[HTML][HTML] Cystathionine protects against endoplasmic reticulum stress-induced lipid accumulation, tissue injury, and apoptotic cell death
KN Maclean, LS Greiner, JR Evans, SK Sood… - Journal of Biological …, 2012 - ASBMB
Cystathionine (RS-(2-amino-2-carboxyethyl)-l-homocysteine) is a non-proteinogenic
thioether containing amino acid. In mammals, cystathionine is formed as an intermediate of
the transsulfuration pathway by the condensation of serine and homocysteine (Hcy) in a
reaction catalyzed by cystathionine β-synthase (CBS). Cystathionine is subsequently
converted to cysteine plus ammonia and α-ketobutyrate by the action of cystathionine γ-
lyase (CGL). Pathogenic mutations in CBS result in CBS-deficient homocystinuria (HCU) …
thioether containing amino acid. In mammals, cystathionine is formed as an intermediate of
the transsulfuration pathway by the condensation of serine and homocysteine (Hcy) in a
reaction catalyzed by cystathionine β-synthase (CBS). Cystathionine is subsequently
converted to cysteine plus ammonia and α-ketobutyrate by the action of cystathionine γ-
lyase (CGL). Pathogenic mutations in CBS result in CBS-deficient homocystinuria (HCU) …