Catalan
Albanian
Arabic
Armenian
Azerbaijani
Belarusian
Bengali
Bosnian
Catalan
Czech
Danish
Deutsch
Dutch
English
Estonian
Finnish
Français
Greek
Haitian Creole
Hebrew
Hindi
Hungarian
Icelandic
Indonesian
Irish
Italian
Japanese
Korean
Latvian
Lithuanian
Macedonian
Mongolian
Norwegian
Persian
Polish
Portuguese
Romanian
Russian
Serbian
Slovak
Slovenian
Spanish
Swahili
Swedish
Turkish
Ukrainian
Vietnamese
Български
中文(简体)
中文(繁體)
homocystinuria/glutatió
L'enllaç es desa al porta-retalls
Pàgina 1 des de 34 resultats
Effect of cysteine dosage on erythrocyte glutathione synthesis rate in a patient with cystathionine beta synthase deficiency.
Només els usuaris registrats poden traduir articles
Inicieu sessió / registreu-vos
Cystathionine β-synthase (CBS)-deficient patients develop premature arteriosclerosis and thrombosis leading to a high risk of a vascular event before the age of 30 years. In CBS deficiency the transsulfuration pathway is impaired, leading to markedly elevated levels of homocysteine and methionine,
Homocysteine and cysteine - albumin binding in homocystinuria: assessment of cysteine status and implications for glutathione synthesis?
Només els usuaris registrats poden traduir articles
Inicieu sessió / registreu-vos
Measurement of plasma total cysteine rather than free dimeric cystine gives a better indication of cysteine status in homocystinuric patients. This is the result of displacement of cysteine from albumin by homocysteine and is related to the plasma homocysteine concentration. In control subjects the
Taurine alleviates repression of betaine-homocysteine S-methyltransferase and significantly improves the efficacy of long-term betaine treatment in a mouse model of cystathionine β-synthase-deficient homocystinuria.
Només els usuaris registrats poden traduir articles
Inicieu sessió / registreu-vos
Classical cystathionine β-synthase-deficient homocystinuria (HCU) is a life-threatening inborn error of sulfur metabolism. Treatment for pyridoxine-nonresponsive HCU involves lowering homocysteine (Hcy) with a methionine (Met)-restricted diet and betaine supplementation. Betaine treatment efficacy
Homocystinuria due to cystathionine beta-synthase deficiency: novel biochemical findings and treatment efficacy.
Només els usuaris registrats poden traduir articles
Inicieu sessió / registreu-vos
To explore the pathogenesis of cystathionine beta-synthase (CBS) deficiency and to test the efficacy of pharmacological therapy we examined a panel of metabolites in nine homocystinuric patients under treated and/or untreated conditions. Off pharmacological treatment, the biochemical phenotype was
Taurine treatment prevents derangement of the hepatic γ-glutamyl cycle and methylglyoxal metabolism in a mouse model of classical homocystinuria: regulatory crosstalk between thiol and sulfinic acid metabolism.
Només els usuaris registrats poden traduir articles
Inicieu sessió / registreu-vos
Cystathionine β-synthase-deficient homocystinuria (HCU) is a poorly understood, life-threatening inborn error of sulfur metabolism. Analysis of hepatic glutathione (GSH) metabolism in a mouse model of HCU demonstrated significant depletion of cysteine, GSH, and GSH disulfide independent of the block
Genetic disorders of glutathione and sulfur amino-acid metabolism. New biochemical insights and therapeutic approaches.
Només els usuaris registrats poden traduir articles
Inicieu sessió / registreu-vos
Important insights have recently been derived from studies of inborn human defects of sulfur metabolism. Metabolic lesions responsible for homocystinuria have been elucidated, with possible implications for understanding atherogenesis in the general population. The cause of cystinosis remains
Cystathionine beta-synthase deficiency alters hepatic phospholipid and choline metabolism: Post-translational repression of phosphatidylethanolamine N-methyltransferase is a consequence rather than a cause of liver injury in homocystinuria.
Només els usuaris registrats poden traduir articles
Inicieu sessió / registreu-vos
Classical homocystinuria (HCU) due to inactivating mutation of cystathionine β-synthase (CBS) is a poorly understood life-threatening inborn error of sulfur metabolism. A previously described cbs-/- mouse model exhibits a semi-lethal phenotype due to neonatal liver failure. The transgenic HO mouse
Leptin concentrations and SCD-1 indices in classical homocystinuria: Evidence for the role of sulfur amino acids in the regulation of lipid metabolism.
