(Q45086488)

English

Maple syrup urine disease: interrelations between branched-chain amino-, oxo- and hydroxyacids; implications for treatment; associations with CNS dysmyelination

scientific article published on January 1, 1992

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scholarly article

1 reference

Europe PubMed Central

9 December 2017

Maple syrup urine disease: interrelations between branched-chain amino-, oxo- and hydroxyacids; implications for treatment; associations with CNS dysmyelination (English)

1 reference

Europe PubMed Central

https://www.ebi.ac.uk/europepmc/webservices/rest/search?resulttype=core&format=json&query=SRC%3AMED%20AND%20EXT_ID%3A1583867

11 October 2022

maple syrup urine disease

1 reference

based on heuristic

inferred from title

Eileen P Treacy

1

object named as

E. Treacy

1 reference

Europe PubMed Central

https://www.ebi.ac.uk/europepmc/webservices/rest/search?resulttype=core&format=json&query=SRC%3AMED%20AND%20EXT_ID%3A1583867

11 October 2022

author name string

C. L. Clow

2

1 reference

Europe PubMed Central

https://www.ebi.ac.uk/europepmc/webservices/rest/search?resulttype=core&format=json&query=SRC%3AMED%20AND%20EXT_ID%3A1583867

11 October 2022

T. R. Reade

3

1 reference

Europe PubMed Central

https://www.ebi.ac.uk/europepmc/webservices/rest/search?resulttype=core&format=json&query=SRC%3AMED%20AND%20EXT_ID%3A1583867

11 October 2022

D. Chitayat

4

1 reference

Europe PubMed Central

https://www.ebi.ac.uk/europepmc/webservices/rest/search?resulttype=core&format=json&query=SRC%3AMED%20AND%20EXT_ID%3A1583867

11 October 2022

O. A. Mamer

5

1 reference

Europe PubMed Central

https://www.ebi.ac.uk/europepmc/webservices/rest/search?resulttype=core&format=json&query=SRC%3AMED%20AND%20EXT_ID%3A1583867

11 October 2022

C. R. Scriver

6

1 reference

Europe PubMed Central

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11 October 2022

language of work or name

1 reference

National Center for Biotechnology Information

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/esummary.fcgi?db=pubmed&retmode=json&id=1583867

11 October 2022

publication date

1 January 1992

1 reference

Europe PubMed Central

9 December 2017

Journal of Inherited Metabolic Disease

1 reference

Europe PubMed Central

9 December 2017

15

1 reference

Europe PubMed Central

9 December 2017

121-135

1 reference

Europe PubMed Central

9 December 2017

1

1 reference

Europe PubMed Central

9 December 2017

https://scigraph.springernature.com/pub.10.1007/bf01800354

0 references

Demonstration of a new mammalian isoleucine catabolic pathway yielding an Rseries of metabolites

1 reference

https://api.crossref.org/works/10.1007%2FBF01800354

21 January 2018

Key role of L-alanine in the control of hepatic protein synthesis

1 reference

https://api.crossref.org/works/10.1007%2FBF01800354

21 January 2018

Branched-chain amino acid metabolism and alanine formation in rat muscles in vitro. Mitochondrial-cytosolic interrelationships

1 reference

https://api.crossref.org/works/10.1007%2FBF01800354

21 January 2018

CT and MRI in maple syrup urine disease.

1 reference

https://api.crossref.org/works/10.1007%2FBF01800354

21 January 2018

The 2-oxo acid dehydrogenase complexes: recent advances

1 reference

https://api.crossref.org/works/10.1007%2FBF01800354

21 January 2018

Maple syrup urine disease: findings on CT and MR scans of the brain in 10 infants

1 reference

https://pubmed.ncbi.nlm.nih.gov/1583867

12 December 2020

based on heuristic

inferred from PubMed ID database lookup

Stable isotope dilution assay for branched chain alpha-hydroxy-and alpha-ketoacids: serum concentrations for normal children

1 reference

https://pubmed.ncbi.nlm.nih.gov/1583867

12 December 2020

based on heuristic

inferred from PubMed ID database lookup

Maple syrup urine disease metabolites studies in cerebellum cultures.

1 reference

https://pubmed.ncbi.nlm.nih.gov/1583867

12 December 2020

based on heuristic

inferred from PubMed ID database lookup

Neutral amino acid transport at the human blood-brain barrier

1 reference

https://pubmed.ncbi.nlm.nih.gov/1583867

12 December 2020

based on heuristic

inferred from PubMed ID database lookup

Blood-brain transport of nutrients. Introduction

1 reference

https://pubmed.ncbi.nlm.nih.gov/1583867

12 December 2020

based on heuristic

inferred from PubMed ID database lookup

On the mechanism of L-alloisoleucine formation: studies on a healthy subject and in fibroblasts from normals and patients with maple syrup urine disease

1 reference

https://pubmed.ncbi.nlm.nih.gov/1583867

12 December 2020

based on heuristic

inferred from PubMed ID database lookup

Branched-chain alpha-keto acids isolated as oxime derivatives: relationship to the corresponding hydroxy acids and amino acids in maple syrup urine disease

