tyrosinemias/glutationa

O link é salvo na área de transferência

ArtigosTestes clínicosPatentes

Escolha o tipo de classificação

Página 1 a partir de 20 resultados

Biochemical studies of a patient with hereditary hepatorenal tyrosinemia: evidence of glutathione deficiency.

Apenas usuários registrados podem traduzir artigos

Entrar Inscrever-se

Metabolic and enzymatic studies in a patient with hereditary tyrosinemia demonstrated for the first time a deficiency of erythrocyte and hepatic glutathione. Markedly decreased hepatic fumarylacetoacetate hydrolase activity was demonstrated in this patient. The activities of hepatic enzymes not

Rescue from neonatal death in the murine model of hereditary tyrosinemia by glutathione monoethylester and vitamin C treatment.

Apenas usuários registrados podem traduzir artigos

Entrar Inscrever-se

Hereditary tyrosinemia type 1 (HT1) is a recessive disease caused by a deficiency of the enzyme fumarylacetoacetate hydrolase (FAH) that catalyzes the conversion of fumarylacetoacetate (FAA) into fumarate and acetoacetate. In mice models of HT1, FAH deficiency causes death within the first 24h after

Perturbation of maleylacetoacetic acid metabolism in rats with dichloroacetic Acid-induced glutathione transferase zeta deficiency.

Apenas usuários registrados podem traduzir artigos

Entrar Inscrever-se

Glutathione transferase zeta (GSTZ1-1) catalyzes the isomerization of maleylacetoacetate (MAA) to fumarylacetoacetate, the penultimate step in the tyrosine degradation pathway. GSTZ1-1 is inactivated by dichloroacetic acid (DCA), which is used for the clinical management of congenital lactic

Alkylation and inactivation of human glutathione transferase zeta (hGSTZ1-1) by maleylacetone and fumarylacetone.

Apenas usuários registrados podem traduzir artigos

Entrar Inscrever-se

Glutathione transferase zeta (GSTZ1-1) catalyzes the cis-trans isomerization of maleylacetoacetate or maleylacetone (MA) to fumarylacetoacetate or fumarylacetone (FA), respectively. GSTZ1-1 also catalyzes the glutathione-dependent biotransformation of a range of alpha-haloacids, including

Hereditary tyrosinemias (type I): a new vista on tyrosine toxicity and cancer.

Apenas usuários registrados podem traduzir artigos

Entrar Inscrever-se

Review of the literature of the past 40 years on tyrosine and its toxicity shows that no direct link between this aromatic amino acid and carcinogenesis has been well established. Ten years ago, studies of tyrosine toxicity in mice suggested the formation of an epoxide adduction product presumably

Crystal structure of maleylacetoacetate isomerase/glutathione transferase zeta reveals the molecular basis for its remarkable catalytic promiscuity.

Apenas usuários registrados podem traduzir artigos

Entrar Inscrever-se

Maleylacetoacetate isomerase (MAAI), a key enzyme in the metabolic degradation of phenylalanine and tyrosine, catalyzes the glutathione-dependent isomerization of maleylacetoacetate to fumarylacetoacetate. Deficiencies in enzymes along the degradation pathway lead to serious diseases including

Point mutation instability (PIN) mutator phenotype as model for true back mutations seen in hereditary tyrosinemia type 1 - a hypothesis.

Apenas usuários registrados podem traduzir artigos

Entrar Inscrever-se

Hereditary tyrosinemia type 1 (HT1) is an autosomal recessive disorder affecting fumarylacetoacetate hydrolase (FAH), the last enzyme in the tyrosine catabolism pathway. The liver mosaicism observed in HT1 patients is due to the reversion to the wild type of one allele of the original point mutation

Tyrosine administration decreases glutathione and stimulates lipid and protein oxidation in rat cerebral cortex.

Apenas usuários registrados podem traduzir artigos

Entrar Inscrever-se

Tyrosine levels are abnormally elevated in tissues and physiological fluids of patients with inborn errors of tyrosine catabolism especially in tyrosinemia type II which is caused by deficiency of tyrosine aminotransferase (TAT) and provokes eyes, skin and central nervous system disturbances. We

Hereditary tyrosinemia. Formation of succinylacetone-amino acid adducts.

