(Q35813392)

10 August 2017

N-carbamylglutamate augments ureagenesis and reduces ammonia and glutamine in propionic acidemia (English)

1 reference

10 August 2017

Nicholas Ah Mew

1

1 reference

10 August 2017

Robert McCarter

2

1 reference

10 August 2017

Yevgeny Daikhin

3

1 reference

10 August 2017

Itzhak Nissim

4

1 reference

10 August 2017

Marc Yudkoff

5

1 reference

10 August 2017

Mendel Tuchman

6

1 reference

10 August 2017

language of work or name

English

0 references

publication date

21 June 2010

1 reference

10 August 2017

1 reference

10 August 2017

126

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10 August 2017

1

1 reference

10 August 2017

e208-14

1 reference

Effects of a single dose of N-carbamylglutamate on the rate of ureagenesis

10 August 2017

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=3297024

Cross-sectional multicenter study of patients with urea cycle disorders in the United States

11 July 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=3297024

N-carbamylglutamate markedly enhances ureagenesis in N-acetylglutamate deficiency and propionic acidemia as measured by isotopic incorporation and blood biomarkers

11 July 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=3297024

Restoration of ureagenesis in N-acetylglutamate synthase deficiency by N-carbamylglutamate

11 July 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=3297024

Hyperinsulinism/hyperammonemia syndrome: insights into the regulatory role of glutamate dehydrogenase in ammonia metabolism

11 July 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=3297024

Isolation and characterization of a naturally occurring cofactor of carbamyl phosphate biosynthesis

11 July 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=3297024

In vivo research with stable isotopes in biochemistry, nutrition and clinical medicine: an overview

11 July 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=3297024

N-Acetylglutamate synthetase deficiency responding to carbamylglutamate

11 July 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=3297024

Urea cycle disorders: diagnosis, pathophysiology, and therapy.

11 July 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=3297024

Mutations participating in interallelic complementation in propionic acidemia

11 July 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=3297024

Failure of the normal ureagenic response to amino acids in organic acid-loaded rats. Proposed mechanism for the hyperammonemia of propionic and methylmalonic acidemia

11 July 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=3297024

Treatment of inborn errors of urea synthesis: activation of alternative pathways of waste nitrogen synthesis and excretion

11 July 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=3297024

Inhibition by propionyl-coenzyme A of N-acetylglutamate synthetase in rat liver mitochondria. A possible explanation for hyperammonemia in propionic and methylmalonic acidemia

11 July 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=3297024

N-carbamylglutamate in emergency management of hyperammonemia in neonatal acute onset propionic and methylmalonic aciduria.

11 July 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=3297024

Biochemical efficacy of N-carbamylglutamate in neonatal severe hyperammonaemia due to propionic acidaemia

24 September 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=3297024

N-carbamylglutamate for neonatal hyperammonaemia in propionic acidaemia

24 September 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=3297024

N-carbamylglutamate protects patients with decompensated propionic aciduria from hyperammonaemia

24 September 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=3297024

N-carbamylglutamate enhances ammonia detoxification in a patient with decompensated methylmalonic aciduria

24 September 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=3297024

N-acetylglutamate synthetase deficiency: favourable experience with carbamylglutamate.

24 September 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=3297024

Effect of propionic acid on fatty acid oxidation and ureagenesis

24 September 2018

1 reference

12 December 2020

Treatment of hyperammonemic coma caused by inborn errors of urea synthesis

1 reference

12 December 2020

N-acetylglutamate synthetase deficiency: a disorder of ammonia detoxication

1 reference

12 December 2020

Glycine cleavage system in ketotic hyperglycinemia: a reduction of H-protein activity

1 reference

12 December 2020

Valproic acid-induced hyperammonemia in mentally retarded adults

1 reference

12 December 2020

Role of glucose in the regulation of endogenous glucose production in the human newborn

1 reference

12 December 2020

A new neonatal case of N-acetylglutamate synthase deficiency treated by carbamylglutamate

1 reference

12 December 2020

Identifiers

DOI

10.1542/PEDS.2010-0008

1 reference

10 August 2017

1 reference

10 August 2017

1 reference