The cell line is not validated yet.

General

Cell Line

hPSCreg name UTSWi004-A
Cite as:
UTSWi004-A
Cell line type Human induced pluripotent stem cell (hiPSC)
Similar lines No similar lines found.
Last update 26th January 2026
Notes These cells are derived from a patient with c.165+5G>C FXN point mutation.
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Provider

Generator University of Texas Southwestern Medical Center (UTSW)

External Databases

BioSamples SAMEA121036550

General Information

* 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.
The donor is a carrier of a disease-associated mutation and affected.
Synonyms
  • Friedreich's Ataxia
  • Friedreich Ataxia
  • Friedreich ataxia
Genetic variants
donor is a compound heterozygote with GAA repeat expansion mutation on one allele and point mutation FXN c.165+5G>C on the second allele.

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 SAMEA121037395

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
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 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? No
Does consent prevent CELLS DERIVED FROM THE DONATED BIOSAMPLE from being made available to researchers anywhere in the world? No
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? CHOP IRB
Approval number IRB 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
White blood cells formed in the body's lymphoid tissue. The nucleus is round or ovoid with coarse, irregularly clumped chromatin while the cytoplasm is typically pale blue with azurophilic (if any) granules. Most lymphocytes can be classified as either T or B (with subpopulations of each); those with characteristics of neither major class are called null cells.
Synonyms
  • Lymphocyte
  • Lymph Cell
  • Lymphocytes
  • lymphocyte
  • LYMPHOCYTE
show more synonyms
Source cell origin
A liquid tissue; its major function is to transport oxygen throughout the body. It also supplies the tissues with nutrients, removes waste products, and contains various components of the immune system defending the body against infection. Several hormones also travel in the blood.
Synonyms
  • Reticuloendothelial System, Blood
  • Peripheral Blood
  • peripheral blood
  • blood
  • Blood
  • Whole Blood
  • BLOOD
  • portion of blood
  • vertebrate blood
show more synonyms

Reprogramming method

Vector type Non-integrating
Vector Sendai virus
Genes
Is reprogramming vector detectable?
No
Methods used
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
NANOG
Yes
TRA 1-60
Yes
Self-renewal
Positive
Endoderm
Negative
Mesoderm
Negative
Ectoderm score
Negative
Differentiation Potency
Endoderm
Ont Id: UBERON_0000925
In vitro spontaneous differentiation
Scorecard
Mesoderm
Ont Id: UBERON_0000926
In vitro spontaneous differentiation
Scorecard
Ectoderm
Ont Id: UBERON_0000924
In vitro spontaneous differentiation
Scorecard

Genotyping

Karyotyping (Cell Line)

Has the cell line karyotype been analysed?
Yes
46,XY
Passage number: 5
Karyotyping method: G-Banding

Other Genotyping (Cell Line)