Title:
AMYOTROPHIC LATERAL SCLEROSIS AND/OR FRONTOTEMPORAL LOBAR DEGENERATION MODEL MOUSE
Document Type and Number:
WIPO Patent Application WO/2013/180214
Kind Code:
A1
Abstract:
[Problem] To construct a model mouse that has symptoms similar to human amyotrophic lateral sclerosis and/or human frontotemporal lobar degeneration.
[Solution] A model mouse having symptoms similar to human amyotrophic lateral sclerosis and/or human frontotemporal lobar degeneration can be constructed by inducing a mutation in a vertebrate so that, in at least one allele of vertebrate endogenous TDP-43 gene, the expression of a mutant protein in which alanine corresponding to the amino acid at the 382-position in SEQ ID NO:1 is substituted by tyrosine, or the expression of a mutant protein in which glycine corresponding to the amino acid at the 348-position in SEQ ID NO:1 is substituted by cysteine, is regulated by an endogenous TDP-43 gene promoter.
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Inventors:
OKANO HIDEYUKI (JP)
OKANO JAMES HIROTAKA (JP)
MIYAUCHI CHIKAKO (JP)
OKANO JAMES HIROTAKA (JP)
MIYAUCHI CHIKAKO (JP)
Application Number:
PCT/JP2013/065029
Publication Date:
December 05, 2013
Filing Date:
May 30, 2013
Export Citation:
Assignee:
UNIV KEIO (JP)
International Classes:
A01K67/027; C12N15/09
Other References:
TAKUYA KONNO ET AL.: "ALSIO (Idensei ALS-TDP) no Rinsho to Byori", CLINICAL NEUROSCIENCE, vol. 29, no. 9, 1 September 2011 (2011-09-01), pages 1019 - 1021, XP008176047
MAKOTO URUSHITANI: "Future perspectives of immunotherapy against ALS", CLINICAL NEUROLOGY, vol. 4, no. 11, 2009, pages 818 - 820, XP055180064
KOJI YAMANAKA: "Glial pathology in amyotrophic lateral sclerosis", NEUROLOGY RESEARCH INTERNATIONAL, vol. 2011, no. 718987, 2011, pages 1192 - 1194, XP055180075
HITOSHI TAKAHASHI: "Amytrophic latheral sclerosis and TDP-43: elucidation of the entire neuropathological picture and molecular pathomechanism", KAGAKU KENKYUHI HOJOKIN KENKYU SEIKA HOKOKUSHO, 2011, XP055180083, Retrieved from the Internet [retrieved on 20130611]
"Molecular cloning, a laboratory manual", 2012, COLD SPRING HARBOR PRESS
"Current Protocols in Molecular Biology", JOHN WILEY & SONS LTD.
NUC. ACIDS RES., vol. 28, 2000, pages E78
MAKOTO URUSHITANI: "Future perspectives of immunotherapy against ALS", CLINICAL NEUROLOGY, vol. 4, no. 11, 2009, pages 818 - 820, XP055180064
KOJI YAMANAKA: "Glial pathology in amyotrophic lateral sclerosis", NEUROLOGY RESEARCH INTERNATIONAL, vol. 2011, no. 718987, 2011, pages 1192 - 1194, XP055180075
HITOSHI TAKAHASHI: "Amytrophic latheral sclerosis and TDP-43: elucidation of the entire neuropathological picture and molecular pathomechanism", KAGAKU KENKYUHI HOJOKIN KENKYU SEIKA HOKOKUSHO, 2011, XP055180083, Retrieved from the Internet
"Molecular cloning, a laboratory manual", 2012, COLD SPRING HARBOR PRESS
"Current Protocols in Molecular Biology", JOHN WILEY & SONS LTD.
NUC. ACIDS RES., vol. 28, 2000, pages E78
Attorney, Agent or Firm:
ISSHIKI & CO. (JP)
All international patent business corporation (JP)
All international patent business corporation (JP)
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