TISSUE STORAGE OF MUCOPOLYSACCHARIDES IN HURLER-PFAUNDLER'S DISEASE

Mucopolysaccharides Produced in Tissue Culture

1. A method of mass tissue culture has been devised by which, in a relatively short period of time, samples large enough for chemical isolation of mucopolysaccharides can be obtained. 2. Chemical isolation of acid mucopolysaccharides from mass cultures of human fetal skin, human fetal bone, bovine fetal skin, and rat subcutaneous tissue has been carried out. It has been found that the fibroblas...

Glaucoma in a case of Hurler disease.

The electron microscopic appearances of the corneoscleral and iris tissue removed at operation from a child with Hurler disease and glaucoma showed distinctive swollen cells with intracellular inclusions similar to those which are observed in other tissues in these patients and which are due to abnormal lysosomal storages of mucopolysaccharides. Some recent observations on the possible relation...

Metabolism of the mucopolysaccharides of connective tissue.

The Biochemistry of Mucopolysaccharides of Connective Tissue

suitably curved surfaces. 3) Point projection whereby electrons are focussed to a fine spot on a thin target which then becomes a source of X-rays to project a magnified image of an object by rectilinear propagation, and 4) scanning methods whereby a fine electron beam probe is made to scan point-by-point the surface of the object itself or a thin metal target in close contact with the object. ...

Early disease progression of Hurler syndrome

BACKGROUND Newborn screening for mucopolysaccharidosis type I (MPS I) shows promise to improve outcomes by facilitating early diagnosis and treatment. However, diagnostic tests for MPS I are of limited value in predicting whether a child will develop severe central nervous system disease associated with Hurler syndrome, or minimal or no central nervous system involvement associated with the att...