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Description
  • Biochemistry of the α-galactosidase A deficiency leading to lysosomal accumulation of neutral glycosphingolipids with terminal α-galactosyl moieties is described. The possible role of storage substrates in the pathogenesis of this disease is discussed.
  • Biochemistry of the α-galactosidase A deficiency leading to lysosomal accumulation of neutral glycosphingolipids with terminal α-galactosyl moieties is described. The possible role of storage substrates in the pathogenesis of this disease is discussed. (en)
Title
  • Biochemistry of Fabry Disease
  • Biochemistry of Fabry Disease (en)
skos:prefLabel
  • Biochemistry of Fabry Disease
  • Biochemistry of Fabry Disease (en)
skos:notation
  • RIV/00064165:_____/10:6935!RIV11-MZ0-00064165
http://linked.open...avai/riv/aktivita
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  • I, Z(MSM0021620806)
http://linked.open...vai/riv/dodaniDat
http://linked.open...aciTvurceVysledku
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  • 248832
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  • RIV/00064165:_____/10:6935
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  • Fabry disease; α-galactosidase A; Globotriaosylceramide; Glycosphingolipids; Saposin B (en)
http://linked.open.../riv/klicoveSlovo
http://linked.open...ontrolniKodProRIV
  • [49ED86EF4556]
http://linked.open...i/riv/mistoVydani
  • Heidelberg
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  • Fabry Disease
http://linked.open...in/vavai/riv/obor
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  • Hřebíček, Martin
  • Ledvinová, Jana
http://linked.open...n/vavai/riv/zamer
number of pages
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  • Springer-Verlag
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  • 978-90-481-9032-4
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