Expression of mutant human cystathionine beta-synthase rescues neonatal lethality but not homocystinuria in a mouse model.
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Cysteine and hydrogen sulphide in the regulation of metabolism: insights from genetics and pharmacologyA systematic analysis of cell cycle regulators in yeast reveals that most factors act independently of cell size to control initiation of divisionActivation of mutant enzyme function in vivo by proteasome inhibitors and treatments that induce Hsp70Diet-induced hyperhomocysteinemia increases amyloid-beta formation and deposition in a mouse model of Alzheimer's disease.Cystathionine gamma-Lyase-deficient mice require dietary cysteine to protect against acute lethal myopathy and oxidative injury.Regulation of homocysteine metabolism and methylation in human and mouse tissuesProtein arginine hypomethylation in a mouse model of cystathionine β-synthase deficiency.A revisit to the natural history of homocystinuria due to cystathionine beta-synthase deficiency.Cystathionine beta-synthase deficiency causes fat loss in mice.Methionine-deficient diet induces post-transcriptional downregulation of cystathionine β-synthase.Vascular complications of cystathionine β-synthase deficiency: future directions for homocysteine-to-hydrogen sulfide researchInhibition of betaine-homocysteine S-methyltransferase in rats causes hyperhomocysteinemia and reduces liver cystathionine β-synthase activity and methylation capacityCystathionine beta synthase gene dose dependent vascular remodeling in murine model of hyperhomocysteinemia.Paradoxical absence of a prothrombotic phenotype in a mouse model of severe hyperhomocysteinemia.Chemical chaperone rescue of mutant human cystathionine beta-synthaseEmerging role of hydrogen sulfide in hypertension and related cardiovascular diseasesA functional variant in the cystathionine β-synthase gene promoter significantly reduces congenital heart disease susceptibility in a Han Chinese population.Betaine supplementation is less effective than methionine restriction in correcting phenotypes of CBS deficient mice.The effect of dietary modulation of sulfur amino acids on cystathionine β synthase-deficient mice.Murine models of hyperhomocysteinemia and their vascular phenotypesValproic acid increases expression of methylenetetrahydrofolate reductase (MTHFR) and induces lower teratogenicity in MTHFR deficiencyCystathionine beta-synthase p.S466L mutation causes hyperhomocysteinemia in mice.Diversity of cystathionine beta-synthase haplotypes bearing the most common homocystinuria mutation c.833T>C: a possible role for gene conversionMouse models of cystathionine beta-synthase deficiency reveal significant threshold effects of hyperhomocysteinemia.The Herp protein pathway is not involved in the pro-amyloidogenic effect of hyperhomocysteinemiaEffect of endotoxemia in mice genetically deficient in cystathionine-γ-lyase, cystathionine-β-synthase or 3-mercaptopyruvate sulfurtransferase.Hyperhomocysteinemia promotes inflammatory monocyte generation and accelerates atherosclerosis in transgenic cystathionine beta-synthase-deficient miceCystathionine β-synthase-deficient mice thrive on a low-methionine diet.Correction of cystathionine β-synthase deficiency in mice by treatment with proteasome inhibitors.H2S biosynthesis and catabolism: new insights from molecular studies.Hypermethioninemias of genetic and non-genetic origin: A review.Cystathionine β-synthase deficiency: Of mice and men.Lack of global epigenetic methylation defects in CBS deficient mice.Dietary intake of S-(alpha-carboxybutyl)-DL-homocysteine induces hyperhomocysteinemia in rats.Cystathionine protects against endoplasmic reticulum stress-induced lipid accumulation, tissue injury, and apoptotic cell death.A novel transgenic mouse model of CBS-deficient homocystinuria does not incur hepatic steatosis or fibrosis and exhibits a hypercoagulative phenotype that is ameliorated by betaine treatment.Cystathionine beta-synthase mutations: effect of mutation topology on folding and activity.Enzyme replacement therapy prevents loss of bone and fat mass in murine homocystinuria.International Union of Basic and Clinical Pharmacology. CII: Pharmacological Modulation of H2S Levels: H2S Donors and H2S Biosynthesis Inhibitors.The c.797 G>A (p.R266K) cystathionine β-synthase mutation causes homocystinuria by affecting protein stability.
P2860
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P2860
Expression of mutant human cystathionine beta-synthase rescues neonatal lethality but not homocystinuria in a mouse model.
description
2005 nî lūn-bûn
@nan
2005 թուականի Յունիսին հրատարակուած գիտական յօդուած
@hyw
2005 թվականի հունիսին հրատարակված գիտական հոդված
@hy
2005年の論文
@ja
2005年論文
@yue
2005年論文
@zh-hant
2005年論文
@zh-hk
2005年論文
@zh-mo
2005年論文
@zh-tw
2005年论文
@wuu
name
Expression of mutant human cys ...... mocystinuria in a mouse model.
@ast
Expression of mutant human cys ...... mocystinuria in a mouse model.
@en
Expression of mutant human cys ...... mocystinuria in a mouse model.
@nl
type
label
Expression of mutant human cys ...... mocystinuria in a mouse model.
@ast
Expression of mutant human cys ...... mocystinuria in a mouse model.
@en
Expression of mutant human cys ...... mocystinuria in a mouse model.
@nl
prefLabel
Expression of mutant human cys ...... mocystinuria in a mouse model.
@ast
Expression of mutant human cys ...... mocystinuria in a mouse model.
@en
Expression of mutant human cys ...... mocystinuria in a mouse model.
@nl
P2093
P2860
P356
P1476
Expression of mutant human cys ...... mocystinuria in a mouse model.
@en
P2093
Andres Klein-Szanto
Baiqing Tang
Liqun Wang
Warren D Kruger
Xulin Chen
P2860
P304
P356
10.1093/HMG/DDI224
P577
2005-06-22T00:00:00Z