General

Cell Line
hPSCreg name	INSRMe004-A
Cite as: When citing this cell line, please use the hPSCreg name (see Naming Tool ) and the corresponding Research Resource ID (RRID).	INSRMe004-A (RRID:CVCL_C083)
Alternative name(s)	STR-I-171-GLA
Cell line type	Human embryonic stem cell (hESC)
Similar lines	CMCi006-A-1 (CMC-Fb001(A4GALT-KO)) Donor diseases: Fabry Disease CMCi010-A (CMC-Fb-002) Donor diseases: Fabry Disease CMCi007-A (CMC-Fb-004) Donor diseases: Fabry Disease UKWNLi006-B (GLA-D170N-iPSC-2, GLA-508G>A-2, FD1089/21) Donor diseases: Fabry Disease UKWNLi007-B (GLA-515-G>A-2, FD1210/2, GLA-C172Y-iPSC-2) Donor diseases: Fabry Disease CMCi006-A (CMC-Fb-001) Donor diseases: Fabry Disease CTGUi001-A (FD01, F01) Donor diseases: Fabry Disease INSAi002-A Donor diseases: Fabry Disease UKWNLi006-A (GLA-D170N-iPSC-1, FD1089/20, GLA-508G>A-1) Donor diseases: Fabry Disease UKWNLi007-A (GLA-515G>A-1, GLA-C172Y-iPSC-1, FD1210/1) Donor diseases: Fabry Disease INSRMi005-A (PC119_c7)
Last update	3rd September 2022
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Provider
Generator	INSERM (INSRM) Contact: Ontogeny of primordial germ cells
Derivation country	France
External Databases
Cellosaurus	CVCL_C083
Wikidata	Q54970609
General Information
Publications	Tropel P et al. High-efficiency derivation of human embryonic stem cell lines following pre-implantation genetic diagnosis. In vitro cellular & developmental biology. Animal. 2010 Apr;46(3-4):376-85.
* Is the cell line readily obtainable for third parties?	Yes

Donor Information

General Donor Information
Sex	female
Phenotype and Disease related information (Donor)
Diseases	A disease was diagnosed. Fabry disease The donor is affected. Synonyms Alpha-galactosidase A deficiency Angiokeratoma Corporis Diffusum Fabry's disease alpha galactosidase deficiency deficiency of melibiase Fabry Disease, Cardiac Variant show more synonyms show less synonyms
Disease associated phenotypes	Turner Syndrom
Donor Relations
All cell lines of this donor's relatives	Has mother: INSRMi005-A

Ethics

Alternatives to consent are available?	Yes
Alternatives to consent
Alternative consent approval number
How may genetic information associated with the cell line be accessed?

hESC Derivation

Embryo stage	Blastula with ICM and Trophoblast
PGD Embryo?	Yes
ZP removal technique	Enzymatic

Culture Conditions

Surface coating	Gelatin
Passage method	Mechanically
CO2 Concentration	5 %
Medium	Other medium: Base medium: Knockout-DMEM Main protein source: Knock-out serum replacement Serum concentration: 20 % Supplements Supplement Amount Unit L-Glutamine bFGF

Characterisation

Differentiation Potency

Endoderm

Endoderm
Ont Id: UBERON_0000925

In vitro spontaneous differentiation

Mesoderm

Mesoderm
Ont Id: UBERON_0000926

In vitro spontaneous differentiation

Ectoderm

Ectoderm
Ont Id: UBERON_0000924

In vitro spontaneous differentiation

Microbiology / Virus Screening
HIV 1	Negative
HIV 2	Negative
Hepatitis B	Negative
Hepatitis C	Negative

Genotyping

Karyotyping (Cell Line)
Has the cell line karyotype been analysed?	Yes 45, XO
Other Genotyping (Cell Line)