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UKBi008-A

iLB-MJD4-34m-r1, LB-34-1

iPSC line

Stock not immediately available - enquire for details
We are currently making some changes to how EBiSC operates and because of this there is a short period of time where orders cannot be placed.

If you are interested in accessing these cells, please contact EBiSC directly. For more information about the current transition process see here.
Timepoint: 48 hours post-passage
Magnification: 4x
Timepoint: 48 hours post-passage
Magnification: 10x
Timepoint: Confluence
Magnification: 4x
Timepoint: Confluence
Magnification: 10x
A CLIP contains information about a cell line including any specific third party obligations relating to, for example, licensing obligations or the donor consent which affect the use of the cell line.

The EBiSC Access and Use Agreement must be completed along with an individual Cell Line Information Pack for each line. Complete the EAUA and send to Contact@EBiSC.org for countersignature. The EAUA must be fully signed before proceeding with your order.
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General#

Cell Line

hPSCreg name UKBi008-A
Alternative name(s)
iLB-MJD4-34m-r1, LB-34-1
Cell line type Human induced pluripotent stem cell (hiPSC)
Similar lines
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ATXN3, ATXN3
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Spinocerebellar ataxia type 3
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Provider

Depositor Universitätsklinikum Bonn (UKB)
Owner Universitätsklinikum Bonn
Distributors
EBiSC
Institut für Rekonstruktive Neurobiologie
Scottish Centre for Regenerative Medicine

External Databases

hPSCreg UKBi008-A
BioSamples SAMEA2590882
Cellosaurus CVCL_W575
Wikidata Q54905794

General Information

Publications
This EBiSC line can be used for:
Yes
Research use: allowed
Clinical use: no
Commercial use: no

Donor Information#

General Donor Information

Sex male
Age of donor (at collection) 40-44
Ethnicity Caucasian

Phenotype and Disease related information (Donor)

Diseases A disease was diagnosed.
The donor is a carrier of a disease-associated mutation and affected.
Stage
Scores on the Scale for the Assessment and Rating of Ataxia (SARA, range: 0 – 40) was 10.5.
Synonyms
  • Nigro-spino-dentatal degeneration with nuclear ophthalmoplegia
  • Machado-Joseph disease
  • SCA3
  • MJD
  • Machado disease
  • Azorean disease of the nervous system
show more synonyms
Genetic variants
ATXN3 (target)
14q32.12
NM_004993​.5:c.886_888CAG[73_20]
NP_004984​.2:p.Gln296[73_20]
Heterozygous
PMID: 22113611
ATXN3: CAG repeat length of 73/20
Disease associated phenotypes
  • Symptomatic, 15 years post disease onset.
Family history de novo mutation

External Databases (Donor)

BioSamples SAMEA2590985

hIPSC Derivation#

General

Source cell type
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
Age of donor (at collection) 40-44
Collected in 2008
Passage number reprogrammed P4

Reprogramming method

Vector type Integrating
Vector Virus (Retrovirus)
Genes
Is the used vector excisable?
No
Absence of reprogramming vector(s)?
Yes
Reprogramming vectors silenced?
Yes
Methods used
PCR
Files and images showing reprogramming vector expressed or silenced

Vector free reprogramming

Type of used vector free reprogramming factor(s)
None

Other

Selection criteria for clones Morphology
Derived under xeno-free conditions
No
Derived under GMP?
No
Available as clinical grade?
No

Culture Conditions#

Latest released batch

Culture medium mTeSR
Passage method EDTA
Surface coating Matrigel / Geltrex
O2 concentration 21
CO2 concentration 5
Temperature 37
The following are the depositor culture conditions, they do not refer to any specific batch.
Surface coating Matrigel/Geltrex
Feeder cells
No
Passage method Enzyme-free cell dissociation
EDTA
O2 Concentration 21 %
CO2 Concentration 5 %
Medium mTeSR™ 1
Has Rock inhibitor (Y27632) been used at passage previously with this cell line?
No
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?
No

Characterisation#

Analysis of Undifferentiated Cells
Marker Expressed Immunostaining RT-PCR Flow Cytometry Enzymatic Assay Expression Profiles
POU5F1 (OCT-4)
Yes
SSEA-3
Yes
TRA 1-60
Yes
TRA 1-81
Yes
Morphology pictures
Differentiation Potency
Endoderm
Ont Id: UBERON_0000925
In vivo teratoma
Morphology
Mesoderm
Ont Id: UBERON_0000926
In vivo teratoma
Morphology
Neuroepithelial Stem Cell
Ont Id: CL_0002259
In vivo teratoma
In vitro directed differentiation
Morphology

Microbiology / Virus Screening

HIV 1 Negative
HIV 2 Negative
Hepatitis B Negative
Hepatitis C Negative
Mycoplasma Negative

Genotyping#

Karyotyping (Cell Line)

Has the cell line karyotype been analysed?
Yes
XY
Passage number: 26
Karyotyping method: Molecular karyotyping by SNP array
http://

Other Genotyping (Cell Line)

Is there genome-wide genotyping or functional data available?
Yes