Therapeutic chaperone effect of N-Octyl 4-Epi-β-valienamine on murine GM1-gangliosidosis

Molecular Genetics and Metabolism

Volumn 106, Issue 1, 2012, Pages 92-98

  Suzuki, Yoshiyuki ^a   Ichinomiya, Satoshi ^a,b   Kurosawa, Mieko ^a   Matsuda, Junichiro ^c   Ogawa, Seiichiro ^d   Iida, Masami ^e   Kubo, Takatoshi ^e   Tabe, Miho ^f   Itoh, Masayuki ^g   Higaki, Katsumi ^h   Nanba, Eiji ^h   Ohno, Kousaku ⁱ  

^a INTERNATIONAL UNIVERSITY OF HEALTH AND WELFARE   (Japan)

^b NASU RED CROSS HOSPITAL   (Japan)

^c NATIONAL INSTITUTE OF BIOMEDICAL INNOVATION   (Japan)

^d KEIO UNIVERSITY   (Japan)

^e SEIKAGAKU CORPORATION   (Japan)

^f SRL INC   (Japan)

^g NATIONAL INSTITUTE OF NEUROSCIENCE   (Japan)

^h TOTTORI UNIVERSITY   (Japan)

ⁱ TOTTORI UNIVERSITY   (Japan)

Author keywords

Galactosidase; Chaperone therapy; GM1 gangliosidosis; N octyl 4 epi valienamine; NOEV

Indexed keywords

BETA GALACTOSIDASE; CHAPERONE; N OCTYL 4 EPI BETA VALIENAMINE; UNCLASSIFIED DRUG;

ANIMAL EXPERIMENT; ANIMAL MODEL; ANIMAL TISSUE; AREA UNDER THE CURVE; ARTICLE; BLOOD BRAIN BARRIER; DISEASE COURSE; DRUG ABSORPTION; DRUG BLOOD LEVEL; DRUG DETERMINATION; DRUG EXCRETION; DRUG HALF LIFE; DRUG MEGADOSE; DRUG SYNTHESIS; DRUG TISSUE LEVEL; GM1 GANGLIOSIDOSIS; INTESTINE ABSORPTION; LOW DRUG DOSE; MAXIMUM PLASMA CONCENTRATION; MOUSE; NONHUMAN; OUTCOME ASSESSMENT; PRIORITY JOURNAL; PROTEIN EXPRESSION; SURVIVAL; TIME TO MAXIMUM PLASMA CONCENTRATION; URINARY EXCRETION;

ANIMALS; BETA-GALACTOSIDASE; BETA-GLUCOSIDASE; BLOOD-BRAIN BARRIER; CENTRAL NERVOUS SYSTEM; DISEASE MODELS, ANIMAL; GANGLIOSIDOSIS, GM1; GENE EXPRESSION REGULATION; HEXOSAMINES; HUMANS; INTESTINES; MICE; MICE, INBRED C57BL; MICE, KNOCKOUT; MOLECULAR CHAPERONES; URINALYSIS;

MURINAE; MUS;

EID: 84860141738     PISSN: 10967192     EISSN: 10967206     Source Type: Journal    
DOI: 10.1016/j.ymgme.2012.02.012     Document Type: Article

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