Can I read Generalized accumulation of neutral glycosphingolipids with GM2 ganglioside accumulation in the brain on EtoBox?

Generalized accumulation of neutral glycosphingolipids with GM2 ganglioside accumulation in the brain by Paul D. Snyder; William Krivit; Charles C. Sweeley is a Medicine article available to read on EtoBox.

What is Generalized accumulation of neutral glycosphingolipids with GM2 ganglioside accumulation in the brain about?

Analyses have been made of glycosphingolipids from visceral organs and brain of a patient with an unusual lipid storage disorder diagnosed initially as classical Tay-Sachs disease. Levels of the lipids from fresh-frozen sections of gray and white matter, kidney, spleen, liver, and heart from this patient were compared with those of normal juvenile controls, and the fatty acid composition of accumulated glycosphingolipids was compared with reference compounds. This patient was found to have abnormally high concentrations of a globoside in liver, kidney, and spleen, asialo Gnlz ganglioside in brain and liver, and GY2 ganglioside in the brain. On the basis of these findings along with the clinical manifestations of Tay-Sachs disease with visceral involvement (hepatosplenomegaly) and demonstration of total deficiency of both A and B components of b-N-acetylhexosaminidase activity, this glycosphingolipidosis is the same as two previously reported cases of GY2 gangliosidosis with globoside accumulation and total b-N-ace tylhexosaminidase deficiency. Supplementary key words globoside . Gnfz ganglioside . asialo GMz . glycosphingolipid storage disease . total hexosaminidase deficiency AN I

Who reads Generalized accumulation of neutral glycosphingolipids with GM2 ganglioside accumulation in the brain?

Author

Paul D. Snyder; William Krivit; Charles C. Sweeley

Publisher

Elsevier BV

Published

1972

Language

EN

Field

Medicine (Health Sciences)