(Q36900927)

17 August 2017

Classical maple syrup urine disease and brain development: principles of management and formula design (English)

1 reference

maple syrup urine disease

17 August 2017

1 reference

Kevin A Strauss

1

1 reference

17 August 2017

Bridget Wardley

2

1 reference

17 August 2017

Donna Robinson

3

1 reference

17 August 2017

Christine Hendrickson

4

1 reference

17 August 2017

Nicholas L Rider

5

1 reference

17 August 2017

Erik G Puffenberger

6

1 reference

17 August 2017

Diana Shellmer

7

1 reference

17 August 2017

Ann B Moser

8

1 reference

17 August 2017

D Holmes Morton

9

1 reference

17 August 2017

language of work or name

1 reference

12 January 2010

1 reference

Molecular Genetics and Metabolism

17 August 2017

1 reference

17 August 2017

99

1 reference

17 August 2017

4

1 reference

17 August 2017

333-345

1 reference

Genetics, medicine, and the Plain people

17 August 2017

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=3671925

Zinc: the brain's dark horse.

29 September 2017

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=3671925

Dual mechanism of brain injury and novel treatment strategy in maple syrup urine disease

29 September 2017

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=3671925

Role of docosahexaenoic acid in maternal and child mental health

29 September 2017

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=3671925

Nutritional and dietary influences on attention deficit hyperactivity disorder

29 September 2017

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=3671925

Omega-3 fatty acids as treatments for mental illness: which disorder and which fatty acid?

29 September 2017

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=3671925

Genetic polymorphisms in the amino acid transporters LAT1 and LAT2 in relation to the pharmacokinetics and side effects of melphalan

29 September 2017

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=3671925

The case for regulating indispensable amino acid metabolism: the branched-chain alpha-keto acid dehydrogenase kinase-knockout mouse

29 September 2017

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=3671925

Impaired growth and neurological abnormalities in branched-chain alpha-keto acid dehydrogenase kinase-deficient mice.

29 September 2017

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=3671925

Branched-chain amino acids and brain function

29 September 2017

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=3671925

Brain amino acid requirements and toxicity: the example of leucine

29 September 2017

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=3671925

The Oxford-Durham study: a randomized, controlled trial of dietary supplementation with fatty acids in children with developmental coordination disorder

29 September 2017

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=3671925

Nutrients for cognitive development in school-aged children

29 September 2017

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=3671925

Future role of large neutral amino acids in transport of phenylalanine into the brain

29 September 2017

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=3671925

BODY COMPOSITION IN SEVERE INFANTILE MALNUTRITION.

29 September 2017

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=3671925

Pediatric medicine and the genetic disorders of the Amish and Mennonite people of Pennsylvania

29 September 2017

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=3671925

Selenium and selenoproteins in the brain and brain diseases

29 September 2017

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=3671925

Relation between glutamine, branched-chain amino acids, and protein metabolism

29 September 2017

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=3671925

Can nutrient supplements modify brain function?

29 September 2017

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=3671925

Tracers to investigate protein and amino acid metabolism in human subjects

29 September 2017

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=3671925

Renal clearance of branched-chain L-amino and 2-oxo acids in maple syrup urine disease

29 September 2017

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=3671925

Large neutral amino acids block phenylalanine transport into brain tissue in patients with phenylketonuria

29 September 2017

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=3671925

Hypothesis: where the depleted plasma amino acids go in phenylketonuria, and why.

29 September 2017

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=3671925

Transport of amino acids across the blood-brain barrier: implications for treatment of maternal phenylketonuria

29 September 2017

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=3671925

Protein energy malnutrition (PEM), brain and various facets of child development

29 September 2017

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=3671925

Interaction Between Parallel Transport Systems Examined With Tryptophan and Related Amino Acids

29 September 2017

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=3671925

Developmental protein malnutrition: Influences on the central nervous system of the rat

29 September 2017

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=3671925

Plasma and red blood cell fatty acids in peroxisomal disorders

29 September 2017

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=3671925

Metabolism of branched-chain amino acids in maple syrup urine disease

27 June 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=3671925

Low selenium diet increases the dopamine turnover in prefrontal cortex of the rat.

27 June 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=3671925

The effect of valine deficiency on neutral amino acid patterns in plasma and brain of the rat.

27 June 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=3671925

Effect of undernutrition on amino acid compartimentation in the developing rat brain

27 June 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=3671925

Amino acid depletion in the blood and brain tissue of hyperphenylalaninemic rats is abolished by the administration of additional lysine: a contribution to the understanding of the metabolic defects in phenylketonuria.

27 June 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=3671925

1987 McCollum award lecture. Kinetics of human amino acid metabolism: nutritional implications and some lessons.

27 June 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=3671925

Kinetics of amino acid transport at the blood-brain barrier studied using an in situ brain perfusion technique.

27 June 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=3671925

Cerebral edema causing death in children with maple syrup urine disease

27 June 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=3671925

Abnormal dendritic development in maple syrup urine disease

27 June 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=3671925

Glutamate and gamma-aminobutyric acid neurotransmitter systems in the acute phase of maple syrup urine disease and citrullinemia encephalopathies in newborn calves

27 June 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=3671925

Influence of the Postoperative State on the Intracellular Free Amino Acids in Human Muscle Tissue

27 June 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=3671925

The effect of a low protein diet on the anatomical development of the rat brain

27 June 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=3671925

Regulation of leucine and alpha-ketoisocaproate metabolism in skeletal muscle

27 June 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=3671925

Cognitive functions and the antioxidant system in phenylketonuric patients

27 June 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=3671925

Prevention of brain disease from severe 5,10-methylenetetrahydrofolate reductase deficiency.

