Renal Accumulation of Glycosphingolipids

Miyasaki, Kichihei

doi:10.1159/000180480

Cited by 2 publications

(1 citation statement)

References 5 publications

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“…More importantly, a second autopsy case reported accumulation of GL-3 in gotes. These cases and others [19,[35][36][37][38][39] suggest that residual enzyme activity, while perhaps not eliminating both capillary endothelial cells and tubular epithelial cells in a renal allograft 14 years after transplant [27]. Urine is all signs of clinical or histologic disease, is still able to significantly extend the patient's lifespan by obviating apy.…”

Section: Post-treatment Biopsiesmentioning

confidence: 86%

Globotriaosylceramide accumulation in the Fabry kidney is cleared from multiple cell types after enzyme replacement therapy

Thurberg¹,

Rennke²,

Colvin³

et al. 2002

Kidney International

308

250

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Globotriaosylceramide accumulation in the Fabry kidney is ance was more limited than that observed in other cell types. cleared from multiple cell types after enzyme replacement therapy.No evidence of immune complex disease was found by immuno-Background. Fabry disease, a lysosomal storage disease caused fluorescence despite circulating anti-r-h␣GalA IgG antibodies. by deficient lysosomal ␣-galactosidase A activity, is character-Conclusions. These findings indicate a striking reversal of ized by globotriaosylceramide (GL-3) accumulation in multiple renal glycosphingolipid accumulation in the vasculature and in cell types, particularly the vasculature, leading to end organ other renal cell types, and suggest that long-term treatment failure. Accumulation in the kidney is responsible for progreswith r-h␣GalA may halt the progression of pathology and sive decline in renal function in male patients with the classical prevent renal failure in patients with Fabry disease. phenotype, resulting in renal failure in their third to fifth decades of life. With the advent of recombinant protein synthesis technology, enzyme replacement therapy has become a viable alternative to dialysis or renal transplantation, previously the Fabry disease is an X-linked recessive disorder in which only available treatment options for end-stage renal disease.affected males are deficient in the lysosomal enzyme Methods. The pre-and post-treatment renal biopsies were ␣-galactosidase A. This deficiency leads to accumulation analyzed from fifty-eight Fabry patients enrolled in a Phase 3 double-blind, randomized, placebo-controlled trial followed by

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Section: Post-treatment Biopsiesmentioning

confidence: 86%

Globotriaosylceramide accumulation in the Fabry kidney is cleared from multiple cell types after enzyme replacement therapy

Thurberg¹,

Rennke²,

Colvin³

et al. 2002

Kidney International

308

250

View full text Add to dashboard Cite

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Experimental polymer storage disease in rabbits

Miyasaki

1975

Virchows Arch. A Path. Anat. and Histol.

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Water-soluble polymer compound, polyvinyl alcohol(PVA), and water-insoluble polymer compounds, polyvinyl acetate (PVAc) and polystylol (PS), were administered in 297 rabbits. When high polymerized PVA, PVAc or PS were continuously injected intravenously for a long period of time, lesions resembled to those of Gaucher and Niemann-Pick diseases were developed. From these experimental results, pathological development of various sphingolipidoses found in the human body was discussed and pathological findings were analysed polymer-chemically from the chemical properties of the substances stored.

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Renal Accumulation of Glycosphingolipids

Cited by 2 publications

References 5 publications

Globotriaosylceramide accumulation in the Fabry kidney is cleared from multiple cell types after enzyme replacement therapy

Globotriaosylceramide accumulation in the Fabry kidney is cleared from multiple cell types after enzyme replacement therapy

Experimental polymer storage disease in rabbits

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