Cell No. : Cell Name HPS0292 : CiRA00024  update : 2023/06/14
Comment			Disease specific iPS cell line derived from a patient : Amyotrophic lateral sclerosis (ALS). HPS0292, HPS0293, HPS0294, HPS1717 were derived from the same patient. Analysis of causal genetic changes. Cryopreserved by vitrification method.
Comment from the depositor
Terms and conditions			1) The BIOLOGICAL RESOURCE must be used for academic research purpose conducted by RECIPIENT who belongs to a not-for profit academic organization (i.e. a university or another institution of higher education or any non-profit scientific or educational organization, including government agencies) . 2) The RECIPIENT agrees to obtain the prior written approval of the distribution from the APPROVER. 3) Contact Us : RIKEN Cell Bank
Remarks
Order Form			Order Form(C-0042.pdf)   Approval Form(C-0057.pdf)   MTA(C-0007.pdf)
			Regarding MTA between user institutions and RIKEN BRC, there are two kinds of MTA, not-for-profit academic purpose (C-XXXX) and for-profit research purpose (C-XXXXp) , depending on the sort of user institutions and the purposes of use. Please use an appropriate MTA(to see). In relation to commercial use and use for patent filing, first of all Please contact RIKEN BRC (cellbank.brc@riken.jp).
	Basic information	Depositor	https://www.cira.kyoto-u.ac.jp/
		Animal	_human < Mammals
		Genus	Homo
		Species	sapiens
		Race	Japanese
		Gender	Female
		Age at sampling	60s
		Tissue	skin
		Disease name	amyotrophic lateral sclerosis (ALS)
		Classification	iPS
		Recombinant	recombinant
		Exogene	Oct3/4, Sox2, Klf4, L-Myc, Lin28, shp53
		Lifespan	infinite
		Morphology	ES-like
		Cellosaurus(Expasy)	CVCL_T781
			deposit info	lot info
		Medium	Medium List
		Culture type		Adherent cells
		Culture medium		Primate ES Cell Medium + 4ng/ml human basic FGF
		Antibiotics		Free
		Passage method		0.25% Trypsin + 0.1% collagenase type IV + 20% KSR + 1mM CaCl2 + PBS(-)
		Coating dish		0.1% gelatin coated dish
	Culture information	Passage ratio		1 : 3-5 split
		SC frequency		Subculture : once/4-6 days, Medium Renewal : every day
		Temperature		37 ℃
		CO2 concentration		5 %
		Feeder cells		SNL 76/7
		Feeder treatment		X-rays 5000R (or MMC)
		Feeder seed cells		1.5-2.5 x 10 6 cells/100 mm dish
		Freeze medium		DAP213 : Medium + 2M DMSO + 1M Acetamide + 3M Propyleneglycol
		Freezing method		Vitrification
		Mycoplasma/Acholeplasma		(-)
		STR(human)		OK
Images			deposit info	lot info
Relational File			deposit info	lot info
				SDS_DAP(MEM)_Japanese.pdf
Reference information		Reference	1
		User's Publication	2

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Reference

4006  Egawa N1, Kitaoka S, Tsukita K, Naitoh M, Takahashi K, Yamamoto T, Adachi F, Kondo T, Okita K, Asaka I, Aoi T, Watanabe A, Yamada Y, Morizane A, Takahashi J, Ayaki T, Ito H, Yoshikawa K, Yamawaki S, Suzuki S, Watanabe D, Hioki H, Kaneko T, Makioka K, Okamoto K, Takuma H, Tamaoka A, Hasegawa K, Nonaka T, Hasegawa M, Kawata A, Yoshida M, Nakahata T, Takahashi R, Marchetto MC, Gage FH, Yamanaka S, Inoue H.  Drug screening for ALS using patient-specific induced pluripotent stem cells.  Sci Transl Med  2012  4:145  PubMed ID: 22855461   DOI: 10.1126/scitranslmed.3004052

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User's Publication

19541  Fang M, Deibler SK, Nana AL, Vatsavayai SC, Banday S, Zhou Y, Almeida S, Weiss A, Brown RH, Seeley WW, Gao FB, Green MR.  Loss of TDP-43 function contributes to genomic instability in amyotrophic lateral sclerosis  Front Neurosci  2023  17:1251228  PubMed ID: 37849894   DOI: 10.3389/fnins.2023.1251228

11123  Tsuburaya N, Homma K, Higuchi T, Balia A, Yamakoshi H, Shibata N, Nakamura S, Nakagawa H, Ikeda SI, Umezawa N, Kato N, Yokoshima S, Shibuya M, Shimonishi M, Kojima H, Okabe T, Nagano T, Naguro I, Imamura K, Inoue H, Fujisawa T, Ichijo H.  A small-molecule inhibitor of SOD1-Derlin-1 interaction ameliorates pathology in an ALS mouse model.  Nat Commun  2018  9(1):2668  PubMed ID: 29991716   DOI: 10.1038/s41467-018-05127-2