BGU101iCCHS

General

Cell Line

hPSCreg name BGUi004-A
Cite as:
BGUi004-A (RRID:CVCL_A9XA)
Alternative name(s)
BGU101iCCHS
Cell line type Human induced pluripotent stem cell (hiPSC)
Similar lines
LUMCi002-A
(113-6, LUMC0113iATAX06)
Donor diseases:
Spinocerebellar Ataxia Type 1
ZZUi030-A
(ZZU-iPS-SPG7-001)
Donor diseases:
Spastic paraplegia type 7
LUMCi002-B
(113-7, LUMC0113iATAX07)
Donor diseases:
Spinocerebellar Ataxia Type 1
LUMCi002-C
(113-8, LUMC0113iATAX08)
Donor diseases:
Spinocerebellar Ataxia Type 1
HDZi003-A
(hiPSC NP0038)
Donor's gene variants:
TMEM43
Donor diseases:
arrhythmogenic right ventricular dysplasia 5
FRIMOi004-A
(STGD2_ FiPS4F1.7)
Donor diseases:
Stargardt Disease
HIHDNDi001-A
(A30P-3, SNCA3, Tue_020_A)
Donor's gene variants:
SNCA, SNCA, SNCA
Donor diseases:
autosomal dominant Parkinson disease 1
HIHDNDi001-B
(A30P-4, SNCA4, Tue_020_B)
Donor's gene variants:
SNCA, SNCA, SNCA
Donor diseases:
autosomal dominant Parkinson disease 1
ZZUi026-A
(ZZU-iPS-SCA3-003)
Donor diseases:
Spinocerebellar Ataxia Type 3
UTSWi001-A
(FA1)
Donor diseases:
Friedreich Ataxia
VUi011-A
(SCZ 3.5)
Donor diseases:
Schizophrenia
DHMi004-A
(HOS_1460)
Donor diseases:
Holt-Oram Syndrome
UPITTi004-A
(CN090 C5A5J2)
Donor diseases:
Sickle cell anemia
UPITTi004-B
(CN090 C1B5B5)
Donor diseases:
Sickle cell anemia
AIBNi015-A
(SPG1-AU01C15)
Donor diseases:
hereditary spastic paraplegia 56
RMCGENi020-A
(IPS15-00004)
Donor diseases:
Stargardt Disease
RMCGENi021-A
(IPS22-00087)
Donor diseases:
Stargardt Disease
SCTCi017-A
(IPS15-00006)
Donor diseases:
Stargardt disease
SCTCi018-A
(IPS15-00007)
Donor diseases:
Stargardt disease
ZZUi017-A
(ZZU-iPS-SCA6-001)
Donor diseases:
Spinocerebellar Ataxia Type 6
UKBi014-A
(A-257s2)
Donor diseases:
Walker-Warburg syndrome
FRIMOi003-A
(STGD1_ FiPS4F1.5)
Donor diseases:
Stargardt Disease
LUMCi022-A
(115-1, LUMC0115iATAX01)
Donor diseases:
Spinocerebellar Ataxia Type 1
LUMCi022-B
(115-2, LUMC0115iATAX02)
Donor diseases:
Spinocerebellar Ataxia Type 1
LUMCi022-C
(115-7, LUMC0115iATAX07)
Donor diseases:
Spinocerebellar Ataxia Type 1
FINCBi005-A
(mt1072 clone #103, mt1072 #103)
Donor's gene variants:
MT-ND6, MT-ND6, MT-ND4L, MT-ND4L
Donor diseases:
Leber hereditary optic neuropathy
FINCBi006-A
(mt1108 clone #121, mt1108 #121)
Donor's gene variants:
MT-ND6, MT-ND6, MT-ND4L, MT-ND4L
Donor diseases:
Leber hereditary optic neuropathy
UNIZARi001-A
(FiPSTK2-2)
Donor's gene variants:
TK2, TK2
Donor diseases:
Mitochondrial DNA depletion syndrome, myopathic form
IDIBGIi003-A
(​RB20235)
Donor diseases:
Brugada syndrome
BGUi001-A
(BGU01iPORhet)
Donor diseases:
P450 Oxidoreductase Deficiency
BGUi002-A
(BGU02iPOR)
Donor diseases:
P450 Oxidoreductase Deficiency
BGUi003-A
(BGU03iPOR)
Donor diseases:
P450 Oxidoreductase Deficiency
Last update 4th February 2021
User feedback
No feedback available yet.

Login to share your feedback, experiences or results with the research community.

Provider

Generator Ben Gurion University of the Negev (BGU)
Owner Ben Gurion University of the Negev (BGU)
Distributors
Derivation country Israel

External Databases

Cellosaurus CVCL_A9XA
BioSamples SAMEA8072981
Wikidata Q102113618

General Information

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

Donor Information

General Donor Information

Sex male
Ethnicity Ashkenazi Jew

Phenotype and Disease related information (Donor)

Diseases A disease was diagnosed.
The donor is a carrier of a disease-associated mutation and affected.
Synonyms
  • CCHS
  • Ondine curse
  • Ondine syndrome
  • central congenital hypoventilation syndrome
  • congenital central alveolar hypoventilation syndrome
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 SAMEA8152183

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? No
Please provide contact information of the holder of the original Donor Information Sheet. Avital Adato, Yad La-Neshima Association, CCHS, Israel
Do you (Depositor/Provider) hold the original Donor Consent Form? No
If you do not hold the Donor Consent Form, do you know who does? Yes
Please provide the contact information Avital Adato, Yad La-Neshima Association, CCHS, Israel
Please indicate whether the data associated with the donated material has been pseudonymised or anonymised. anonymised
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? No

Does consent permit research by

an academic institution? Yes
a public organisation? Yes
a non-profit company? 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
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? No
Has a favourable opinion been obtained from a research ethics committee, or other ethics review panel, in relation to the PROPOSED PROJECT, involving use of donated embryo/tissue or derived cells? No
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 Episomal
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

Feeder cells
No
Medium Other medium:
Base medium: NutriStem® hPSC XF Medium
Main protein source:
Serum concentration: %

Characterisation

Analysis of Undifferentiated Cells
Marker Expressed Immunostaining RT-PCR Flow Cytometry Enzymatic Assay Expression Profiles
NANOG
Yes
OCT3/4
Yes
SOX2
Yes
SSEA-4
Yes
TRA 1-60
Yes
Differentiation Potency
Endoderm
Ont Id: UBERON_0000925
In vitro spontaneous differentiation
Marker Expressed
AFP
Yes
Morphology
Mesoderm
Ont Id: UBERON_0000926
In vitro spontaneous differentiation
Marker Expressed
ACTA2
Yes
Morphology
Ectoderm
Ont Id: UBERON_0000924
In vitro spontaneous differentiation
Marker Expressed
NEFL
Yes
Morphology

Genotyping

Karyotyping (Cell Line)

Has the cell line karyotype been analysed?
Yes
Normal, male
Passage number: 4
Karyotyping method: G-Banding

Other Genotyping (Cell Line)