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Hydrogen sulfide generation in mammals: the molecular biology of cystathionine-β- synthase (CBS) and cystathionine-γ-lyase (CSE).
Inflamm Allergy Drug Targets
April 2011
Department of Experimental and Clinical Medicine, Street E, dal Pozzo, Pad. W, University of Perugia, Perugia, Italy.
Cystathionine-β-synthase (CBS) and cystathionine-γ-lyase (CSE) are two key enzymes involved in the synthesis of hydrogen sulphide (H(2)S). CBS catalyzes the pyridoxal 5'-phosphate (PLP)-dependent conversion of homocysteine in Cystathionine whilst CSE the pyridoxal 5'-phosphate (PLP)-dependent synthesis of L-cysteine from Cystathionine. In mammals, CBS gene transcription is poorly investigated and the activity of the enzyme is highly regulated.
View Article and Find Full Text PDFAncient origin of the CTH alelle carrying the c.200C>T (p.T67I) variant in patients with cystathioninuria.
Clin Genet
December 2010
CIBERER BIOBANK, CIBER de Enfermedades Raras (CIBERER), Valencia, Spain.
Hereditary cystathioninuria is due to mutations in the CTH gene that encodes for cystathionase, a pyridoxal-5'-phosphate (PLP) dependent enzyme. To date, mutations in this gene have been described in 10 unrelated cystathioninuric patients. Enzyme assays have showed that mutated cystathionase exhibits lower activity than controls.
View Article and Find Full Text PDFKinetic properties of polymorphic variants and pathogenic mutants in human cystathionine gamma-lyase.
Biochemistry
June 2008
Redox Biology Center and Department of Biochemistry, University of Nebraska, Lincoln, Nebraska 68588-0664, USA.
Human cystathionine-gamma-lyase (CGL) is a pyridoxal-5'-phosphate (PLP)-dependent enzyme, which functions in the transsulfuration pathway that converts homocysteine to cysteine. In addition, CGL is one of two major enzymes that can catalyze the formation of hydrogen sulfide, an important gaseous signaling molecule. Recently, several mutations in CGL have been described in patients with cystathioninuria, a rare but poorly understood genetic disease.
View Article and Find Full Text PDFEffect of sulfur amino acids on stimulus-induced superoxide generation and translocation of p47phox and p67phox to cell membrane in human neutrophils and the scavenging of free radical.
Clin Chim Acta
March 2005
Department of Anesthesiology and Critical Care Medicine, Kochi Medical School, Nankoku-shi, Kochi 783-8505, Japan.
Background: Various cystathionine metabolites are in the urine of the patients with cystathioninuria. Among these metabolites, cystathionine ketimine significantly enhanced N-formyl-methionyl-leucyl-phenylalanine (fMLP)-induced superoxide generation in parallel with tyrosyl phosphorylation of 45 kDa protein in human neutrophils.
Methods: We investigated the effect of various sulfur amino acids on fMLP-, phorbol-12-myristate-13-acetate (PMA)- and arachidonic acid (AA)-induced superoxide generation in human neutrophils.
Novel priming compounds of cystathionine metabolites on superoxide generation in human neutrophils.
Biochem Biophys Res Commun
March 2000
Department of Chemistry, Kochi Medical School, Oko-cho, Nankoku-shi, Kochi, 783-8505, Japan.
Human peripheral blood polymorphonuclear leukocytes were preincubated with cystathionine and cystathionine metabolites found in the urine of patients with cystathioninuria. Among the cystathionine metabolites, cystathionine ketimine and N-acetyl-S-(3-oxo-3-carboxy-n-propyl) cysteine (NAc-OCPC) significantly enhanced the N-formylmethionylleucylphenylalanine (fMLP)-induced superoxide generation, but cystathionine, NAc-cystathionine, and cyclothionine did not enhance the superoxide generation. Cystathionine ketimine and NAc-OCPC also enhanced superoxide generation induced by opsonized zymosan (OZ) but not that induced by arachidonic acid (AA) and phorbol 12-myristate 13-acetate (PMA).
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