Metabolic changes associated with hyperammonemia in patients with propionic acidemia-Reference-Cited by-同舟云学术

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Metabolic changes associated with hyperammonemia in patients with propionic acidemia

Published:2006-06 Issue:2 Volume:88 Page:123-130
ISSN:1096-7192
Container-title:Molecular Genetics and Metabolism
language:en
Short-container-title:Molecular Genetics and Metabolism

Author:

Filipowicz Heather R.,Ernst Sharon L.,Ashurst Carrie L.,Pasquali Marzia,Longo Nicola

Publisher

Elsevier BV

Subject

Endocrinology,Genetics,Molecular Biology,Biochemistry,Endocrinology, Diabetes and Metabolism

Reference36 articles.

1. Disorders of propionate and methylmalonate metabolism;Fenton,2001

2. The glutamine paradox in a neonate with propionic acidaemia and severe hyperammonaemia;Ierardi-Curto;J. Inherit. Metab. Dis.,2000

3. Urea cycle enzymes;Brusilow,2001

4. Failure of the normal ureagenic response to amino acids in organic acid-loaded rats. Proposed mechanism for the hyperammonemia of propionic and methylmalonic acidemia;Stewart;J. Clin. Invest.,1980

5. Inhibition by propionyl-coenzyme A of N-acetylglutamate synthetase in rat liver mitochondria. A possible explanation for hyperammonemia in propionic and methylmalonic acidemia;Coude;J. Clin. Invest.,1979

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1. Fasting alleviates metabolic alterations in mice with propionyl-CoA carboxylase deficiency due to Pcca mutation;Communications Biology;2024-05-29

2. Intellectual disability and autism in propionic acidemia: a biomarker-behavioral investigation implicating dysregulated mitochondrial biology;Molecular Psychiatry;2024-01-11

3. Recognizing and Managing a Metabolic Crisis;Pediatric Clinics of North America;2023-10

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