Article
Real-time detection of enzymatically formed hydrogen sulfide by pathogenic variants of cystathionine beta-synthase using hemoglobin I of Lucina pectinata as a biosensor.
Free radical biology & medicine - 1 Oct 2024
Myszkowska Joanna, Klotz Katharina, Leandro Paula, Kruger Warren D, Froese D Sean, Baumgartner Matthias R, Spiekerkoetter Ute, Hannibal Luciana
Abstract excerpt
Classical homocystinuria is a rare disease caused by mutations in cystathionine β-synthase (CBS) gene (OMIM 613381). CBS catalyzes the first step of the transsulfuration pathway that converts homocysteine (Hcy) into cystathionine (Cysta) via a number of co-substrates and mechanisms. Formation of Cysta by condensation of Hcy and cysteine (Cys) produces a molar equivalent of hydrogen sulfide (H2S). H2S plays...
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