Aging accentuates and bone marrow transplantation ameliorates metabolic defects in Fabry disease mice. - Wikidata - awgldk - TriplyDB

Aging accentuates and bone marrow transplantation ameliorates metabolic defects in Fabry disease mice.

Aging accentuates and bone marrow transplantation ameliorates metabolic defects in Fabry disease mice.

http://www.wikidata.org/entity/Q37210520

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Local and global cerebral blood flow and glucose utilization in the alpha-galactosidase A knockout mouse model of Fabry disease Long-term enzyme correction and lipid reduction in multiple organs of primary and secondary transplanted Fabry mice receiving transduced bone marrow cells Substrate reduction augments the efficacy of enzyme therapy in a mouse model of Fabry disease The development and use of small molecule inhibitors of glycosphingolipid metabolism for lysosomal storage diseases.A symptomatic Fabry disease mouse model generated by inducing globotriaosylceramide synthesis.Depletion of globosides and isoglobosides fully reverts the morphologic phenotype of Fabry disease Adeno-associated viral vector-mediated gene transfer results in long-term enzymatic and functional correction in multiple organs of Fabry mice.Increased globotriaosylceramide levels in a transgenic mouse expressing human alpha1,4-galactosyltransferase and a mouse model for treating Fabry disease.Migalastat HCl reduces globotriaosylsphingosine (lyso-Gb3) in Fabry transgenic mice and in the plasma of Fabry patients.Fabry disease: recent advances in enzyme replacement therapy.Sex differences of urinary and kidney globotriaosylceramide and lyso-globotriaosylceramide in Fabry mice.Efficient uptake of recombinant α-galactosidase A produced with a gene-manipulated yeast by Fabry mice kidneys Cellular and tissue distribution of intravenously administered agalsidase alfa.Genomic abnormalities of the murine model of Fabry disease after disease-related perturbation, a systems biology approach.Distributions of Globotriaosylceramide Isoforms, and Globotriaosylsphingosine and Its Analogues in an α-Galactosidase A Knockout Mouse, a Model of Fabry Disease.Lentivector transduction improves outcomes over transplantation of human HSCs alone in NOD/SCID/Fabry mice.Sphingolipids and membrane biology as determined from genetic models.Use of a modified alpha-N-acetylgalactosaminidase in the development of enzyme replacement therapy for Fabry disease.Spontaneous Accumulation of Globotriaosylceramide (Gb3) in Proximal Renal Tubules in an ICR Mouse Glycosphingolipid storage in Fabry mice extends beyond globotriaosylceramide and is affected by ABCB1 depletion.Bioluminescent imaging of a marking transgene and correction of Fabry mice by neonatal injection of recombinant lentiviral vectors.Fabry disease: case report with emphasis on enzyme replacement therapy and possible future therapeutic options.Changes in Ionic Conductance Signature of Nociceptive Neurons Underlying Fabry Disease Phenotype.Minimum requirement of donor cells to reduce the glycolipid storage following bone marrow transplantation in a murine model of Fabry disease.Fabry disease: renal sphingolipid distribution in the α-Gal A knockout mouse model by mass spectrometric and immunohistochemical imaging.Neuropathic pain in a Fabry disease rat model.Medullary thick ascending limb impairment in the GlatmTg(CAG-A4GALT) Fabry model mice.Fabry disease

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Aging accentuates and bone marrow transplantation ameliorates metabolic defects in Fabry disease mice.

http://www.wikidata.org/entity/Q37210520

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article científic

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bilimsel makale

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scientific article published on May 1999

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vetenskaplig artikel

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name

Aging accentuates and bone mar ...... defects in Fabry disease mice.

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Aging accentuates and bone mar ...... defects in Fabry disease mice.

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Aging accentuates and bone mar ...... defects in Fabry disease mice.

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Aging accentuates and bone mar ...... defects in Fabry disease mice.

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Aging accentuates and bone mar ...... defects in Fabry disease mice.

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Aging accentuates and bone mar ...... defects in Fabry disease mice.

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PNAS.96.11.6423

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Proceedings of the National Academy of Sciences of the United States of America

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Aging accentuates and bone mar ...... defects in Fabry disease mice.

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Brady RO

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Chan CC

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Kopp J

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Kulkarni AB

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Murray GJ

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Ohshima T

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Quirk JM

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Schiffmann R

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Stahl S

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Tao-Cheng JH

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P2860

Protein measurement with the Folin phenol reagent

alpha-Galactosidase A deficient mice: a model of Fabry disease

Endocytosis of lysosomal alpha-galactosidase A by cultured fibroblasts from patients with Fabry disease.

State of the art review. Bone marrow transplantation treatment for storage diseases. Keystone. January 23, 1992.

Treatment of genetic defects in hematopoietic cell function by gene transfer.

Correction in trans for Fabry disease: expression, secretion and uptake of alpha-galactosidase A in patient-derived cells driven by a high-titer recombinant retroviral vector.

Gene therapy for metabolic disorders.

Quantitative analysis of plasma neutral glycosphingolipids by high performance liquid chromatography of their perbenzoyl derivatives.

Biochemical and clinical analysis of accumulated glycolipids in symptomatic heterozygotes of angiokeratoma corporis diffusum (Fabry's disease) in comparison with hemizygotes.

Transfer of the human glucocerebrosidase gene into hematopoietic stem cells of nonablated recipients: successful engraftment and long-term expression of the transgene.

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6423-6427

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