FA1

General

Cell Line

hPSCreg name UTSWi001-A
Cite as:
UTSWi001-A
Alternative name(s)
FA1
Cell line type Human induced pluripotent stem cell (hiPSC)
Similar lines
UTSWi001-A-1
(FA1ic)
Donor diseases:
Friedreich Ataxia
UTSWi001-A-2
(FA1-N)
Donor diseases:
Friedreich Ataxia
UTSWi001-A-3
(FA1ic-N)
Donor diseases:
Friedreich Ataxia
UTSWi002-A-1
(FA2ic)
Donor diseases:
Friedreich Ataxia
UTSWi003-A-1
(FA3ic)
Donor's gene variants:
fxn
Donor diseases:
Friedreich Ataxia
UTSWi002-A
(FA2)
Donor diseases:
Friedreich Ataxia
USFi001-A
(1CN1.5)
Donor diseases:
Friedreich Ataxia
IGIBi012-A
(FA-hiPSC_001)
Donor diseases:
Friedreich Ataxia
IGIBi013-A
(FA_hiPSC_002)
Donor diseases:
Friedreich Ataxia
IGIBi016-A
(FA-hiPSC_005)
Donor diseases:
Friedreich Ataxia
UTSWi003-A
(FA3)
Donor's gene variants:
fxn
Donor diseases:
Friedreich Ataxia
UTSWi002-A-2
(FA2-N)
Donor diseases:
Friedreich Ataxia
UTSWi002-A-3
(FA2ic-N)
Donor diseases:
Friedreich Ataxia
UTSWi003-A-2
(FA3-N)
Donor's gene variants:
fxn
Donor diseases:
Friedreich Ataxia
UTSWi003-A-3
(FA3ic-N)
Donor's gene variants:
fxn
Donor diseases:
Friedreich Ataxia
ZZUi030-A
(ZZU-iPS-SPG7-001)
Donor diseases:
Spastic paraplegia type 7
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
UPITTi004-A
(CN090 C5A5J2)
Donor diseases:
Sickle cell anemia
UPITTi004-B
(CN090 C1B5B5)
Donor diseases:
Sickle cell anemia
LUMCi002-A
(113-6, LUMC0113iATAX06)
Donor diseases:
Spinocerebellar Ataxia Type 1
LUMCi002-B
(113-7, LUMC0113iATAX07)
Donor diseases:
Spinocerebellar Ataxia Type 1
LUMCi002-C
(113-8, LUMC0113iATAX08)
Donor diseases:
Spinocerebellar Ataxia Type 1
FRIMOi004-A
(STGD2_ FiPS4F1.7)
Donor diseases:
Stargardt Disease
ZZUi026-A
(ZZU-iPS-SCA3-003)
Donor diseases:
Spinocerebellar Ataxia Type 3
ZZUi012-A
(ZZU-iPS-PFBC-SLC20A2-001)
Donor diseases:
Bilateral striopallidodentate calcinosis
LCPHi003-A
Donor's gene variants:
LRP10
Donor diseases:
Parkinson Disease
UMi036-A
(ND34263)
Donor diseases:
Parkinson Disease
ZZUi013-A
(ZZU-iPS-AD-MEOX2-001)
Donor diseases:
obsolete_Alzheimer's disease
ZZUi027-A
(ZZU-iPS-PD-RAB39b-002)
Donor diseases:
Parkinson Disease
LCSBi001-A
(VPS35 1_2)
Donor diseases:
obsolete_Parkinson's disease
FDHSi002-A
(FDITBRi002-A)
Donor diseases:
Parkinson Disease
UCSCi001-A
(SII-1802)
Donor diseases:
Amyotrophic Lateral Sclerosis
STBCi320-A
(SFC031-03-03)
Donor diseases:
Parkinson disease
STBCi295-B
(SFC847-03-08)
Donor diseases:
Parkinson disease
LCSBi013-A
(GL2)
Donor diseases:
Parkinson Disease
UNIZARi001-A
(FiPSTK2-2)
Donor's gene variants:
TK2, TK2
Donor diseases:
Mitochondrial DNA depletion syndrome, myopathic form
Last update 25th March 2024
Notes iPSC line derived from skin fibroblasts of Friedreich's ataxia patient
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Provider

Generator University of Texas Southwestern Medical Center (UTSW)
Owner University of Texas Southwestern Medical Center (UTSW)
Distributors
Derivation country United States

External Databases

BioSamples SAMEA115427378

General Information

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

no distribution without permission

Subclones

Donor Information

General Donor Information

Sex male

Phenotype and Disease related information (Donor)

Diseases A disease was diagnosed.
The donor is not a carrier of a disease-associated mutation and affected.
Synonyms
  • Friedreich's Ataxia
  • Friedreich Ataxia
  • Friedreich ataxia
Genetic variants
cell line with homozygous GAA expansion
Family history none
Is the medical history available upon request? limited
Is clinical information available? limited

Karyotyping (Donor)

Has the donor karyotype been analysed?
Yes
46 XY
Karyotyping method: G-Banding

Other Genotyping (Donor)

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

External Databases (Donor)

BioSamples SAMEA115425256

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. Dr. David Lynch; CHOP
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 Dr. David Lynch; CHOP
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 expressly prevent the derivation of pluripotent stem cells? No
Does consent pertain to a specific research project? No
Does consent permit unforeseen future research, without further consent? Yes
Does the consent permit uses of donated embryo/tissue or derived cell line intended for clinical treatment or human applications? No
Does consent expressly prevent development of commercial products? Yes
Does consent expressly prevent financial gain from any use of the donated embryo/tissue, including any product made from it? No
Does consent expressly permit storage of donated embryo/tissue for an unlimited time? Yes
Does consent expressly permit storage of cells derived from the donated embryo/tissue for an unlimited time? Yes
Does consent prevent the DONATED BIOSAMPLE from being made available to researchers anywhere in the world? No
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? No
Does consent expressly permit storage of genetic information? No
Does consent prevent dissemination of genetic information? No
Has the donor been informed that their donated biosample or derived cells may be tested for the presence of microbiological agents / pathogens? No
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
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? CHOP IRB
Approval number 10-007864
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
Immunostaining, RT-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
Dispase
O2 Concentration 21 %
CO2 Concentration 5 %
Medium mTeSR™ 1
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
POU5F1 (OCT-4)
Yes
SSEA-4
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
tbxt2
Yes
ncam1
Yes
Ectoderm
Ont Id: UBERON_0000924
In vitro directed differentiation
Marker Expressed
pax6
Yes
nestin
Yes

Microbiology / Virus Screening

Mycoplasma Negative

Genotyping

Karyotyping (Cell Line)

Has the cell line karyotype been analysed?
Yes
46XY
Karyotyping method: G-Banding

Other Genotyping (Cell Line)