HEL13.1, ALS50

General

Cell Line

hPSCreg name UHi002-A
Cite as:
UHi002-A (RRID:CVCL_RL11)
Alternative name(s)
HEL13.1, ALS50
Cell line type Human induced pluripotent stem cell (hiPSC)
Similar lines
UHi003-A
(ALS75, HEL15.14)
Donor diseases:
obsolete_amyotrophic lateral sclerosis
UCLi004-A
(RCi173, RCFB60c6)
Donor's gene variants:
C9orf72, C9orf72
Donor diseases:
Amyotrophic lateral sclerosis
Frontotemporal dementia
UCLi004-B
(RCFB60c7, RCi177)
Donor's gene variants:
C9orf72, C9orf72
Donor diseases:
Amyotrophic lateral sclerosis
Frontotemporal dementia
UCLi004-C
(RCi172, RCFB60c2)
Donor's gene variants:
C9orf72, C9orf72
Donor diseases:
Amyotrophic lateral sclerosis
Frontotemporal dementia
PFIZi013-A
(RCi215, RCFB59 C9)
Donor's gene variants:
TARDBP, TARDBP
Donor diseases:
Amyotrophic lateral sclerosis
HVRDi009-A
(29e SOD1 L144F, 29e)
Donor diseases:
obsolete_amyotrophic lateral sclerosis
NIMHi008-A
(NphyiALS1)
Donor's gene variants:
LDB3
Donor diseases:
Amyotrophic Lateral Sclerosis
UCSCi001-A
(SII-1802)
Donor diseases:
Amyotrophic Lateral Sclerosis
SAPi003-A
(ALS II, FUS-R521C/wt, ALS II–FUS-R521C/wt)
Donor diseases:
Amyotrophic lateral sclerosis
SAPi004-A
(ALS III, TDP43-A382T/A382T, ALS III-TDP43-A382T/A382T)
Donor diseases:
Amyotrophic lateral sclerosis
CSSi012-A
Donor's gene variants:
TARDBP
Donor diseases:
Amyotrophic lateral sclerosis
SAPi002-A
(ALS I, ALS I–FUS-R514S/wt, FUS-R514S/wt)
Donor diseases:
amyotrophic lateral sclerosis
STBCi071-C
(SFC057-07-02)
Donor diseases:
Alzheimer disease
STBCi090-B
(SFC867-04-12)
Donor diseases:
Parkinson disease
CHDIi002-A
(#2c3, CHDI-90002150)
Donor's gene variants:
HTT, HTT
Donor diseases:
Huntington disease
CHDIi006-A
(#6c7, CHDI-90002154)
Donor's gene variants:
HTT, HTT
Donor diseases:
Huntington disease
CHDIi008-A
(#8c3, CHDI-90002156)
Donor's gene variants:
HTT, HTT
Donor diseases:
Huntington disease
CHDIi011-A
(#11c2, CHDI-90002159)
Donor's gene variants:
HTT, HTT
Donor diseases:
Huntington disease
CHDIi017-A
(#17c1, CHDI-90002165)
Donor's gene variants:
HTT, HTT
Donor diseases:
Huntington disease
CHDIi018-A
(#18c1, CHDI-90002166)
Donor's gene variants:
HTT, HTT
Donor diseases:
Huntington disease
CHDIi019-A
(#19c9, CHDI-90002167)
Donor's gene variants:
HTT, HTT
Donor diseases:
Huntington disease
CHDIi021-A
(#21c5, CHDI-90002169)
Donor's gene variants:
HTT, HTT
Donor diseases:
Huntington disease
CHDIi028-A
(#28c3, CHDI-90002176)
Donor's gene variants:
HTT, HTT
Donor diseases:
Huntington disease
CHDIi035-A
(#108c5, CHDI-90002183)
Donor's gene variants:
HTT, HTT
Donor diseases:
Huntington disease
CHDIi036-A
(#110c5, CHDI-90002184)
Donor's gene variants:
HTT, HTT
Donor diseases:
Huntington disease
CHDIi038-A
(#112c7, CHDI-90002186)
Donor's gene variants:
HTT, HTT
Donor diseases:
Huntington disease
CHDIi040-A
(#115c6, CHDI-90002188)
Donor's gene variants:
HTT, HTT
Donor diseases:
Huntington disease
CHDIi045-A
(#121c6, CHDI-90002193)
Donor's gene variants:
HTT, HTT
Donor diseases:
Huntington disease
CHDIi046-A
(#122c1, CHDI-90002194)
Donor's gene variants:
HTT, HTT
Donor diseases:
Huntington disease
STBCi005-B
(SFC833-03-05)
Donor's gene variants:
LRRK2
Donor diseases:
Parkinson disease
STBCi005-C
(SFC833-03-14)
Donor's gene variants:
LRRK2
Donor diseases:
Parkinson disease
STBCi097-A
(SFC055-04-02)
Donor diseases:
Alzheimer disease
STBCi097-B
(SFC055-04-01)
Donor diseases:
Alzheimer disease
STBCi097-C
(SFC055-04-03)
Donor diseases:
Alzheimer disease
STBCi007-B
(SFC855-03-08)
Donor's gene variants:
LRRK2
Donor diseases:
Parkinson disease
STBCi099-A
(SFC059-03-01)
Donor diseases:
Alzheimer disease
Last update 14th May 2022
Notes Derived at Biomedicum Stem Cell Center, University of Helsinki, Finland.
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Provider

