(Q37442646)

20 August 2017

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

1 reference

20 August 2017

Nicholas Ah Mew

1

1 reference

20 August 2017

Irma Payan

2

1 reference

20 August 2017

Yevgeny Daikhin

3

1 reference

20 August 2017

Ilana Nissim

4

1 reference

20 August 2017

Itzhak Nissim

5

1 reference

20 August 2017

Mendel Tuchman

6

1 reference

20 August 2017

Marc Yudkoff

7

1 reference

20 August 2017

14 July 2009

1 reference

Molecular Genetics and Metabolism

20 August 2017

1 reference

20 August 2017

98

1 reference

20 August 2017

325-330

1 reference

20 August 2017

4

1 reference

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

20 August 2017

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=2784258

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

23 August 2017

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=2784258

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

23 August 2017

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=2784258

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

23 August 2017

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=2784258

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

23 August 2017

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=2784258

Null mutations in the N-acetylglutamate synthase gene associated with acute neonatal disease and hyperammonemia

23 August 2017

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=2784258

Mixture of N-carbamoyl-L-glutamate plus L-arginine can protect rats with liver cirrhosis from acute ammonia intoxication

23 August 2017

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=2784258

In vivo urea cycle flux distinguishes and correlates with phenotypic severity in disorders of the urea cycle

23 August 2017

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=2784258

In vivo nitrogen metabolism in ornithine transcarbamylase deficiency

23 August 2017

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=2784258

N-Acetylglutamate synthetase deficiency responding to carbamylglutamate

23 August 2017

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=2784258

The glucose-alanine cycle

14 September 2017

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=2784258

Recent advances in the mechanism of pyridoxine-responsive disorders

14 September 2017

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=2784258

Urea cycle disorders: diagnosis, pathophysiology, and therapy.

14 September 2017

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=2784258

Orotic aciduria due to arginine deprivation: changes in the levels of carbamoyl phosphate and of other urea cycle intermediates in mouse liver

28 September 2017

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=2784258

Reassessment of primed constant-infusion tracer method to measure urea kinetics.

28 September 2017

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=2784258

N-acetylglutamate synthetase deficiency: diagnosis, management and follow-up of a rare disorder of ammonia detoxication

26 June 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=2784258

Role of Acetylglutamate in Ureotelism. Variations in Acetylglutamate Level and Its Possible Significance in Control of Urea Synthesis in Mammalian Liver

26 June 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=2784258

N-carbamylglutamate protects patients with decompensated propionic aciduria from hyperammonaemia

26 June 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=2784258

Late onset N-acetylglutamate synthase deficiency caused by hypomorphic alleles

2 September 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=2784258

Biopterin responsive phenylalanine hydroxylase deficiency

2 September 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=2784258

Differential effects of fasting and protein-free diets on levels of urea cycle enzymes in rat liver

2 September 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=2784258

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

2 September 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=2784258

Alanine metabolism in the perfused rat liver. Studies with (15)N.

2 September 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=2784258

N-acetylglutamate synthetase deficiency: favourable experience with carbamylglutamate.

2 September 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=2784258

2 September 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=2784258

N-acetylglutamate synthetase (NAGS) deficiency: diagnosis, clinical observations and treatment

2 September 2018

1 reference

https://api.crossref.org/works/10.1016%2FJ.YMGME.2009.07.010

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

7 January 2021

1 reference

https://api.crossref.org/works/10.1016%2FJ.YMGME.2009.07.010

Propionic acidemia with severe hyperammonemia and defective glycine metabolism

7 January 2021

1 reference

https://api.crossref.org/works/10.1016%2FJ.YMGME.2009.07.010

Occurrence of hyperammonemia in the course of 17 cases of methylmalonic acidemia

7 January 2021

1 reference

https://api.crossref.org/works/10.1016%2FJ.YMGME.2009.07.010

Hyperinsulinism-hyperammonemia syndrome caused by mutant glutamate dehydrogenase accompanied by novel enzyme kinetics

7 January 2021

1 reference

https://api.crossref.org/works/10.1016%2FJ.YMGME.2009.07.010

A mechanism for valproate-induced hyperammonemia

7 January 2021

1 reference

https://api.crossref.org/works/10.1016%2FJ.YMGME.2009.07.010

7 January 2021

Control of ureogenesis

1 reference

https://api.crossref.org/works/10.1016%2FJ.YMGME.2009.07.010

o-Phthaldialdehyde precolumn derivatization and reversed-phase high-performance liquid chromatography of polypeptide hydrolysates and physiological fluids

7 January 2021

1 reference

https://api.crossref.org/works/10.1016%2FJ.YMGME.2009.07.010

An improved glucose-oxidase method for determining blood, C.S.F. and urine glucose levels

7 January 2021

1 reference

https://api.crossref.org/works/10.1016%2FJ.YMGME.2009.07.010

Total body water measurement in humans with 18O and 2H labeled water

7 January 2021

1 reference

https://api.crossref.org/works/10.1016%2FJ.YMGME.2009.07.010

Integrative physiology of splanchnic glutamine and ammonium metabolism

7 January 2021

1 reference

https://api.crossref.org/works/10.1016%2FJ.YMGME.2009.07.010

Estimated frequency of urea cycle enzymopathies in Japan

7 January 2021

1 reference

https://api.crossref.org/works/10.1016%2FJ.YMGME.2009.07.010

Studies on the availability of intramitochondrial carbamoylphosphate for utilization in extramitochondrial reactions in rat liver

7 January 2021

1 reference

https://api.crossref.org/works/10.1016%2FJ.YMGME.2009.07.010

The catalytic rôle of carbamyl glutamate in citrulline biosynthesis

7 January 2021

1 reference

12 December 2020

Mitochondrial carbamyl phosphate and citrulline synthesis at high matrix acetylglutamate

1 reference

12 December 2020

Measurement of urea kinetics in humans: a validation of stable isotope tracer methods

1 reference

12 December 2020

The regulation of carbamyl phosphate synthetase (ammonia) in rat liver mitochondria. Effects of acetylglutamate concentration and ATP translocation

1 reference

12 December 2020

10.1016/J.YMGME.2009.07.010

Identifiers

DOI

1 reference

20 August 2017

1 reference

20 August 2017

1 reference