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Article

The pharmacological chaperone effect of N-octyl-beta-valienamine on human mutant acid beta-glucosidases.

Blood cells, molecules & diseases - 15 Jan 2010

Luan Zhuo, Li Linjing, Ninomiya Haruaki, Ohno Kousaku, Ogawa Seiichiro, Kubo Takatoshi, Iida Masami, Suzuki Yoshiyuki

Abstract excerpt

Gaucher's disease (GD), mainly caused by a defect of acid beta-glucosidase (beta-Glu), is the most common form of sphingolipidosis. We have previously shown that the carbohydrate mimic and inhibitor of beta-Glu, N-octyl-beta-valienamine (NOV), could increase the protein level and enzyme activity of various mutant beta-Glus in cultured GD fibroblasts and in COS cells, suggesting that NOV acts as a pharmacological...

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PMID: 19857976
DOI: 10.1016/j.bcmd.2009.10.003

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PubMed metadata and Literature Corpus relationships. Corpus snapshot updated 7 Sept 2026. Manifest publication-graph-cohort-2026-09-07.1.

Source receipt: pubmed, retrieved 7 Sept 2026.

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