1 reference

https://pubmed.ncbi.nlm.nih.gov/1583867

12 December 2020

based on heuristic

inferred from PubMed ID database lookup

Studies on requirements for amino acids in infants with disorders of amino acid metabolism. I. Effect of alanine

1 reference

https://pubmed.ncbi.nlm.nih.gov/1583867

12 December 2020

based on heuristic

inferred from PubMed ID database lookup

Correlations between branched-chain amino acids and branched-chain alpha-keto acids in blood in maple syrup urine disease

1 reference

https://pubmed.ncbi.nlm.nih.gov/1583867

12 December 2020

based on heuristic

inferred from PubMed ID database lookup

The effect of Mendelian disease on human health. II: Response to treatment

1 reference

https://pubmed.ncbi.nlm.nih.gov/1583867

12 December 2020

based on heuristic

inferred from PubMed ID database lookup

[Cranial computed tomography in maple syrup urine disease]

1 reference

https://pubmed.ncbi.nlm.nih.gov/1583867

12 December 2020

based on heuristic

inferred from PubMed ID database lookup

Protein and leucine metabolism in maple syrup urine disease

1 reference

https://pubmed.ncbi.nlm.nih.gov/1583867

12 December 2020

based on heuristic

inferred from PubMed ID database lookup

Transamination and oxidative decarboxylation rates of branched-chain 2-oxo acids in cultured human skin fibroblasts

1 reference

https://pubmed.ncbi.nlm.nih.gov/1583867

12 December 2020

based on heuristic

inferred from PubMed ID database lookup

So-called thiamin-responsive maple syrup urine disease: 15-year follow-up of the original patient

1 reference

https://pubmed.ncbi.nlm.nih.gov/1583867

12 December 2020

based on heuristic

inferred from PubMed ID database lookup

Molecular studies of mammalian branched-chain alpha-keto acid dehydrogenase complexes: domain structures, expression, and inborn errors

1 reference

https://pubmed.ncbi.nlm.nih.gov/1583867

12 December 2020

based on heuristic

inferred from PubMed ID database lookup

Blood-brain barrier transport of the alpha-keto acid analogs of amino acids.

1 reference

https://pubmed.ncbi.nlm.nih.gov/1583867

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/1583867

12 December 2020

based on heuristic

inferred from PubMed ID database lookup

Outcome of early and long-term management of classical maple syrup urine disease

1 reference

https://pubmed.ncbi.nlm.nih.gov/1583867

12 December 2020

based on heuristic

inferred from PubMed ID database lookup

Glucose and alanine metabolism in children with maple syrup urine disease

1 reference

https://pubmed.ncbi.nlm.nih.gov/1583867

12 December 2020

based on heuristic

inferred from PubMed ID database lookup

The relationship between the branched chain amino acids and their alpha-ketoacids in maple syrup urine disease

1 reference

https://pubmed.ncbi.nlm.nih.gov/1583867

12 December 2020

based on heuristic

inferred from PubMed ID database lookup

Maple syrup urine disease in calves: a clinical, pathological and biochemical study

1 reference

https://pubmed.ncbi.nlm.nih.gov/1583867

12 December 2020

based on heuristic

inferred from PubMed ID database lookup

Relationship of plasma leucine and alpha-ketoisocaproate during a L-[1-13C]leucine infusion in man: a method for measuring human intracellular leucine tracer enrichment

1 reference

https://pubmed.ncbi.nlm.nih.gov/1583867

12 December 2020

based on heuristic

inferred from PubMed ID database lookup

Myelin proteins: degradation in rat brain initiated by metabolites causative of maple syrup urine disease.

1 reference

https://pubmed.ncbi.nlm.nih.gov/1583867

12 December 2020

based on heuristic

inferred from PubMed ID database lookup

Acute metabolic encephalopathy: a review of causes, mechanisms and treatment

1 reference

https://pubmed.ncbi.nlm.nih.gov/1583867

12 December 2020

based on heuristic

inferred from PubMed ID database lookup

Interrelation between the metabolism of L-isoleucine and L-allo-isoleucine in patients with maple syrup urine disease

1 reference

https://pubmed.ncbi.nlm.nih.gov/1583867

12 December 2020

based on heuristic

inferred from PubMed ID database lookup

Plasma free amino acid values in normal children and adolescents

1 reference

https://pubmed.ncbi.nlm.nih.gov/1583867

12 December 2020

based on heuristic

inferred from PubMed ID database lookup

Organic acids and branched-chain amino acids in body fluids before and after multiple exchange transfusions in maple syrup urine disease

1 reference

https://pubmed.ncbi.nlm.nih.gov/1583867

12 December 2020

based on heuristic

inferred from PubMed ID database lookup

Thiamine-responsive maple-syrup-urine disease

1 reference

https://pubmed.ncbi.nlm.nih.gov/1583867

12 December 2020

based on heuristic

inferred from PubMed ID database lookup

Identifiers

10.1007/BF01800354

1 reference

Europe PubMed Central

9 December 2017

Dimensions Publication ID

0 references

1 reference

Europe PubMed Central

9 December 2017

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