Només els usuaris registrats poden traduir articles
Inicieu sessió / registreu-vos
BACKGROUND
We describe body composition, lipid metabolism and Stearoyl-CoA desaturase-1 (SCD-1) indices in patients with classical homocystinuria (HCU).
METHODS
Eleven treated HCU patients and 16 healthy controls were included. Body composition and bone mineral density were assessed by dual X-ray
Homocystinuria with congenital/developmental cataract.
Només els usuaris registrats poden traduir articles
Inicieu sessió / registreu-vos
The aim of the study is to screen patients for homocystinuria with and without cataract and analyse for homocystine and methionine. Fifty-eight samples from 29 patients, i.e., plasma and urine collected after overnight fasting were analysed by the screening test for homocystine, and paper
Accumulation of pyroglutamic acid (5-oxoproline) in homocystinuria.
Només els usuaris registrats poden traduir articles
Inicieu sessió / registreu-vos
A 12-year-old boy with homocystinuria due to a cystathionine synthetase defect was shown to accumulate large amounts of pyroglutamic acid (pyroglu). In four different urine samples the concentration ranged between 4.1 and 9.7 mol/mol of creatinine. The pyroglu concentration in plasma was 1.6 mmol/l,
Cystathionine beta synthase deficiency induces catalase-mediated hydrogen peroxide detoxification in mice liver.
Només els usuaris registrats poden traduir articles
Inicieu sessió / registreu-vos
Cystathionine beta synthase deficiency induces hyperhomocysteinemia which is considered as a risk factor for vascular diseases. Studies underlined the importance of altered cellular redox reactions in hyperhomocysteinemia-induced vascular pathologies. Nevertheless, hyperhomocysteinemia also induces
Glutathione metabolism in cobalamin deficiency type C (cblC).
Només els usuaris registrats poden traduir articles
Inicieu sessió / registreu-vos
BACKGROUND
Methylmalonic aciduria with homocystinuria, cblC defect, is the most frequent disorder of vitamin B12 metabolism. CblC patients are commonly treated with a multidrug therapy to reduce metabolite accumulation and to increase deficient substrates. However the long-term outcome is often
Cystathionine beta-synthase deficiency causes fat loss in mice.
Només els usuaris registrats poden traduir articles
Inicieu sessió / registreu-vos
Cystathionine beta synthase (CBS) is the rate-limiting enzyme responsible for the de novo synthesis of cysteine. Patients with CBS deficiency have greatly elevated plasma total homocysteine (tHcy), decreased levels of plasma total cysteine (tCys), and often a marfanoid appearance characterized by
Effect of cadmium administration in hyperhomocysteinemic mice due to cystathionine beta synthase deficiency.
Només els usuaris registrats poden traduir articles
Inicieu sessió / registreu-vos
Homocysteine, a sulfur-containing amino acid formed during the metabolism of methionine, is commonly slightly elevated in the plasma of the general population. Additionally, we previously found that cystathionine beta synthase-deficient mice, a murine model of hyperhomocysteinemia, exhibit altered
Analysis of Total Thiols in the Urine of a Cystathionine β-Synthase-Deficient Mouse Model of Homocystinuria Using Hydrophilic Interaction Chromatography.
Només els usuaris registrats poden traduir articles
Inicieu sessió / registreu-vos
Homocysteine and related thiols (cysteine, cysteinylglycine, and glutathione) in the urine of a cystathionine β-synthase (CBS)-deficient mouse model were quantified using hydrophilic interaction chromatography with fluorescence detection. Urine samples were incubated with tris(2-carboxyethyl)
La base de dades d’herbes medicinals més completa avalada per la ciència
- Funciona en 55 idiomes
- Cures a base d'herbes recolzades per la ciència
- Reconeixement d’herbes per imatge
- Mapa GPS interactiu: etiqueta les herbes a la ubicació (properament)
- Llegiu publicacions científiques relacionades amb la vostra cerca
- Cerqueu herbes medicinals pels seus efectes
- Organitzeu els vostres interessos i estigueu al dia de les novetats, els assajos clínics i les patents
Escriviu un símptoma o una malaltia i llegiu sobre herbes que us poden ajudar, escriviu una herba i vegeu malalties i símptomes contra els quals s’utilitza.
* Tota la informació es basa en investigacions científiques publicades
