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

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)
Last update 25th June 2018
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Provider

Generator Universitätsklinikum Bonn (UKB)
Owner Universitätsklinikum Bonn
Distributors

External Databases

BioSamples SAMEA2590882
Cellosaurus CVCL_W575
ECACC 66540003
EBiSC UKBi008-A

General Information

Publications
Projects
Is the cell line available in principle?
Yes

Donor Information#

General Donor Information

Sex male
Ethnicity Caucasian

Phenotype and Disease related information (Donor)

Diseases A disease was diagnosed.
Spinocerebellar ataxia type 3
The donor is 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
Disease associated phenotypes
  • Symptomatic, 15 years post disease onset.
Family history de novo mutation

External Databases (Donor)

BioSamples SAMEA2590985

Ethics#

We are currently revising this section.

hIPSC Derivation#

General

Source cell type
fibroblast of dermis
Source cell origin
zone of skin
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
  • skin zone
  • region of skin
  • skin region
  • skin
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#

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 FACS 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
Hepatitis B Negative
Hepatitis C Negative
Mycoplasma Negative

Genotyping#

Karyotyping (Cell Line)

Has the cell line karyotype been analysed?
Yes
Karyotyping method: Molecular karyotyping by SNP array

Other Genotyping (Cell Line)

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