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Article

Enzyme enhancement activity of N-octyl-beta-valienamine on beta-glucosidase mutants associated with Gaucher disease.

Biochimica et biophysica acta - 1 May 2007

Lei Ke, Ninomiya Haruaki, Suzuki Michitaka, Inoue Takehiko, Sawa Miwa, Iida Masami, Ida Hiroyuki, Eto Yoshikatsu, Ogawa Seiichiro, Ohno Kousaku, Suzuki Yoshiyuki

Abstract excerpt

Gaucher disease (GD), caused by a defect of beta-glucosidase (beta-Glu), is the most common form of sphingolipidosis. We have previously shown that a carbohydrate mimic N-octyl-beta-valienamine (NOV), an inhibitor of beta-Glu, could increase the protein level and enzyme activity of F213I mutant beta-Glu in cultured GD fibroblasts, suggesting that NOV acted as a pharmacological chaperone to accelerate transport...

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Identifiers

PMID: 17363227
DOI: 10.1016/j.bbadis.2007.02.003

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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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