Specifically, odiparcil acts on chondroitin sulfate ("CS") and dermatan
sulfate ("DS"), either or both of which accumulate in patients with MPS I, II, IVa, VI and VII.
It was shown that loss of sulfated proteoglycans and anionic regions in the basal membrane of the glomerulus and the mesangial mat rix caused excess accumulation of proteoglycans such as chondroitin sulphate and dermatan
sulphate in these areas.3 This causes a decrease in charge-dependent renal selectivity and an increase in the thickness of the basal membrane.
Odiparcil has also the potential to address other MPS types, characterized by the accumulation of chondroitin or dermatan
sulfate (MPS I or Hurler/Sheie syndrome, MPS II or Hunter syndrome, MPS IVa or Morquio syndrome and MPS VII or Sly syndrome).
Hastalik, iduronat-2-sulfataz enzim eksikligi nedeniyle yikilamayan dermatan
ve heparan sulfat gibi glukoazminoglikanlarin (GAG) solunum, sinir, dolasim ve kas-iskelet sistemlerinde birikmesi ile karakterizedir.
Epidural fibrosis is a natural response of the body to the impact of surgery, leading to marked reduction in the cellularity of the tissue and excessive deposition of the extracellular matrix components such as collagen, fibronectin, and dermatan
60 kDa) (Rodrigues et al., 2013), dermatan
sulfate (DS) and/or heparan sulfate (HS) (Salles et al., 2017).
MPS II is a genetic lysosomal storage disease caused by deficiency of the iduronate-2-sulfatase enzyme which is needed to break down or recycle glycosaminoglycans dermatan
sulfate and heparan sulfate.
Decorin (DCN) is one of the most common dermatan
sulphate proteoglycan (molecular protein polysaccharide) with small leucine rich proteins.
sulphate deposition in the MPS syndromes results cardiac valve problems.
sulphate concentrations were elevated in both of them and the gross elevations of plasma beta-hexosaminidase, arylsulphatase A and alphamannosidase were consistent with a diagnosis of ML II/III.