CIRAi005-A-1

IsC2-F2KU1#4, IsC-F2KU1

General#

Cell Line

hPSCreg Name CIRAi005-A-1
Alternative name(s)
IsC2-F2KU1#4, IsC-F2KU1
Cell line type Human induced pluripotent stem cell (hiPSC)
Last update 29th May 2020
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Provider

Generator Center for iPS Cell Research and Application (CIRA)
Owner Center for iPS Cell Research and Application (CIRA)
Distributors
Derivation country Japan

External Databases

BioSamples SAMEA7001537
ECACC 66770001
Cellosaurus CVCL_ZA42
CLO CLO_0102828

General Information

Notes FSHD2 patient-derived iPSC with SMCHD1 mutation corrected and without transgenes
* Is the cell line readily obtainable for third parties?
No
Subclone of

Donor Information#

General Donor Information

Sex female
Ethnicity Japanese

Phenotype and Disease related information (Donor)

Diseases A disease was diagnosed.
facioscapulohumeral muscular dystrophy 2
The donor is a carrier of a disease-associated mutation and affected.
Synonyms
  • facioscapulohumeral muscular dystrophy 1B
  • FSHD2
  • facioscapulohumeral muscular dystrophy type 2

Karyotyping (Donor)

Has the donor karyotype been analysed?
Unknown

Other Genotyping (Donor)

Is there genome-wide genotyping or functional data available?
No

Donor Relations

Other cell lines of this donor

External Databases (Donor)

BioSamples SAMEA6983573

Ethics#

Also have a look at the ethics information for the parental line CIRAi005-A .
Is there an MTA available for the cell line? No
For generation of the cell line, who was the supplier of any recombined DNA vectors or commercial kits used?
Are you aware of any constraints on the use or distribution of the cell line from the owner or any parties identified in the query above? No

hIPSC Derivation#

General

The source cell information can be found in the parental cell line CIRAi005-A.

Reprogramming method

Vector type Non-integrating
Vector Episomal
Is reprogramming vector detectable?
No
Methods used
PCR
Files and images showing reprogramming vector expressed or silenced

Vector free reprogramming

Other

Derived under xeno-free conditions
No
Derived under GMP?
No
Available as clinical grade?
No

Culture Conditions#

Surface coating Laminin
Feeder cells
No
Passage method Enzymatically
Accutase
CO2 Concentration 5 %
Medium Other medium:
Base medium: StemFit® AK02N
Main protein source:
Serum concentration: %
Has Rock inhibitor (Y27632) been used at passage previously with this cell line?
Yes
Has Rock inhibitor (Y27632) been used at cryo previously with this cell line?
Yes
Has Rock inhibitor (Y27632) been used at thaw previously with this cell line?
Yes

Characterisation#

Analysis of Undifferentiated Cells
Marker Expressed Immunostaining RT-PCR FACS Enzymatic Assay Expression Profiles
SSEA-4
Yes
TRA 1-60
Yes
NANOG
Yes
POU5F1 (OCT-4)
Yes
SOX2
Yes
Differentiation Potency
Endoderm
Ont Id: UBERON_0000925
In vitro directed differentiation
Marker Expressed
FOXA2
Yes
SOX17
Yes
Mesoderm
Ont Id: UBERON_0000926
In vitro directed differentiation
Marker Expressed
T
Yes
NCAM1
Yes
Ectoderm
Ont Id: UBERON_0000924
In vitro directed differentiation
Marker Expressed
SOX1
Yes
PAX6
Yes

Microbiology / Virus Screening

HIV 1 Negative
HIV 2 Negative
Hepatitis B Negative
Hepatitis C Negative
Mycoplasma Negative

Genotyping#

Karyotyping (Cell Line)

Has the cell line karyotype been analysed?
Yes
(46, X, X)
Karyotyping method: G-Banding

Other Genotyping (Cell Line)

Genetic Modification#

Disease/phenotype related modifications
facioscapulohumeral muscular dystrophy 2
Synonyms
  • facioscapulohumeral muscular dystrophy 1B
  • FSHD2
  • facioscapulohumeral muscular dystrophy type 2
Genetic modifications
SMCHD1 (target)
Isogenic modification
18p11.32
NM_015295.3:c.4118_4132del and c.4137A>G
NP_056110.2:p.(Val1373_Lys1377del, Ala1379Ala)
Heterozygous
Repaired