| SKIP000910 | |
| SKIP ID | SKIP000910 |
| Organism(En) | - |
| Organism(Ja) | - |
| Cell Type(En) | - |
| Cell Type(Ja) | - |
| Cell Tissue(En) | Peripheral Blood |
| Cell Tissue(Ja) | 末梢血 |
| Cell Origin | Diseased |
| Cell Name 1(En) | Alex2 |
| Cell Name 1(Ja) | Alex2 |
| Cell Name 2(En) | - |
| Cell Name 2(Ja) | - |
| Disease Name 1(Ja) | アレキサンダー病 |
| ICD Code 1 | E752 |
| Disease Name 1(En) | Alexander disease |
| OMIM1 | 203450 |
| Disease Name 2(Ja) | - |
| ICD Code 2 | - |
| Disease Name 2(En) | - |
| OMIM 2 | - |
| Disease Name 3(Ja) | - |
| ICD Code 3 | - |
| Disease Name 3(En) | - |
| OMIM 3 | - |
| Age | 10 |
| Age Range | 10-19 |
| Sex | Female |
| Race(En) | - |
| Race(Ja) | - |
| Genetic Diagnosis | Yes |
| Not Detected | No |
| Description(En) | iPSCs derived from Alexander's disease patient by using TS-SeV vectors,TS12KOS. The TS12KOS vector contains three mutations that produce alanine residues (D433A, R434A, and K437A) in the large protein (L)-binding domain of the phosphoprotein (P), a component of SeV RNA polymerase. |
| Description(Ja) | アレキサンダー病患者由来のiPS細胞。TS-センダイウィルスベクターTS12KOSを使用。 P遺伝子領域内に3か所(D433A,R434A,K437A)の変異を含み、L遺伝子領域内にc-MYCが挿入され、2か所（L1361CとL1558I)の変異を含む。 |
| Cell Morphology | human ES-like |
| Grade | Research Grade |
| Vector | Sendai virus |
| Transgene | SOX2, KLF4, OCT3/4 |
| Adhesiveness | - |
| Feeder | Yes |
| Feeder Cell | MEF |
| Medium | DMEM/F12 (SIGMA) supplemented with 20% KNOCKOUT serum replacement (KSR, Invitrogen), 2 mM L-glutamine (Life technologies), 0.1 mM nonessential amino acids (NEAA, SIGMA), 0.1 mM 2-mercaptoethanol (2ME, SIGMA), 0.5% penicillin and streptomycin (Nacalai Tesque, Japan) and 5 ng/ml or 30 ng/ml basic fibroblast growth factor (bFGF, WAKO, Japan). |
| Genome Editing | - |
| CO2 | - |
| Mycoplasma | Unknown |
| Detection of Contaminants Mycoplasma | - |
| Pluripotent Markers | Yes |
| Pluripotent Markers Assay | Immunofluorescent assay |
| in vitro Differentiation | Yes |
| in vitro Differentiation Assay | Immunofluorescent assay,EB formation |
| in vivo Differentiation | - |
| in vivo Differentiation Assay | - |
| Other 1 Assay | Yes |
| Other 1 Assay Method | - |
| Other 2 Assay | - |
| Other 2 Assay Method | - |
| Other 3 Assay | - |
| Other 3 Assay Method | - |
| Karyotype | Yes |
| Karyotype Assay | Karyotype analysis |
| Remaining Vector Detection | - |
| Remaining Vector Detection Assay | - |
| STR | - |
| HLA | - |
| Stem Cell Transcriptome analysis | - |
| Stem Cell Transcriptome analysis Assay | - |
| Author Name(En) | Takayuki Kondo |
| Author Name(Ja) | 近藤 孝之 |
| Author Organization(En) | Center for iPS Cell Research and Application (CiRA), Kyoto University |
| Author Organization(Ja) | 京都大学iPS細胞研究所 |
| Author Contact Email | ips-contact[at]cira[dot]kyoto-u[dot]ac[dot]jp |
| PI Organization(En) | Center for iPS Cell Research and Application (CiRA), Kyoto University |
| PI Organization(Ja) | 京都大学CiRA増殖機構分化機構研究部門 |
| PI Name(En) | Haruhisa Inoue |
| PI Name(Ja) | 井上 治久 |
| PI Contact Email | - |
| Availability | Information Only |
| Provider Organization(En) | Center for iPS Cell Research and Application (CiRA), Kyoto University |
| Provider Organization(Ja) | 京都大学iPS細胞研究所 |
| Provider Email | ips-contact[at]cira[dot]kyoto-u[dot]ac[dot]jp |
| Provider URL | http://www.cira.kyoto-u.ac.jp/ |
| Ethical Statement(En) | - |
| Ethical Statement(Ja) | - |
| Terms of Use(En) | - |
| Terms of Use(Ja) | - |
| PubMed ID | 27402089 -- 25479600 |
| DOI | 10.1186/s40478-016-0337-0 -- 10.1371/journal.pone.0113052 |
| Title | Modeling Alexander disease with patient iPSCs reveals cellular and molecular pathology of astrocytes. -- New type of Sendai virus vector provides transgene-free iPS cells derived from chimpanzee blood. |
| Authors | Kondo T, Funayama M, Miyake M, Tsukita K, Era T, Osaka H, Ayaki T, Takahashi R, Inoue H -- Fujie Y, Fusaki N, Katayama T, Hamasaki M, Soejima Y, Soga M, Ban H, Hasegawa M, Yamashita S, Kimura S, Suzuki S, Matsuzawa T, Akari H, Era T |
| Journal | Acta Neuropathol Commun -- PLoS One |
| Year | 2016 -- 2014 |
| Volume | 4 -- 9 |
| Issue | 1 -- 12 |
| Pages | 69 -- e113052 |
| URL | http://www.ncbi.nlm.nih.gov/pubmed/27402089 -- http://www.ncbi.nlm.nih.gov/pubmed/25479600 |
| Free input | -- |
| Note | - |