8993-A12

General

Cell Line

hPSCreg name MDCi007-A
Cite as:
MDCi007-A (RRID:CVCL_C0HD)
Alternative name(s)
8993-A12
Cell line type Human induced pluripotent stem cell (hiPSC)
Similar lines
BIHi276-A
Donor's gene variants:
MT-ATP6
Donor diseases:
Leigh Disease
MDCi008-A
(8993-B12)
Donor diseases:
Leigh Disease
MDCi010-A
(8993-D7)
Donor diseases:
Leigh Disease
BIHi267-B
Donor's gene variants:
MT-ATP6
Donor diseases:
Leigh Disease
MDCi009-A
(8993-C11)
Donor diseases:
Leigh Disease
IUFi002-A
Donor diseases:
Leigh syndrome
NTUHi004-A
Donor diseases:
Leigh syndrome
UNIZARi001-A
(FiPSTK2-2)
Donor's gene variants:
TK2, TK2
Donor diseases:
Mitochondrial DNA depletion syndrome, myopathic form
BGUi001-A
(BGU01iPORhet)
Donor diseases:
P450 Oxidoreductase Deficiency
BGUi002-A
(BGU02iPOR)
Donor diseases:
P450 Oxidoreductase Deficiency
BGUi003-A
(BGU03iPOR)
Donor diseases:
P450 Oxidoreductase Deficiency
CTGUi001-A
(FD01, F01)
Donor diseases:
Fabry Disease
UKWNLi007-A
(GLA-515G>A-1, GLA-C172Y-iPSC-1, FD1210/1)
Donor diseases:
Fabry Disease
Last update 23rd August 2022
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Provider

Generator Max Delbrück Center Berlin Buch (MDC)
Distributors
Derivation country Germany

External Databases

BioSamples SAMEA10563812
Cellosaurus CVCL_C0HD
Wikidata Q112930100

General Information

Publications
* Is the cell line readily obtainable for third parties?
No

Donor Information

General Donor Information

Sex male

Phenotype and Disease related information (Donor)

Diseases A disease was diagnosed.
Mutation in the MT-ATP6 gene m.8993T>G
The donor is a carrier of a disease-associated mutation and affected.
Synonyms
  • Leigh's Disease
  • Leigh Disease
  • Leigh Syndrome
  • Leigh's disease
show more synonyms

Karyotyping (Donor)

Has the donor karyotype been analysed?
Unknown

Other Genotyping (Donor)

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

External Databases (Donor)

BioSamples SAMEA13000844

Ethics

Has informed consent been obtained from the donor of the embryo/tissue from which the pluripotent stem cells have been derived? Yes
Was the consent voluntarily given? Yes
Has the donor been informed that participation will not directly influence their personal treatment? Yes
Can you provide us with a copy of the Donor Information Sheet provided to the donor? Yes
Do you (Depositor/Provider) hold the original Donor Consent Form? Yes
Please indicate whether the data associated with the donated material has been pseudonymised or anonymised. pseudonymised
Does consent explicitly allow the derivation of pluripotent stem cells? Yes
Does consent prevent CELLS DERIVED FROM THE DONATED BIOSAMPLE from being made available to researchers anywhere in the world? Yes
How may genetic information associated with the cell line be accessed? Controlled Access
Will the donor expect to receive financial benefit, beyond reasonable expenses, in return for donating the biosample? No
Has a favourable opinion been obtained from a research ethics committee, or other ethics review panel, in relation to the Research Protocol including the consent provisions? Yes
Name of accrediting authority involved? Ethikausschuss am Campus Virchow-Klinikum
Approval number EA2/131/13
For generation of the cell line, who was the supplier of any recombined DNA vectors or commercial kits used?

hIPSC Derivation

General

Source cell type

Reprogramming method

Vector type Non-integrating
Vector Sendai virus
Is reprogramming vector detectable?
No
Methods used
PCR

Vector free reprogramming

Other

Derived under xeno-free conditions
Unknown
Derived under GMP?
Unknown
Available as clinical grade?
Unknown

Culture Conditions

Surface coating Matrigel/Geltrex
Feeder cells
No
Passage method Enzymatically
O2 Concentration 5 %
CO2 Concentration 5 %
Medium Other medium:
Base medium: StemMACS™ iPS-Brew XF, human
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?
No
Has Rock inhibitor (Y27632) been used at thaw previously with this cell line?
Yes

Characterisation

Analysis of Undifferentiated Cells
Marker Expressed Immunostaining RT-PCR Flow Cytometry Enzymatic Assay Expression Profiles
TRA 1-60
Yes
NANOG
Yes
Differentiation Potency
Endoderm
Ont Id: UBERON_0000925
In vitro spontaneous differentiation
Marker Expressed
AFP
Yes
SOX17
Yes
Mesoderm
Ont Id: UBERON_0000926
In vitro spontaneous differentiation
Marker Expressed
SMA
Yes
FN1
Yes
Ectoderm
Ont Id: UBERON_0000924
In vitro spontaneous differentiation
Marker Expressed
PAX6
Yes
beta-tubulin III (TUJ1)
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
figure of virtual karyotype
Karyotyping method: Molecular karyotyping by SNP array
http://

Other Genotyping (Cell Line)