{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,2,14]],"date-time":"2026-02-14T00:53:09Z","timestamp":1771030389974,"version":"3.50.1"},"reference-count":41,"publisher":"Portland Press Ltd.","issue":"1","content-domain":{"domain":["portlandpress.com"],"crossmark-restriction":true},"short-container-title":[],"published-print":{"date-parts":[[2016,1,1]]},"abstract":"<jats:p>Hydrogen sulfide is an endogenous gasotransmitter and its mechanism of action involves activation of ATP-sensitive K+ channels and phosphodiesterase inhibition. As both mechanisms are potentially involved in malignant hyperthermia (MH), in the present study we addressed the involvement of the L-cysteine\/hydrogen sulfide pathway in MH. Skeletal muscle biopsies obtained from 25 MH-susceptible (MHS) and 56 MH-negative (MHN) individuals have been used to perform the in\u00a0vitro contracture test (IVCT). Quantitative real-time PCR (qPCR) and Western blotting studies have also been performed. Hydrogen sulfide levels are measured in both tissue samples and plasma. In MHS biopsies an increase in cystathionine \u03b2-synthase (CBS) occurs, as both mRNA and protein expression compared with MHN biopsies. Hydrogen sulfide biosynthesis is increased in MHS biopsies (0.128\u00b10.12 compared with 0.943\u00b10.13 nmol\/mg of protein per min for MHN and MHS biopsies, respectively; P&amp;lt;0.01). Addition of sodium hydrosulfide (NaHS) to MHS samples evokes a response similar, in the IVCT, to that elicited by either caffeine or halothane. Incubation of MHN biopsies with NaHS, before caffeine or halothane challenge, switches an MHN to an MHS response. In conclusion we demonstrate the involvement of the L-cysteine\/hydrogen sulfide pathway in MH, giving new insight into MH molecular mechanisms. This finding has potential implications for clinical care and could help to define less invasive diagnostic procedures.<\/jats:p>","DOI":"10.1042\/cs20150521","type":"journal-article","created":{"date-parts":[[2015,10,13]],"date-time":"2015-10-13T17:07:40Z","timestamp":1444756060000},"page":"35-44","update-policy":"https:\/\/doi.org\/10.1042\/crossmark_policy","source":"Crossref","is-referenced-by-count":23,"title":["Cystathionine \u03b2-synthase-derived hydrogen sulfide is involved in human malignant hyperthermia"],"prefix":"10.1042","volume":"130","author":[{"given":"Valentina","family":"Vellecco","sequence":"first","affiliation":[{"name":"Department of Pharmacy, University of Naples \u2018Federico II\u2019, Naples, Italy"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Antonio","family":"Mancini","sequence":"additional","affiliation":[{"name":"Centre of Biotechnology, Cardarelli Hospital, Naples, Italy"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Angela","family":"Ianaro","sequence":"additional","affiliation":[{"name":"Department of Pharmacy, University of Naples \u2018Federico II\u2019, Naples, Italy"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Vincenzo","family":"Calderone","sequence":"additional","affiliation":[{"name":"Department of Pharmacy, University of Pisa, Pisa, Italy"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Chiara","family":"Attanasio","sequence":"additional","affiliation":[{"name":"Centre for Advanced Biomaterials for Health Care (CRIB), Italian Institute of Technology, Naples, Italy"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Anna","family":"Cantalupo","sequence":"additional","affiliation":[{"name":"Department of Pharmacy, University of Naples \u2018Federico II\u2019, Naples, Italy"},{"name":"Weill Cornell University Medical College, New York City, NY 10065, U.S.A."}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Barbara","family":"Andria","sequence":"additional","affiliation":[{"name":"Centre of Biotechnology, Cardarelli Hospital, Naples, Italy"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Gennaro","family":"Savoia","sequence":"additional","affiliation":[{"name":"Unit of Anaesthesiology and Resuscitation IV, Cardarelli Hospital, Naples, Italy"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Elisabetta","family":"Panza","sequence":"additional","affiliation":[{"name":"Department of Pharmacy, University of Naples \u2018Federico II\u2019, Naples, Italy"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Antonietta","family":"Di Martino","sequence":"additional","affiliation":[{"name":"Centre of Biotechnology, Cardarelli Hospital, Naples, Italy"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Giuseppe","family":"Cirino","sequence":"additional","affiliation":[{"name":"Department of Pharmacy, University of Naples \u2018Federico II\u2019, Naples, Italy"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Mariarosaria","family":"Bucci","sequence":"additional","affiliation":[{"name":"Department of Pharmacy, University of Naples \u2018Federico II\u2019, Naples, Italy"}],"role":[{"role":"author","vocabulary":"crossref"}]}],"member":"288","published-online":{"date-parts":[[2015,11,30]]},"reference":[{"key":"2021113010394146500_B1","doi-asserted-by":"publisher","first-page":"1131","DOI":"10.1016\/S0140-6736(98)03078-5","article-title":"Malignant hyperthermia","volume":"352","author":"Denborough","year":"1998","journal-title":"Lancet"},{"key":"2021113010394146500_B2","doi-asserted-by":"publisher","first-page":"281","DOI":"10.1007\/BF03012921","article-title":"Metabolic events with spontaneous malignant hyperthermia","volume":"45","author":"Gronert","year":"1998","journal-title":"Can. 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