Apenas usuários registrados podem traduzir artigos

Entrar Inscrever-se

Succinylacetone (SA) (4,6-dioxoheptanoic acid) is an abnormal metabolite produced in patients with hereditary tyrosinemia as a consequence of an inherited deficiency of fumaryl acetoacetate hydrolase activity. Patients with this disease are associated with a number of abnormalities, including

The mutagenicity of the tyrosine metabolite, fumarylacetoacetate, is enhanced by glutathione depletion.

Apenas usuários registrados podem traduzir artigos

Entrar Inscrever-se

The toxicity of tyrosine metabolites has been suggested, but not proven, to play a role in the ethiopathogenesis of hepatic alterations observed in hereditary tyrosinemia type I (HT I), a metabolic disease caused by a deficiency of the last enzyme in the tyrosine catabolic pathway,

Fumarylacetoacetate, the metabolite accumulating in hereditary tyrosinemia, activates the ERK pathway and induces mitotic abnormalities and genomic instability.

Apenas usuários registrados podem traduzir artigos

Entrar Inscrever-se

Patients suffering from the metabolic disease hereditary tyrosinemia type I (HT1), caused by fumarylacetoacetate hydrolase deficiency, have a high risk of developing liver cancer. We report that a sub-apoptogenic dose of fumarylacetoacetate (FAA), the mutagenic metabolite accumulating in HT1,

Activation of nuclear factor E2-related factor 2 in hereditary tyrosinemia type 1 and its role in survival and tumor development.

Apenas usuários registrados podem traduzir artigos

Entrar Inscrever-se

In tyrosinemia type 1 (HT1), accumulation of toxic metabolites results in oxidative stress and DNA damage, leading to a high incidence of hepatocellular carcinomas. Nuclear factor erythroid-2 related factor 2 (Nrf2) is a key transcription factor important for cellular protection against oxidative

Evidence for the possible formation of a toxic tyrosine metabolite by the liver microsomal drug metabolizing system.

Apenas usuários registrados podem traduzir artigos

Entrar Inscrever-se

The toxicity of tyrosine in the mouse was found to be affected by agents which interact with the liver microsomal drug metabolizing system. Pretreatment of mice with SKF 525A or cobaltous chloride, two different types of inhibitors of the drug metabolizing system, afforded a marked protection

Cyclin B-dependent kinase and caspase-1 activation precedes mitochondrial dysfunction in fumarylacetoacetate-induced apoptosis.

Apenas usuários registrados podem traduzir artigos

Entrar Inscrever-se

Hereditary tyrosinemia type I is the most severe metabolic disease of the tyrosine catabolic pathway mainly affecting the liver. It is caused by deficiency of fumarylacetoacetate hydrolase, which prevents degradation of the toxic metabolite fumarylacetoacetate (FAA). We report here that FAA induces

TRAP-seq identifies cystine/glutamate antiporter as a driver of recovery from liver injury.

Apenas usuários registrados podem traduzir artigos

Entrar Inscrever-se

Understanding the molecular basis of the regenerative response following hepatic injury holds promise for improved treatment of liver diseases. Here, we report an innovative method to profile gene expression specifically in the hepatocytes that regenerate the liver following toxic injury. We used

Junte-se à nossa
página do facebook

Herbal & Natural Medicine

O mais completo banco de dados de ervas medicinais apoiado pela ciência

Funciona em 55 idiomas
Curas herbais apoiadas pela ciência
Reconhecimento de ervas por imagem
Mapa GPS interativo - marcar ervas no local (em breve)
Leia publicações científicas relacionadas à sua pesquisa
Pesquise ervas medicinais por seus efeitos
Organize seus interesses e mantenha-se atualizado com as notícias de pesquisa, testes clínicos e patentes

Digite um sintoma ou doença e leia sobre ervas que podem ajudar, digite uma erva e veja as doenças e sintomas contra os quais ela é usada.
* Todas as informações são baseadas em pesquisas científicas publicadas