PreprintCited by 229Amino Acid Enzymes and MetabolismMetabolism and Genetic DisordersBiochemical and Molecular Research

Amino Acid Metabolism

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Chandel, Navdeep S.·2021·Cold Spring Harbor Perspectives in Biology

Abstract

Figure 7. Overview of the urea cycle.The initial step for urea synthesis is catalyzed by the mitochondrial matrix enzyme carbamoyl phosphate synthetase I to incorporate the first nitrogen molecule.Subsequently, ornithine transcarbamoylase uses carbamoyl phosphate and ornithine as substrates to generate citrulline in the mitochondrial matrix.Next, citrulline is exported to the cytosol and converted into argininosuccinate by argininosuccinate synthetase.This reaction results in the incorporation of a second nitrogen atom from aspartate.Next, the enzyme argininosuccinase cleaves argininosuccinate to produce fumarate and arginine.Finally, the enzyme arginase converts arginine to urea and ornithine to complete the urea cycle.

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References 7 · Cited by 229

Research works printed but not linked: 1

  1. Krebs HA. 1964. The citric acid cycle. Nobel lecture, Decem- ber 11, 1953. In Nobel Lectures, Physiology or Medicine 1942-1962. The Nobel Foundation, Elsevier, Amsterdam.

Authors
Chandel, N. S.
Affiliations
Northwestern University (US)
Corresponding author
Chandel, Navdeep S.
Venue
Cold Spring Harbor Perspectives in Biology
Published
2021-04
Volume
13
Issue
4
Pages
a040584
ISSN
1943-0264 (ISSN-L)
OpenAlex
W4206120892
Cited by
229
References
7
Citations by year
2026: 70 · 2025: 91 · 2024: 49 · 2023: 12 · 2022: 3 · 2021: 4
Type
posted content
Language
en
Open access
Locations
  • Cold Spring Harbor Perspectives in Biology (publishedVersion)
  • PubMed Central (publishedVersion)
  • PubMed (publishedVersion)
Topics
  • Life Sciences › Biochemistry, Genetics and Molecular Biology › Biochemistry › Amino Acid Enzymes and Metabolism
  • Life Sciences › Biochemistry, Genetics and Molecular Biology › Clinical Biochemistry › Metabolism and Genetic Disorders
  • Life Sciences › Biochemistry, Genetics and Molecular Biology › Molecular Biology › Biochemical and Molecular Research
Keywords
urea cycle, amino acid metabolism, nitrogen metabolism, carbamoyl phosphate synthetase I, ornithine transcarbamylase, citrulline, argininosuccinate synthetase, arginase, ornithine, aspartic acid
MeSH terms
Amino Acids, Catecholamines, Energy Metabolism, Melanins, Synaptic Transmission, Nitric Oxide, Urea, Epigenesis, Genetic

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