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Transcriptional profiling of a mouse model for Lafora disease reveals dysregulation of genes involved in the expression and modification of proteins

✍ Scribed by Subramaniam Ganesh; Naomi Tsurutani; Kenji Amano; Shuchi Mittal; Chiharu Uchikawa; Antonio V. Delgado-Escueta; Kazuhiro Yamakawa

Book ID
116767986
Publisher
Elsevier Science
Year
2005
Tongue
English
Weight
120 KB
Volume
387
Category
Article
ISSN
0304-3940

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## Abstract Sly disease (MPS VII) is an autosomal‐recessive lysosomal storage disorder resulting from beta‐glucuronidase deficiency, which is characterized by a severe neurological impairment. MPS VII mice accumulate undegraded glycosaminoglycans and mimic the human neurodegenerative disorder, thus