The role of glutamate oxaloacetate transaminases in sulfite biosynthesis and H

Glutamates Oxaloacetates Sulfite Oxidase / genetics Sulfites Transaminases / genetics
6): cysteine catabolism Glutamate oxaloacetate transaminase H(2)S Persulfidation Sulfite oxidase sulfide:quinone oxidoreductase

Journal

Redox biology
ISSN: 2213-2317
Titre abrégé: Redox Biol
Pays: Netherlands
ID NLM: 101605639

Informations de publication

Date de publication:
01 2021
Historique:
received: 02 10 2020
revised: 06 11 2020
accepted: 12 11 2020
pubmed: 4 12 2020
medline: 22 6 2021
entrez: 3 12 2020
Statut: ppublish

Résumé

Molybdenum cofactor deficiency and isolated sulfite oxidase deficiency are two rare genetic disorders that are caused by impairment of the mitochondrial enzyme sulfite oxidase. Sulfite oxidase is catalyzing the terminal reaction of cellular cysteine catabolism, the oxidation of sulfite to sulfate. Absence of sulfite oxidase leads to the accumulation of sulfite, which has been identified as a cellular toxin. However, the molecular pathways leading to the production of sulfite are still not completely understood. In order to identify novel treatment options for both disorders, the understanding of cellular cysteine catabolism - and its alterations upon loss of sulfite oxidase - is of utmost importance. Here we applied a new detection method of sulfite in cellular extracts to dissect the contribution of cytosolic and mitochondrial glutamate oxaloacetate transaminase (GOT) in the transformation of cysteine sulfinic acid to sulfite and pyruvate. We found that the cytosolic isoform GOT1 is primarily responsible for the production of sulfite. Moreover, loss of sulfite oxidase activity results in the accumulation of sulfite, H

Identifiants

DOI: 10.1016/j.redox.2020.101800 PMID: 33271457 PMC: PMC7711302
pubmed: 33271457
pii: S2213-2317(20)31005-3
doi: 10.1016/j.redox.2020.101800
pmc: PMC7711302
pii:
doi:

Substances chimiques

Glutamates 0
Oxaloacetates 0
Sulfites 0
Sulfite Oxidase EC 1.8.3.1
Transaminases EC 2.6.1.-

Types de publication

Journal Article Research Support, N.I.H., Extramural Research Support, Non-U.S. Gov't

Langues

eng

Sous-ensembles de citation

IM

Pagination

101800

Subventions

Organisme : NINDS NIH HHS
ID : K12 NS098482
Pays : United States

Informations de copyright

Copyright © 2020 The Author(s). Published by Elsevier B.V. All rights reserved.

Auteurs

Anna-Theresa Mellis (AT)

Institute for Biochemistry, Department of Chemistry, University of Cologne, Cologne, Germany.

Albert L Misko (AL)

Department of Neurology, Massachusetts General Hospital and Harvard Medical School, Boston, USA.

Sita Arjune (S)

Institute for Biochemistry, Department of Chemistry, University of Cologne, Cologne, Germany.

Ye Liang (Y)

Department of Neurology, Massachusetts General Hospital and Harvard Medical School, Boston, USA.

Katalin Erdélyi (K)

Department of Molecular Immunology and Toxicology, National Institute of Oncology, Budapest, Hungary.

Tamás Ditrói (T)

Department of Molecular Immunology and Toxicology, National Institute of Oncology, Budapest, Hungary.

Alexander T Kaczmarek (AT)

Institute for Biochemistry, Department of Chemistry, University of Cologne, Cologne, Germany.

Peter Nagy (P)

Department of Molecular Immunology and Toxicology, National Institute of Oncology, Budapest, Hungary; Department of Anatomy and Histology, University of Veterinary Medicine, Budapest, Hungary.

Guenter Schwarz (G)

Institute for Biochemistry, Department of Chemistry, University of Cologne, Cologne, Germany; Center for Molecular Medicine, University of Cologne, Cologne, Germany. Electronic address: gschwarz@uni-koeln.de.

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Classifications MeSH

questionsmedicales.fr › Acides aminés, peptides et protéines › Acides aminés › Acides aminés dicarboxyliques › Glutamates › The role of glutamate oxaloacetate...
questionsmedicales.fr › Composés chimiques organiques › Acides carboxyliques › Cétoacides › Oxaloacétates › The role of glutamate oxaloacetate...
questionsmedicales.fr › Enzymes et coenzymes › Enzymes › Oxidoreductases › Oxidoreductases acting on sulfur group donors › Sulfite oxidase › The role of glutamate oxaloacetate...
questionsmedicales.fr › Produits chimiques inorganiques › Sulfites › The role of glutamate oxaloacetate...
questionsmedicales.fr › Enzymes et coenzymes › Enzymes › Transferases › Nitrogenous group transferases › Transaminases › The role of glutamate oxaloacetate...