Re: Hurler's syndrome
Mucopolysaccharidosis type I is an inherited disease that belongs to a group
of diseases called mucopolysaccharidoses. The storage of abnormal quantities
of mucopolysaccharides in different body tissues is responsible for the
symptoms and appearance of the disease. Hurler syndrome is inherited as an
autosomal recessive trait. The metabolic defect that causes Hurler syndrome
is the body's inability to make an enzyme called lysosomal a-L-iduronidase.
0.5% toluidine blue in 25% acetone has been used on alcohol and acetone fixed
cultures and frozen sections for metachromatic staining of the acid
mucopolysaccharide containing cells (histiocytes and hepatocytes) found in
Hurler's syndrome. Slides are stained for 2-4 minutes, then dehydrated in
acetone, clearing in xylene and mounted in an synthetic mountant.
In other forms of mucopolysaccharidosis, glycosaminoglycans (GAG) are
deposited in connective tissues and can be demonstrated in non-paraffin
techniques. The tissue must be fixed in alcohol, acetone or tetrahydrofuron
and stained with a saturated azure A in absolute alcohol and then transfered
to a saturated azure A in 70% alcohol for 10 minutes each. Slides are then
dehydrated through absolute alcohols and mounted in a synthetic mountant. The
GAG become reddish purple against a blue background.
Dorling, J.: Localisation of sulphated glycosaminoglycans by a simple
technique using cryostat sections. Journal of Clinical Pathology, 33,
Eric C. Kellar
University of Pittsburgh Medical Center
What is the best stain to identify the build up of mucopolysaccharide
(glycosaminoglycans) in cultures of cells from Hurler's syndrome?
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