Natural history study of glycan accumulation in large animal models of GM2 gangliosidoses.
β-hexosaminidase is an enzyme responsible for the degradation of gangliosides, glycans, and other glycoconjugates containing β-linked hexosamines that enter the lysosome. GM2 gangliosidoses, such as Tay-Sachs and Sandhoff, are lysosomal storage disorders characterized by β-hexosaminidase deficiency...
Main Authors: | Catlyn Cavender, Linley Mangini, Jeremy L Van Vleet, Carley Corado, Emma McCullagh, Heather L Gray-Edwards, Douglas R Martin, Brett E Crawford, Roger Lawrence |
---|---|
Format: | Article |
Language: | English |
Published: |
Public Library of Science (PLoS)
2020-01-01
|
Series: | PLoS ONE |
Online Access: | https://doi.org/10.1371/journal.pone.0243006 |
Similar Items
-
Characterization of glycan substrates accumulating in GM1 Gangliosidosis
by: Roger Lawrence, et al.
Published: (2019-12-01) -
Juvenile GM2 Gangliosidoses
by: J Gordon Millichap
Published: (2006-11-01) -
Novel Therapies and Biochemical Insights for the GM1 and GM2 Gangliosidoses
by: Arthur, Julian
Published: (2011) -
Thymic alterations in GM2 gangliosidoses model mice.
by: Seiichi Kanzaki, et al.
Published: (2010-01-01) -
GM2 Gangliosidoses: Clinical Features, Pathophysiological Aspects, and Current Therapies
by: Andrés Felipe Leal, et al.
Published: (2020-08-01)