Morquio's syndrome is a genetic disorder caused by the lack of the enzyme responsible for the breakdown of the mucopolysaccharide
This can generate UDP sugars used for mucopolysaccharide
The demonstration of the individual mucopolysaccharides
in human aortas, coronary arteries and cerebral arteries.
It is a heterogenous mix of mucopolysaccharides
with molecular weight varying from 3 000 to 30 000 Da which bind to and enhance the effect of the natural anticoagulant antithrombin III (AT).
8,9) However, subsequent studies also show that the atypical keratinocytes in these tumors stained positive with periodic acid-Schiff, and that the positivity was removed after diastase digestion; this was consistent with the neoplastic cells containing glycogen and no neutral mucopolysaccharides
Urine organic acids, amino acids, mucopolysaccharides
, and oligosaccharides were all within reference values.
It is essential for cutting up the mucopolysaccharides
- long chains of sugar molecules used in the building of connective tissues in the body - called dermatan and heparan sulphate.
It results from the body's inability to make lysosomal alpha-L-iduronate, an enzyme that helps break down mucopolysaccharides
- sugar molecules used to build tissue - and can cause deafness, joint disease and abnormal bones
In patients affected by MPS I, these mucopolysaccharides
accumulate in organs and tissues of the body, particularly in the central nervous system, liver, spleen, heart and skeleton, ultimately leading to cell death and progressive tissue and organ damage.
Ganglia contain a gelatinous fluid that is highly viscous secondary to the presence of hyaluronic acid and other mucopolysaccharides
Meconium represents the intestinal contents of the fetus and is a complex matrix, consisting mainly of water but also containing mucopolysaccharides
, lipids, proteins, bile acids and salts, epithelial cells, cholesterol and sterol precursors, blood-group substances, squamous cells, residual amniotic fluid, and enzymes (25).
are long chains of sugar molecules used to build connective tissue in the body.