4 September 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=3671925

Prolonged selenium-deficient diet in MsrA knockout mice causes enhanced oxidative modification to proteins and affects the levels of antioxidant enzymes in a tissue-specific manner.

4 September 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=3671925

Social outcome in adults with maple syrup urine disease (MSUD).

4 September 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=3671925

Nutrition and the developing brain: nutrient priorities and measurement.

4 September 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=3671925

Elective liver transplantation for the treatment of classical maple syrup urine disease.

4 September 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=3671925

Impact of longitudinal plasma leucine levels on the intellectual outcome in patients with classic MSUD.

4 September 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=3671925

Maple syrup urine disease-treatment and outcome in patients of Turkish descent in Germany.

4 September 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=3671925

Branched-chain Ketoacyl Dehydrogenase Deficiency: Maple Syrup Disease.

4 September 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=3671925

Diagnosis and treatment of maple syrup disease: a study of 36 patients

4 September 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=3671925

Large neutral amino acids auto exchange when infused by microdialysis into the rat brain: implication for maple syrup urine disease and phenylketonuria

4 September 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=3671925

Predominant functional activity of the large, neutral amino acid transporter (LAT1) isoform at the cerebrovasculature

4 September 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=3671925

A tryptophan-deficient corn-based diet induces plastic responses in cerebellar cortex cells of rat offspring

4 September 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=3671925

Function of leucine in excitatory neurotransmitter metabolism in the central nervous system.

4 September 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=3671925

Dendritic spine pathology in infants with severe protein-calorie malnutrition.

4 September 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=3671925

Through my window--remarks at the 125th year celebration of Children's Hospital of Boston.

4 September 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=3671925

Brain lipids, proteolipids, and free amino acids in maple syrup urine disease

4 September 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=3671925

Selenium and brain function: a poorly recognized liaison

4 September 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=3671925

Astrocyte leucine metabolism: significance of branched-chain amino acid transamination

4 December 2018

1 reference

https://api.crossref.org/works/10.1016%2FJ.YMGME.2009.12.007

Inhibition of astrocyte glutamine production by alpha-ketoisocaproic acid

7 January 2021

1 reference

https://api.crossref.org/works/10.1016%2FJ.YMGME.2009.12.007

Blood phospholipid fatty acid analysis of adults with and without attention deficit/hyperactivity disorder

7 January 2021

1 reference

https://api.crossref.org/works/10.1016%2FJ.YMGME.2009.12.007

Increase in dopamine turnover and tyrosine hydroxylase enzyme in hippocampus of rats fed on low selenium diet

7 January 2021

1 reference

https://api.crossref.org/works/10.1016%2FJ.YMGME.2009.12.007

Serum selenium levels in individuals on PKU diets

7 January 2021

1 reference

https://api.crossref.org/works/10.1016%2FJ.YMGME.2009.12.007

Selenium requirements in patients with inborn errors of amino acid metabolism and selenium deficiency

7 January 2021

1 reference

https://api.crossref.org/works/10.1016%2FJ.YMGME.2009.12.007

Estimation of body water compartments of preschool children. I. Normal children

7 January 2021

1 reference

https://api.crossref.org/works/10.1016%2FJ.YMGME.2009.12.007

The dendritic trees of neurons from the hippocampal formation of protein-deprived adult rats. A quantitative Golgi study

7 January 2021

1 reference

https://api.crossref.org/works/10.1016%2FJ.YMGME.2009.12.007

Dendritic development in neocortex of infants with early postnatal life undernutrition

7 January 2021

1 reference

https://api.crossref.org/works/10.1016%2FJ.YMGME.2009.12.007

The effects of protein deprivation on the nucleus raphe dorsalis: A morphometric golgi study in rats of three age groups

7 January 2021

1 reference

https://api.crossref.org/works/10.1016%2FJ.YMGME.2009.12.007

Low selenium diet affects monoamine turnover differentially in substantia nigra and striatum

7 January 2021

1 reference

https://api.crossref.org/works/10.1016%2FJ.YMGME.2009.12.007

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

7 January 2021

1 reference

PubMed

https://pubmed.ncbi.nlm.nih.gov/20061171

12 December 2020

inferred from PubMed ID database lookup

[Advances in the diagnosis and treatment of maple syrup urine disease: experience in Galicia (Spain)]

1 reference

PubMed

https://pubmed.ncbi.nlm.nih.gov/20061171

12 December 2020

inferred from PubMed ID database lookup

Essential fatty acids, DHA and human brain

1 reference

PubMed

https://pubmed.ncbi.nlm.nih.gov/20061171

12 December 2020

inferred from PubMed ID database lookup

Identifiers

DOI

10.1016/J.YMGME.2009.12.007

1 reference

release_pdo7oswdsfc33jx42pz7epni2q

17 August 2017

1 reference

Fatcat

https://api.fatcat.wiki/v0/release/pdo7oswdsfc33jx42pz7epni2q

24 November 2022

mapped directly with Wikidata item

1 reference

17 August 2017

1 reference