{"database":"biostudies-literature","file_versions":[],"scores":null,"additional":{"omics_type":["Unknown"],"volume":["10"],"submitter":["Plate J"],"pubmed_abstract":["Sulfite is a neurotoxin, which is detoxified by the molybdenum cofactor (Moco)-dependent enzyme sulfite oxidase (SOX). In humans, SOX deficiency causes the formation of the glutamate analog S-Sulfocysteine (SSC) resulting in a constant overstimulation of ionotropic glutamatergic receptors. Overstimulation leads to seizures, severe brain damage, and early childhood death. SOX deficiency may be caused either by a mutated <i>sox</i> gene or by mutations in one of the genes of the multi-step Moco biosynthesis pathway. While patients affected in the first step of Moco biosynthesis can be treated by a substitution therapy, no therapy is available for patients affected either in the second or third step of Moco biosynthesis or with isolated SOX deficiency. In the present study, we used a combinat"],"journal":["Frontiers in pharmacology"],"pagination":["122"],"full_dataset_link":["https://www.ebi.ac.uk/biostudies/studies/S-EPMC6454129"],"repository":["biostudies-literature"],"pubmed_title":["S-Sulfocysteine Induces Seizure-Like Behaviors in Zebrafish."],"pmcid":["PMC6454129"],"pubmed_authors":["Koster RW","Hassan AH","Kruse T","Plate J","Lehne F","Sassen WA"],"additional_accession":[]},"is_claimable":false,"name":"S-Sulfocysteine Induces Seizure-Like Behaviors in Zebrafish.","description":"Sulfite is a neurotoxin, which is detoxified by the molybdenum cofactor (Moco)-dependent enzyme sulfite oxidase (SOX). In humans, SOX deficiency causes the formation of the glutamate analog S-Sulfocysteine (SSC) resulting in a constant overstimulation of ionotropic glutamatergic receptors. Overstimulation leads to seizures, severe brain damage, and early childhood death. SOX deficiency may be caused either by a mutated <i>sox</i> gene or by mutations in one of the genes of the multi-step Moco biosynthesis pathway. While patients affected in the first step of Moco biosynthesis can be treated by a substitution therapy, no therapy is available for patients affected either in the second or third step of Moco biosynthesis or with isolated SOX deficiency. In the present study, we used a combinat","dates":{"release":"2019-01-01T00:00:00Z","publication":"2019","modification":"2025-06-01T12:02:32.734Z","creation":"2025-06-01T12:02:32.734Z"},"accession":"S-EPMC6454129","cross_references":{"pubmed":["31001109"],"doi":["10.3389/fphar.2019.00122"]}}