Nutritional and Metabolic Diseases Commons^™

Effects Of Hydrogen Sulfide In Hyperhomocysteinemia-Mediated Skeletal Muscle Myopathy., Avisek Majumder

Electronic Theses and Dissertations

Although hyperhomocysteinemia (HHcy) occurs due to a deficiency in cystathionine-β-synthase (CBS), causing skeletal muscle myopathy, it is still unclear whether this effect is mediated through oxidative, endoplasmic reticulum (ER) stress, both or something else. Cystathionine γ-lyase (CSE) and CBS are the main H₂S producing enzymes, which produce Hydrogen sulfide (H₂S) from Hcy in the transsulfuration reaction. H₂S is an anti-oxidant compound and patients with a CBS deficiency cannot produce enough H₂S, thus rendering them vulnerable to excess Hcy-mediated damage. Both in-vitro (C2C12 cells) and in-vivo (CBS+/- mice) models were used to …