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.
Urine dermatan sulphate
concentrations were elevated in both of them and the gross elevations of plasma beta-hexosaminidase, arylsulphatase A and alpha-mannosidase were consistent with a diagnosis of ML II/III.
Pathological glycosaminoglycans in different mucopolysaccharidosis Normal Mucopolysaccharidosis + I II III IV VI VII IX Chondroitin sulphate (+) + ++ Dermatan sulphate
++ ++ ++ n-+ Heparan sulphate + + + n-+ Keratan sulphate + Typical clinical findings, affected organ systems Chondroitin sulphate Dermatan sulphate
Skeleton + internal organs Heparan sulphate Intellectual disability Keratan sulphate Skeleton ++: Prominent feature, +: Often present, (+): Sometimes present Table II.
GAG family include unsulphated hyaluronic acid (HA), sulphated dermatan sulphate
(DS), keratin sulphate (KS), heparin sulphate (HS) and chondroitin sulphates (C4S, C6S).
Hurler's syndrome (MPS IH), which is a prototype of MPS is caused by a deficiency of alpha-L-iduronidase due to a loss of function mutation leading to excessive accumulation of partially degraded GAGs, dermatan sulphate
and heparan sulphate within the cells.
The major classes of GAG include hyaluronic acid (HA), heparin sulphate, heparin, chondroitin 4-sulphate, chondroitin 6-sulphate, dermatan sulphate
and keratan sulphate.[sup.9]
Heparin and dermatan sulphate
induced capacitation of frozen-thawed bull spermatozoa measured by merocyanine-540.
(3) The metabolism of heparan and dermatan sulphate is normal in Morquio syndrome, which is why patients with Morquio syndrome do not have mental retardation.
Heparan, keratan and dermatan sulphate can be distinguished by electrophoresis techniques to differentiate between the mucopolysaccharidoses.
"We also went on to monitor long-term clinical outcomes in patients with MPSI, MPSII and MPSVI after treatment to show that elevations of both this biomarker, and the dermatan sulphate
:chondroitin sulphate biomarker currently used in the diagnostic laboratory in Manchester, correlated with clinical treatment outcomes in patients.
"For example, dermatan sulphate
, which is extracted from cow hides, is reported to have anti-inflammatory properties and inhibit the formation of blood clots.
Determination of human urinary hyaluronic acid, chondroitin sulphate and dermatan sulphate
as their unsaturated disaccharides by high-performance liquid chromatography.