ReviewCold Spring Harbor perspectives in biology2013
Altered sulfide (H(2)S) metabolism in ethylmalonic encephalopathy.
Review in Cold Spring Harbor perspectives in biology, 2013. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 29 papers.
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Who cites it
29 citing papers in PubMed, 47 citations in OpenAlex.
- Ethylmalonic and Methylsuccinic Acids Disrupt Bioenergetics and Induce Mitochondrial Permeability Transition Through Thiol Redox Modulation in Rat Striatum: Potential Mechanisms Involved in Ethylmalonic Encephalopathy.Neurochemical research · 2026Article
- Intracerebral Administration of Hydrogen Sulfide Impairs Bioenergetics, Redox Status and Mitochondrial Quality Control in Rat Striatum.Neurotoxicity research · 2025Article
- Mitochondrial Unfolded Protein Response (mtUPR) Activation Improves Pathological Alterations in Cellular Models of Ethylmalonic Encephalopathy.Antioxidants (Basel, Switzerland) · 2025Article
- Ethylmalonic Encephalopathy: a literature review and two new cases of mild phenotype.Neurological sciences : official journal of the Italian Neurological Society and of the Italian Society of Clinical Neurophysiology · 2023Review
- Clinical Applications for Gasotransmitters in the Cardiovascular System: Are We There Yet?International journal of molecular sciences · 2023Review
- Cysteine dioxygenase type 1 (CDO1): Its functional role in physiological and pathophysiological processes.Genes & diseases · 2023Review
- Advances in Human Mitochondria-Based Therapies.International journal of molecular sciences · 2022Review
- AAV-vector based gene therapy for mitochondrial disease: progress and future perspectives.Orphanet journal of rare diseases · 2022Review
- Mitochondrial Dysfunction and Redox Homeostasis Impairment as Pathomechanisms of Brain Damage in Ethylmalonic Encephalopathy: Insights from Animal and Human Studies.Cellular and molecular neurobiology · 2022Review
- Defense responses of sulfur dioxygenase to sulfide stress in the razor clam Sinonovacula constricta.Genes & genomics · 2021Article
- Clinical and biochemical footprints of inherited metabolic diseases. IV. Metabolic cardiovascular disease.Molecular genetics and metabolism · 2021Review
- Abnormalities of hydrogen sulfide and glutathione pathways in mitochondrial dysfunction.Journal of advanced research · 2021Review
- Ethylmalonic encephalopathy: Clinical course and therapy response in an uncommon mild case with a severe ETHE1 mutation.Molecular genetics and metabolism reports · 2020Article
- Compromised therapeutic value of pediatric liver transplantation in ethylmalonic encephalopathy: A case report.World journal of gastroenterology · 2020Article
- Application of Antibiotics/Antimicrobial Agents on Dental Caries.BioMed research international · 2020Review
- Neurological manifestations of organic acidurias.Nature reviews. Neurology · 2019Review
- Homeostatic impact of sulfite and hydrogen sulfide on cysteine catabolism.British journal of pharmacology · 2019Review
- Article
- Cysteine Metabolism in Neuronal Redox Homeostasis.Trends in pharmacological sciences · 2018Review
- Successful treatment of a patient with ethylmalonic encephalopathy by intravenous N-acetylcysteine.Metabolic brain disease · 2017Article
Corrections and comments
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Authors and funding
2 authors at 2 institutions in 1 country.
Funding
No grant is acknowledged in the PubMed record.
Abstract
Hydrogen sulfide (sulfide, H(2)S) is a colorless, water-soluble gas with a typical smell of rotten eggs. In the past, it has been investigated for its role as a potent toxic gas emanating from sewers and swamps or as a by-product of industrial processes. At high concentrations, H(2)S is a powerful inhibitor of cytochrome c oxidase; in trace amounts, it is an important signaling molecule, like nitric oxide (NO) and carbon monoxide (CO), together termed "gasotransmitters." This review will cover the physiological role and the pathogenic effects of H(2)S, focusing on ethylmalonic encephalopathy, a human mitochondrial disorder caused by genetic abnormalities of sulfide metabolism. We will also discuss the options that are now conceivable for preventing genetically driven chronic H(2)S toxicity, taking into account that a complete understanding of the physiopathology of H(2)S has still to be achieved.
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Registered trials
Read under generation 80e0d062 · epoch 390. Bibliography from PubMed, PubMed Central and OpenAlex; grants from NIH RePORTER; trial links from ClinicalTrials.gov; estimates, votes and beliefs from the OpenQuestion graph.