General

Cell Line

hPSCreg name BIHi267-B
Cite as:
BIHi267-B (RRID:CVCL_D0QZ)
Cell line type Human induced pluripotent stem cell (hiPSC)
Similar lines
BIHi276-A
Donor's gene variants:
MT-ATP6
Donor diseases:
Leigh Disease
MDCi007-A
(8993-A12)
Donor diseases:
Leigh Disease
MDCi008-A
(8993-B12)
Donor diseases:
Leigh Disease
MDCi010-A
(8993-D7)
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 12th January 2023
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Provider

Generator Berlin Institute of Health (BIH)
Owner Charité - Universitätsmedizin Berlin (CUB)
Distributors
Derivation country Germany

External Databases

BioSamples SAMEA11951375
Cellosaurus CVCL_D0QZ
Wikidata Q123030794

General Information

Publications
Projects
* Is the cell line readily obtainable for third parties?
Yes
Research use: allowed
Clinical use: not allowed
Commercial use: not allowed
Additional restrictions:

The cell line can be obtained by third parties using appropriate MTA.

Donor Information

General Donor Information

Sex male
Ethnicity Caucasian

Phenotype and Disease related information (Donor)

Diseases A disease was diagnosed.
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
Genetic variants
MT-ATP6 (target)
m.9176T>G
Is the medical history available upon request? yes (anonymously)
Is clinical information available? yes (anonymously)

Karyotyping (Donor)

Has the donor karyotype been analysed?
No

Other Genotyping (Donor)

Is there genome-wide genotyping or functional data available?
Yes

Donor Relations

Other cell lines of this donor
All cell lines of this donor's relatives

External Databases (Donor)

BioSamples SAMEA11951376

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
Alternatives to consent are available? No
Is there other documentation provided to the donor for consenting purposes? No
Confirm that consent was obtained by a qualified professional Yes
Has the donor agreed to be re-contacted? Yes
Has the donor been informed about how her/his data will be protected? 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 expressly prevent the derivation of pluripotent stem cells? No
Does consent pertain to a specific research project? Yes
Details on restriction to research project
Does consent permit unforeseen future research, without further consent? No
Does the consent permit uses of donated embryo/tissue or derived cell line intended for clinical treatment or human applications? Yes
Does consent expressly prevent financial gain from any use of the donated embryo/tissue, including any product made from it? Yes
Does consent prevent CELLS DERIVED FROM THE DONATED BIOSAMPLE from being made available to researchers anywhere in the world? No

Does consent permit research by

an academic institution? Yes
a public organisation? Yes
a non-profit company? Yes
a for-profit corporation? Yes
Does consent expressly permit collection of genetic information? Yes
Does consent expressly permit storage of genetic information? Yes
Does consent prevent dissemination of genetic information? Yes
Has the donor been informed that their donated biosample or derived cells may be tested for the presence of microbiological agents / pathogens? Yes
How may genetic information associated with the cell line be accessed? No information
Will the donor expect to receive financial benefit, beyond reasonable expenses, in return for donating the biosample? No
Does the consent anticipate that the donor will be notified of results or outcomes of any research involving the donated samples or derived cells? No
Does the consent permit the donor, upon withdrawal of consent, to stop the use of the derived cell line(s) that have already been created from donated samples? Yes
Does the consent permit the donor, upon withdrawal of consent, to stop delivery or use of information and data about the donor? Yes
Does consent permit access to medical records of the donor? No
Does consent permit access to any other source of information about the clinical treatment or health of the donor? 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? Ethikkommission Ethikausschuss am Campus Virchow-Klinikum
Approval number EA2/131/13
Do you have obligations to third parties in regard to the use of the cell line? No
Are you aware of any further constraints on the use of the donated embryo/tissue or derived cells? No
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

Source cell type
Synonyms
  • fibroblast
  • Fibroblasts
  • Fibroblast
  • FIBROBLAST
show more synonyms
Source cell origin
An organ that constitutes the external surface of the body. It consists of the epidermis, dermis, and skin appendages.
Synonyms
  • Skin
  • Human Skin
  • SKIN
  • Integument
  • Skin, total
  • Skin, NOS
  • Skin structure (body structure)
  • entire skin
  • skin of body
  • skin organ
show more synonyms

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
Yes
Derived under GMP?
No
Available as clinical grade?
No

Culture Conditions

Surface coating Matrigel/Geltrex
Feeder cells
No
Passage method Enzyme-free cell dissociation
EDTA
O2 Concentration 5 %
CO2 Concentration 5 %
Medium Essential 8™
Has Rock inhibitor (Y27632) been used at passage previously with this cell line?
No
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
SSEA-4
Yes
SOX2
Yes
POU5F1 (OCT-4)
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
FN1
Yes
ACTA2
Yes
Ectoderm
Ont Id: UBERON_0000924
In vitro spontaneous differentiation
Marker Expressed
PAX6
Yes
TUBB3
Yes

Microbiology / Virus Screening

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

Certificate of Analysis

Is there a certificate of analysis available?
Yes
Passage:

Genotyping

Karyotyping (Cell Line)

Has the cell line karyotype been analysed?
Yes
normal
Passage number: 16
Karyotyping method: Molecular karyotyping by SNP array
http://

Other Genotyping (Cell Line)