mucopolysaccharidoses/albumin

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[Mass-screening for acid mucopolysaccharidosis using urine samples by modified HCl-albumin turbidity method].

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To establish a method for screening acid mucopolysaccharidosis (AMPS), the HCl-albumin turbidity method was reexamined and modified for semiquantitative analysis. Mucopolysaccharide samples and HCl-albumin in buffer solution were incubated at 37 degrees C for 30 minutes, and the optical density at

Albumin transfusion in non-immune fetal hydrops: Doppler ultrasound evaluation of the acute effects on blood circulation in the fetal aorta and the umbilical arteries.

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A case of non-immune fetal hydrops, diagnosed as mucopolysaccharidosis VII with hypoalbuminemia, was treated in utero with albumin transfusions via cordocentesis on five occasions. Blood samples were taken for analysis of full blood count and blood gases before and after the transfusions. Pulsed

Limited transgene immune response and long-term expression of human alpha-L-iduronidase in young adult mice with mucopolysaccharidosis type I by liver-directed gene therapy.

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Mucopolysaccharidosis type I (MPS I) due to deficient alpha-L-iduronidase (IDUA) activity results in the accumulation of glycosaminoglycans (GAGs) in many of the cells of affected patients. Stable gene replacement by in vivo administration of lentiviral vectors (LVs) has therapeutic potential for

Measuring urinary glycosaminoglycans in the presence of protein: an improved screening procedure for mucopolysaccharidoses based on dimethylmethylene blue.

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Earlier we described a simple and reliable screening procedure in urine for mucopolysaccharidoses based on the color reaction of glycosaminoglycans (GAGs) with dimethylmethylene blue. At physiological concentrations of urinary protein, we observed an obvious interference by protein in the assay. By

A Highly Efficacious PS Gene Editing System Corrects Metabolic and Neurological Complications of Mucopolysaccharidosis Type I.

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Our previous study delivered zinc finger nucleases to treat mice with mucopolysaccharidosis type I (MPS I), resulting in a phase I/II clinical trial (ClinicalTrials.gov: NCT02702115). However, in the clinical trial, the efficacy needs to be improved due to the low transgene expression level. To this

Extracellular mammalian polysaccharides: glycosaminoglycans and proteoglycans.

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This review of the mammalian extracellular matrix polysaccharides covered the glycosaminoglycans (GAGs) and their association into proteoglycans. As they necessarily pertain to the chromatographic and electrophoretic separations of these molecules, the structural features of the five principal GAGs

Mannose 6-phosphate receptor-mediated transport of sulfamidase across the blood-brain barrier in the newborn mouse.

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Mucopolysaccharidosis type IIIA (MPS IIIA), which is a lysosomal storage disorder (LSD) caused by inherited deficiency of sulfamidase, is characterized by severe, progressive central nervous system (CNS) dysfunction. Enzyme replacement therapy (ERT) to treat CNS storage is challenging, because the

Genetically corrected autologous stem cells engraft, but host immune responses limit their utility in canine alpha-L-iduronidase deficiency.

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Canine alpha-L-iduronidase (alpha-ID) deficiency, a model of the human storage disorder mucopolysaccharidosis type I (MPS I), is an ideal system in which to evaluate the clinical benefit of genetically corrected hematopoietic stem cells. We performed adoptive transfer of genetically corrected

Human progenitor cells with high aldehyde dehydrogenase activity efficiently engraft into damaged liver in a novel model.

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Human cord blood stem cells (hCBSCs) have been reported to generate hepatocyte-like cells and thus hold promise for repairing damaged liver. However, the frequency of hCBSC-derived hepatocytes varies tremendously between different studies, and it is still controversial as to whether hCBSC-derived

A spectrophotometric method for the determination of heparan sulfate.

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A simple and reliable spectrophotometric method for the determination of heparan sulfate is described. The method is based on the 1,9-dimethylmethylene blue assay for sulfated glycosaminoglycans. Addition of bovine serum albumin, together with a specific NaCl concentration and pH, results in a

Dose-Dependent Prevention of Metabolic and Neurologic Disease in Murine MPS II by ZFN-Mediated In Vivo Genome Editing.

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Mucopolysaccharidosis type II (MPS II) is an X-linked recessive lysosomal disorder caused by deficiency of iduronate 2-sulfatase (IDS), leading to accumulation of glycosaminoglycans (GAGs) in tissues of affected individuals, progressive disease, and shortened lifespan. Currently available enzyme

Targeted Polymeric Nanoparticles for Brain Delivery of High Molecular Weight Molecules in Lysosomal Storage Disorders.

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Lysosomal Storage Disorders (LSDs) are a group of metabolic syndromes, each one due to the deficit of one lysosomal enzyme. Many LSDs affect most of the organ systems and overall about 75% of the patients present neurological impairment. Enzyme Replacement Therapy, although determining some systemic

Epinephrine enhances lysosomal enzyme delivery across the blood brain barrier by up-regulation of the mannose 6-phosphate receptor.

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Delivering therapeutic levels of lysosomal enzymes across the blood-brain barrier (BBB) has been a pivotal issue in treating CNS storage diseases, including the mucopolysaccharidoses. An inherited deficiency of beta-glucuronidase (GUS) causes mucopolysaccharidosis type VII that is characterized by

ZFN-Mediated In Vivo Genome Editing Corrects Murine Hurler Syndrome.

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Mucopolysaccharidosis type I (MPS I) is a severe disease due to deficiency of the lysosomal hydrolase α-L-iduronidase (IDUA) and the subsequent accumulation of the glycosaminoglycans (GAG), leading to progressive, systemic disease and a shortened lifespan. Current treatment options consist of

Decellularized liver matrix as a carrier for the transplantation of human fetal and primary hepatocytes in mice.

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The transplantation of primary hepatocytes has been shown to augment the function of damaged livers and to bridge patients to liver transplantation. However, primary hepatocytes often have low levels of engraftment and survive for only a short time after transplantation. To explore the potential

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