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lysosomal storage diseases/албумин

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СтатииКлинични изследванияПатенти
Страница 1 от 18 резултата

α-Galactosidase delivery using 30Kc19-human serum albumin nanoparticles for effective treatment of Fabry disease.

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Fabry disease is a genetic lysosomal storage disease caused by deficiency of α-galactosidase, the enzyme-degrading neutral glycosphingolipid that is transported to lysosome. Glycosphingolipid accumulation by this disease causes multi-organ dysfunction and premature death of the patient. Currently,

Amiodarone hepatotoxicity with absent phospholipidosis and steatosis: a case report and review of amiodarone toxicity in various organs.

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We present the first description of amiodarone toxicity in the liver without phospholipidosis or steatosis. In doing so, we will review the various effects of amiodarone toxicity in various organs. The patient is a young adult who had cardiac reconstruction as a child for transposition of the great

Use of 3D Human Liver Organoids to Predict Drug-Induced Phospholipidosis.

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Drug-induced phospholipidosis (PL) is a storage disorder caused by the formation of phospholipid-drug complexes in lysosomes. Because of the diversity of PL between species, human cell-based assays have been used to predict drug-induced PL in humans. We established three-dimensional (3D) human liver

Intense pseudotransport of a cationic drug mediated by vacuolar ATPase: procainamide-induced autophagic cell vacuolization.

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Cationic drugs frequently exhibit large apparent volumes of distribution, consistent with various forms of cellular sequestration. The contributions of organelles and metabolic processes that may mimic drug transport were defined in human vascular smooth muscle cells. We hypothesized that

Induction of lysosomal storage by suramin.

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Isolated livers of rats injected with saline or with suramin (250 mg per kg body weight) 24h previously were perfused with a medium containing radioactively labeled formaldehyde-treated albumin. Suramin-loaded livers released breakdown products at a much lower rate than controls and contained about

Enzyme therapy in Gaucher disease type 1: effect of neutralizing antibodies to acid beta-glucosidase.

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Gaucher disease type 1, a non-neuronopathic lysosomal storage disease, is caused by mutations at the acid beta-glucosidase locus. Periodic infusions of macrophage-targeted acid beta-glucosidase reverse hepatosplenomegaly, hematologic, and bony findings in many patients. Two patients receiving enzyme

Azithromycin, a lysosomotropic antibiotic, impairs fluid-phase pinocytosis in cultured fibroblasts.

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The dicationic macrolide antibiotic azithromycin inhibits the uptake of horseradish peroxidase (HRP) by fluid-phase pinocytosis in fibroblasts in a time- and concentration-dependent fashion without affecting its decay (regurgitation and/or degradation). The azithromycin effect is additive to that of

Effect of chlorphentermine pretreatment on chlorphentermine uptake by isolated perfused rat lung.

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Chlorphentermine (CP), an anorectic agent currently in use, is known to be highly accumulated in the lung, causes pulmonary phospholipidosis, and has been suspected of causing pulmonary hypertension. These studies were undertaken to characterize the uptake and accumulation processes and to examine

An in situ study of beta-glucosidase activity in normal and Gaucher fibroblasts with fluorogenic probes.

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Beta-glucosidase activity was evaluated in situ by means of fluorogenic probes in normal human fibroblasts and fibroblasts from homozygous carriers of the Gaucher trait. Probe internalization, targeting to lysosomes and post-cleavage probe retention were the primary concerns. Internalization and

[Decrease in the rats of intraliposomal proteolysis and labilization of rat liver lysosomes following suramin administration].

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Suramin treatment (250 mg/kg bw) 24 and 48 h after administration is followed by the decreased rate of intralysosomal digestion of 14C-bovine albumin. Inhibition of proteolysis and lysosomal overloading with suramin cause the solubilization of acid hydrolases--beta-galactosidase, acid RNase,

Role of thiols in degradation of proteins by cathepsins.

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The effects of thiols on the breakdown of 125I-labelled insulin, albumin and formaldehyde-treated albumin by highly purified rat liver cathepsins B, D, H and L at pH 4.0 and 5.5 were studied. At both pH values degradation was strongly activated by the thiols cysteamine, cysteine, dithiothreitol,

Delivery of fungal beta-galactosidase to rat brain by means of liposomes.

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A significant increase in beta-galactosidase activity was observed in the brain of rats 1 hr after an intravenous injection of liposomes containing beta-galactosidase purified from Aspergillus oryzae. The increased activity was proved to have features of the fungal enzyme by differentiating it from

Alpha Adrenergic Induction of Transport of Lysosomal Enzyme across the Blood-Brain Barrier.

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The impermeability of the adult blood-brain barrier (BBB) to lysosomal enzymes impedes the ability to treat the central nervous system manifestations of lysosomal storage diseases. Here, we found that simultaneous stimulation of the alpha1 and alpha2 adrenoreceptor restores in adult mice the high

Pulmonary responses to amiodarone in hamsters: comparison of intratracheal and oral administrations.

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Amiodarone (AD) has been shown to produce a transient pulmonary fibrosis in hamsters after intratracheal (i.t.) instillation. The goal of this study was to examine bronchoalveolar lavage (BAL) parameters during the development of fibrosis after i.t. AD in hamsters and to examine the responses to

Protein corona modulation of hepatocyte uptake and molecular mechanisms of gold nanoparticle toxicity.

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Protein corona formation over gold nanoparticles (AuNP) can modulate cellular responses by altering AuNP physicochemical properties. The liver plays an essential role in metabolism, detoxification and elimination of xenobiotics and drugs as well as circulating NP clearance. We investigated human
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