of Human Branched Chain α-Ketoacid Dehydrogenase to Thiamine and Thiamine Pyrophosphate

1 reference

https://pubmed.ncbi.nlm.nih.gov/6954481

12 December 2020

based on heuristic

inferred from PubMed ID database lookup

Effects of thiamine in a patient with a variant form of branched-chian ketoaciduria

1 reference

https://pubmed.ncbi.nlm.nih.gov/6954481

12 December 2020

based on heuristic

inferred from PubMed ID database lookup

Effects of branched chain alpha-ketoacids on the metabolism of isolated rat liver cells. I. Regulation of branched chain alpha-ketoacid metabolism

1 reference

https://pubmed.ncbi.nlm.nih.gov/6954481

12 December 2020

based on heuristic

inferred from PubMed ID database lookup

Identifiers

10.1073/PNAS.79.10.3300

1 reference

Europe PubMed Central

PMC publication ID

13 August 2017

1982PNAS...79.3300C

0 references

PMC publication ID

1 reference

Europe PubMed Central

PMC publication ID

13 August 2017

PubMed publication ID

1 reference

Europe PubMed Central

PMC publication ID

13 August 2017

ResearchGate publication ID

0 references

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