title: Post-natal treatment by a blood-brain-barrier permeable calpain inhibitor, SNJ1945 rescued defective function in lissencephaly.
abstract: Toward a therapeutic intervention of lissencephaly, we applied a novel calpain inhibitor, SNJ1945. Peri-natal or post-natal treatment with SNJ1945 rescued defective neuronal migration in Lis1⁺/⁻ mice, impaired behavioral performance and improvement of ¹⁸F-FDG uptake. Furthermore, SNJ1945 improved the neural circuit formation and retrograde transport of NFG in Lis1⁺/⁻ mice. Thus, SNJ1945 is a potential drug for the treatment of human lissencephaly patients.
authors: Department of Genetic Disease Research, Osaka City University Graduate School of Medicine, Asahi-machi 1-4-3 Abeno, Osaka 545-8586, Japan.
authors: Shiori
authors: S
authors: Toba
authors: Yasuhisa
authors: Y
authors: Tamura
authors: Kanako
authors: K
authors: Kumamoto
authors: Masami
authors: M
authors: Yamada
authors: Keizo
authors: K
authors: Takao
authors: Satoko
authors: S
authors: Hattori
authors: Tsuyoshi
authors: T
authors: Miyakawa
authors: Yosky
authors: Y
authors: Kataoka
authors: Mitsuyoshi
authors: M
authors: Azuma
authors: Kiyoshi
authors: K
authors: Hayasaka
authors: Masano
authors: M
authors: Amamoto
authors: Keiko
authors: K
authors: Tominaga
authors: Anthony
authors: A
authors: Wynshaw-Boris
authors: Hideki
authors: H
authors: Wanibuchi
authors: Yuichiro
authors: Y
authors: Oka
authors: Makoto
authors: M
authors: Sato
authors: Mitsuhiro
authors: M
authors: Kato
authors: Shinji
authors: S
authors: Hirotsune
mesh_terms: D043203:1-Alkyl-2-acetylglycerophosphocholine Esterase / Q000235:genetics / Q000378:metabolism; D000284:Administration, Oral; D000818:Animals; D001812:Blood-Brain Barrier / Q000378:metabolism*; D002154:Calpain / Q000037:antagonists & inhibitors* / Q000378:metabolism; D002219:Carbamates / Q000737:chemistry / Q000494:pharmacology / Q000627:therapeutic use*; D002460:Cell Line; D019788:Fluorodeoxyglucose F18 / Q000737:chemistry / Q000378:metabolism; D006023:Glycoproteins / Q000737:chemistry / Q000494:pharmacology / Q000627:therapeutic use*; D006801:Humans; D054082:Lissencephaly / Q000188:drug therapy* / Q000503:physiopathology / Q000517:prevention & control; D008297:Male; D051379:Mice; D008869:Microtubule-Associated Proteins / Q000235:genetics / Q000378:metabolism; D009043:Motor Activity / Q000187:drug effects; D020932:Nerve Growth Factor / Q000378:metabolism; D009474:Neurons / Q000378:metabolism; D049268:Positron-Emission Tomography; D018079:Receptors, GABA / Q000378:metabolism
chemical_list: C515164:((1S)-1-((((1S)-1-benzyl-3-cyclopropylamino-2,3-di-oxopropyl)amino)carbonyl)-3-methylbutyl)carbamic acid 5-methoxy-3-oxapentyl ester; D002219:Carbamates; D006023:Glycoproteins; D008869:Microtubule-Associated Proteins; D018079:Receptors, GABA; C039304:calpain inhibitors; D019788:Fluorodeoxyglucose F18; D020932:Nerve Growth Factor; D043203:1-Alkyl-2-acetylglycerophosphocholine Esterase; C513190:Pafah1b1 protein, mouse; D002154:Calpain
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