RNRMUi001-A

RDEB-iPSC-d1

General

Cell Line

hPSCreg name RNRMUi001-A
Cite as:
RNRMUi001-A (RRID:CVCL_D0MQ)
Alternative name(s)
RDEB-iPSC-d1
Cell line type Human induced pluripotent stem cell (hiPSC)
Similar lines
ZZUi030-A
(ZZU-iPS-SPG7-001)
Donor diseases:
Spastic paraplegia type 7
UTSWi001-A
(FA1)
Donor diseases:
Friedreich Ataxia
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
UNIZARi001-A
(FiPSTK2-2)
Donor's gene variants:
TK2, TK2
Donor diseases:
Mitochondrial DNA depletion syndrome, myopathic form
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
BGUi001-A
(BGU01iPORhet)
Donor diseases:
P450 Oxidoreductase Deficiency
BGUi002-A
(BGU02iPOR)
Donor diseases:
P450 Oxidoreductase Deficiency
BGUi003-A
(BGU03iPOR)
Donor diseases:
P450 Oxidoreductase Deficiency
AIBNi015-A
(SPG1-AU01C15)
Donor diseases:
hereditary spastic paraplegia 56
UKBi014-A
(A-257s2)
Donor diseases:
Walker-Warburg syndrome
VUi011-A
(SCZ 3.5)
Donor diseases:
Schizophrenia
DHMi004-A
(HOS_1460)
Donor diseases:
Holt-Oram Syndrome
IDIBGIi003-A
(​RB20235)
Donor diseases:
Brugada syndrome
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
TNRMCi001-A
(iTAF32)
Donor's gene variants:
CFTR
Donor diseases:
Cystic fibrosis
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
Last update 16th January 2024
User feedback
No feedback available yet.

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

Provider

Generator Pirogov Russian National Research Medical University (RNRMU)
Owner Pirogov Russian National Research Medical University (RNRMU)
Derivation country Russia

External Databases

BioSamples SAMEA113934129
Cellosaurus CVCL_D0MQ
Wikidata Q123033361

General Information

* 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
Age of donor (at collection) 5-9
Ethnicity Caucasian

Phenotype and Disease related information (Donor)

Diseases A disease was diagnosed.
ORPHA 79408
The donor is a carrier of a disease-associated mutation and affected.
Synonyms
  • Recessive Dystrophic Epidermolysis Bullosa
  • RDEB
  • Epidermolysis Bullosa Dystrophica, Autosomal Recessive

Karyotyping (Donor)

Has the donor karyotype been analysed?
Yes

Other Genotyping (Donor)

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

External Databases (Donor)

BioSamples SAMEA113934130

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
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? 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
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? Local ethic commission of Pirogov RSMU
Approval number №192 from 01.27.2019
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
Age of donor (at collection) 5-9
Collected in 2019

Reprogramming method

Vector type Non-integrating
Vector Sendai virus

Vector free reprogramming

Other

Selection criteria for clones Phase contrast microscopy - large nucleus and densely packed cells grow in colonies. Immunofluorescence - expression of SOX2, OCT4, SSEA-4, TRA-1-60 and TRA-1-81. RT-PCR - expression of LIN28, OCT4, NANOG, REX12, TDGF and SOX2.
Derived under xeno-free conditions
Unknown
Derived under GMP?
Yes
Available as clinical grade?
Unknown

Culture Conditions

Surface coating Matrigel/Geltrex
Feeder cells
No
Passage method Enzymatically
Accutase
CO2 Concentration 5 %
Medium mTeSR™ Plus
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 Flow Cytometry Enzymatic Assay Expression Profiles
SOX2
Yes
SSEA-4
Yes
TRA 1-60
Yes
TRA 1-81
Yes
Differentiation Potency
Endoderm
Ont Id: UBERON_0000925
In vitro spontaneous differentiation
Marker Expressed
sox17
Yes
Mesoderm
Ont Id: UBERON_0000926
In vitro spontaneous differentiation
Marker Expressed
desmin
Yes
Ectoderm
Ont Id: UBERON_0000924
In vitro spontaneous differentiation
Marker Expressed
SOX2
Yes

Microbiology / Virus Screening

Mycoplasma Negative

Genotyping

Karyotyping (Cell Line)

Has the cell line karyotype been analysed?
Yes

Other Genotyping (Cell Line)