Mucopolysaccharidose

The mucopolysaccharidoses ( MPS ) are degenerative genetic diseases related to an enzymatic deficit which is responsible for the accumulation of Mucopolysaccharide S in various fabrics. This accumulation results in the symptoms of a degenerative Maladie which touches more or less many bodies.

A very great clinical and biological heterogeneity characterizes these diseases. The same enzymatic deficit can produce forms as well severe as moderated, which explains the difficulty of their diagnosis. The forecast is variable according to the touched bodies but can usually include a death of the patient in a few years.

  • MPS-I H, Hurler' S syndrome,
  • MPS-I H/S, To howl/Scheie syndrome
  • MPS-I S, Scheie' S syndrome
  • MPS-II, Hunter syndrome, iduronate sulfatase deficiency
  • MPS-III has, Sanfillipo has syndrome, heparan- NR - sulfatase
  • MPS-III B, Sanfillipo B syndrome, NR - acetyl-α-glucosaminidase deficiency
  • MPS-III C. Sanfillipo C syndrome, α-glucoasminide NR - acetyltransferase deficiency
  • MPS-III D, Sanfillipo D syndrome, NR - acetylglucosamine-6-sulfate sulfatase deficiency
  • MPS-IV, Morquio syndrome , NR - Acetylgalactosamine=6-sulfate sulfase deficiency
  • MPS VI, Maroteaux-Lamy syndrome, arylsulfatase B deficiency
  • MPS VII, standard Mucopolysaccharidosis VII Sly syndrome, β-glucuronidase deficiency

    • (Note: MPS-V and MPS-VIII are No to skirt in uses ace designations for any disease.)-->

    See too

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    External bond

    • Association '' Vaincre the diseases lysosomales ''

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