IMBPASi001-A

iPSC-WFS1-#1

General#

Cell Line

hPSCreg Name IMBPASi001-A
Alternative name(s)
iPSC-WFS1-#1
Cell line type Human induced pluripotent stem cell (hiPSC)
Last update 11th March 2020
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Provider

Generator Institute of Medical Biology of Polish Academy of Sciences (IMBPAS)
Owner Institute of Medical Biology of Polish Academy of Sciences (IMBPAS)
Distributors
Derivation country Poland

External Databases

BioSamples SAMEA6877873
Cellosaurus CVCL_YT29

General Information

Notes Wolfram Syndrome manifests in insulin-independent diabetes, diabetes insipidus, optic nerve atrophy, neurodegeneration, and endocrine disorders. It results in death in middle adulthood. The iPSCs line can be applied as a tool for disease modeling, and identification of therapeutic drugs for rescuing a normal phenotype.
* Is the cell line readily obtainable for third parties?
Yes
Research: allowed
Clinical: not allowed
Commercial: not allowed
Additional restrictions:

Distribution of the cell line according to the Coriell Institute for Medical Research policy.

Donor Information#

General Donor Information

Sex female
Age of donor (at collection) 10-14
Ethnicity Caucasian

Phenotype and Disease related information (Donor)

Diseases A disease was diagnosed.
Wolfram syndrome 1 (primary disease)
Wolfram Syndrome 1 (OMIM #222300)
The donor is a carrier of a disease-associated mutation and affected.
Stage
Onset at age 11; optic atrophy; on insulin.
Disease associated phenotypes
  • insulin-independent diabetes
  • diabetes mellitus
  • diabetes insipidus
  • optic nerve atrophy
  • neurodegeneration

Karyotyping (Donor)

Has the donor karyotype been analysed?
No

External Databases (Donor)

BioSamples SAMEA6877872

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
Provide contact information of the holder of the original Donor Information Sheet: Coriell Institute for Medical Research
Do you (Depositor/Provider) hold a copy of the SIGNED Donor Consent Form? No
If you do not hold the SIGNED Donor Consent Form, do you know who does? No
If you do not hold the SIGNED Donor Consent Form, have you obtained a copy of the unsigned Donor Consent Form from the holder? No
Alternatives to consent are available? No
Is there other documentation provided to the donor for consenting purposes? No
Has the donor agreed to be re-contacted? Unknown
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 pertain to a specific research project? No
Does consent permit unforeseen future research, without further consent? Yes
Does consent expressly prevent development of commercial products? Yes
Does consent expressly permit storage of donated embryo/tissue for an unlimited time? No
Does consent expressly permit storage of cells derived from the donated embryo/tissue for an unlimited time? No
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
an academic institution? Yes
a public organisation? Yes
a non-profit company? Yes
a for-profit corporation? 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
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? US National Institutes of Health
Approval number CC-GM-15-004
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
Are you aware of any further constraints on the use of the 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? Addgene

hIPSC Derivation#

General

Source cell line name GM01610
Source cell type
fibroblast of dermis
Age of donor (at collection) 10-14
Source cell line vendor Coriell Institute for Medical Research
Passage number reprogrammed 5

Reprogramming method

Vector type Non-integrating
Vector Episomal
Genes
Is reprogramming vector detectable?
No
Methods used
PCR
Notes on reprogramming vector detection PCR reaction was used to detect reprogramming vectors in the isolated genomic DNA, using episomal plasmid-specific primers. After passage 20, the episomal vectors were found absent, indicating that these iPS cells are essentially integration-free.
Files and images showing reprogramming vector expressed or silenced

Vector free reprogramming

Other

Selection criteria for clones iPSCs from a patient affected by Wolfram Syndrome were generated from fibroblast cells of 13-years old female (GM01610, deposited at Coriell Institute repository). These cells were reprogrammed with the use of EBV-derived oriP/EBNA-1 episomal system carrying Oct3/4, Sox2, Klf4, Lin28 transcription factors, and shRNA against p53 (pCXLE-hOCT3/4-shp53-F, pCXLE-hSK pCXLE-hUL and pCXWB-EBNA1). One of the clones of reprogrammed cells, showing ESC-like morphology was further expanded and characterized.
Derived under xeno-free conditions
No
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 21 %
CO2 Concentration 5 %
Medium Essential 8™
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?
Yes
Has Rock inhibitor (Y27632) been used at thaw previously with this cell line?
Yes

Characterisation#

Analysis of Undifferentiated Cells
Marker Expressed Immunostaining RT-PCR FACS Enzymatic Assay Expression Profiles
SOX2
Yes
NANOG
Yes
TRA 1-60
Yes
TRA 1-81
Yes
POU5F1 (OCT-4)
Yes
Morphology pictures
Embryoid Bodies
Colony of IMBPASi001-A iPS cells
Differentiation Potency
Endoderm
Ont Id: UBERON_0000925
In vitro directed differentiation
Marker Expressed
CXCR4
Yes
Morphology
Clusters of CXCR4-positive cells in IMBPASi001-A differentiated into Definitive Endoderm.
Smooth Muscle Cell
Ont Id: CL_0000192
In vitro spontaneous differentiation
Marker Expressed
ACTA2
Yes
Morphology
ACTA2 expression in differentiated IMBPASi001-A cells
Ectoderm
Ont Id: UBERON_0000924
In vitro spontaneous differentiation
Marker Expressed
TUJ1
Yes
Morphology
TUJ1 expression in differentiated IMBPASi001-A cells
Neuron
Ont Id: CL_0000540
In vitro spontaneous differentiation
Morphology
Neurons
Neuroepithelial Stem Cell
Ont Id: CL_0002259
In vitro spontaneous differentiation
Morphology
Neural rosettes

Microbiology / Virus Screening

Mycoplasma Negative

Genotyping#

Karyotyping (Cell Line)

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

Other Genotyping (Cell Line)