Homocystinuria is a rare inborn-error-inherited-metabolic disorder that results from a deficiency of an enzyme called cystathionine
beta-synthase (CBS) enzyme.
Hydrogen sulfide ([H.sub.2]S) is predominantly synthesized from L-cysteine via cystathionine
[gamma]-lyase in the heart and vasculature.
[gamma]-lyase deficiency protects mice from galactosamine/lipopolysaccharide-induced acute liver failure.
The SNP may also increase/decrease the enzyme's activity in managing its cellular activity as happens with CBS (cystathionine
The presentation describes the engineering of the cystathionine
gamma-lyase enzyme scaffold into a novel molecule with a unique specificity for degrading both cystine and cysteine.
Wilcock et al . demonstrated that DS could be triggered by abnormal proteins, including superoxide dismutase 1, collagen type VI, and cystathionine
[sz]-synthase, which are encoded on chromosome 21.
Psychiatric manifestations of homocystinuria due to cystathionine
beta-synthase deficiency: prevalence, natural history, and relationship to neurologic impairment and vitamin B6-responsiveness.
Some studies demonstrated the interaction between tryparedoxin and cystathionine
[gamma]-lyase (CGL), (38) although the enzyme found in our experiment was cystathionine
[beta]-synthase (CBS) (Tables 2 and 3, spots 5).
Upregulation of cysteine synthase and cystathionine
[beta]-synthase contributes to Leishmania braziliensis survival under oxidative stress.
In the transsulfuration pathway, homocysteine produces cystathionine
, the precursor for cysteine biosynthesis, catalyzed by vitamin B6-dependent cystathionine
beta synthase gene dose dependent vascular remodeling in murine model of hyperhomocysteinemia," International Journal of Physiology, Pathophysiology and Pharmacology, vol.