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, 
897-898, 1980.

Eric C. Kellar
University of Pittsburgh Medical Center

Dear All

What is the best stain to identify the build up of mucopolysaccharide
(glycosaminoglycans) in cultures of cells from Hurler's syndrome?

Mike Bromley 
Histology Department
Paterson Institute
Wilmslow Road
M20 9BX

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