Generator University of Helsinki (UH)
Derivation country Finland

External Databases

BioSamples SAMEA5236073
Cellosaurus CVCL_RL11
Wikidata Q54749292

General Information

Publications
* 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
  • Bulbar motor neuron disease
  • Lateral Scleroses, Amyotrophic
  • Amyotrophic Lateral Sclerosis, Guam Form
  • Amyotrophic lateral sclerosis, Parkinsonism/Dementia complex of Guam
  • Amyotrophic lateral sclerosis (disorder)
  • ALS (Amyotrophic Lateral Sclerosis)
  • LOU GEHRIG DIS
  • ALS
  • Gehrig's Disease
  • Dementia With Amyotrophic Lateral Sclerosis
  • Lou-Gehrigs Disease
  • Gehrigs Disease
  • AMYOTROPHIC SCLEROSIS
  • GEHRIGS DIS
  • ALS - Amyotrophic lateral sclerosis
  • Sclerosis, Amyotrophic Lateral
  • Guam Form of Amyotrophic Lateral Sclerosis
  • Disease, Lou-Gehrigs
  • Motor Neuron Disease, Amyotrophic Lateral Sclerosis
  • Lou Gehrigs Disease
  • MOTOR NEURON DIS AMYOTROPHIC LATERAL SCLEROSIS
  • Amyotrophic Lateral Sclerosis With Dementia
  • Amyotrophic Lateral Sclerosis-Parkinsonism/dementia Complex 1
  • LOU GEHRIGS DIS
  • Gehrig Disease
  • Charcot disease
  • Lou Gehrig's Disease
  • Lou Gehrig Disease
  • Motor neuron disease, bulbar
show more synonyms

External Databases (Donor)

BioSamples SAMEA5236092

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. Prof. Pentti Tienari, Helsinki University hospital (pentti.tienari@hus.fi)
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 Prof. Pentti Tienari, Helsinki University hospital (pentti.tienari@hus.fi)
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 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? Ethics Committee of Helsinki and Uusimaa Hospital District
Approval number Dnro 106/2010
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
A connective tissue cell which secretes an extracellular matrix rich in collagen and other macromolecules. Flattened and irregular in outline with branching processes; appear fusiform or spindle-shaped.; These cells may be vimentin-positive, fibronectin-positive, fsp1-positive, MMP-1-positive, collagen I-positive, collagen III-positive, and alpha-SMA-negative.
Source cell origin
Any portion of the organ that covers that body and consists of a layer of epidermis and a layer of dermis.
Synonyms
  • portion of skin
  • region of skin
  • skin
  • skin region
  • skin zone
show more synonyms

Reprogramming method

Vector type Integrating
Vector Virus (Retrovirus)
Genes
Is the used vector excisable?
Unknown
Absence of reprogramming vector(s)?
Unknown
Reprogramming vectors silenced?

Vector free reprogramming

Other

Derived under xeno-free conditions
Unknown
Derived under GMP?
Unknown
Available as clinical grade?
Unknown

Culture Conditions

Medium Essential 8™

Characterisation

Analysis of Undifferentiated Cells
Marker Expressed Immunostaining RT-PCR Flow Cytometry Enzymatic Assay Expression Profiles
TRA 1-60
Yes
NANOG
Yes
POU5F1 (OCT-4)
Yes
SSEA-3
Yes
Differentiation Potency
Endoderm
Ont Id: UBERON_0000925
In vitro spontaneous differentiation
Marker Expressed
AFP – alpha fetoprotein
Yes
Mesoderm
Ont Id: UBERON_0000926
In vitro spontaneous differentiation
Marker Expressed
SMA - Alpha smooth muscle actin
Yes
Neuron
Ont Id: CL_0000540
In vitro directed differentiation
Marker Expressed
Beta 3 tubulin
Yes
Ectoderm
Ont Id: UBERON_0000924
In vitro spontaneous differentiation
Marker Expressed
OTX2 - orthodenticle homeobox 2
Yes

Genotyping

Karyotyping (Cell Line)

Has the cell line karyotype been analysed?
No

Other Genotyping (Cell Line)