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JHUi007-A-1

Registration Summary:

i0195 SubClone D8

The cell line is not validated yet.
JHUi007-A-1

General

Cell Line
hPSCreg name JHUi007-A-1
Cite as:
JHUi007-A-1
Alternative name(s)
i0195 SubClone D8
Cell line type Human induced pluripotent stem cell (hiPSC)
Similar lines No similar lines found.
Last update 3rd September 2025
Notes Isogenic, normal control for JHUi007-A
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Provider
Generator Johns Hopkins University (JHU)
Owner School of Medicine
Distributors
Derivation country United States

External Databases
BioSamples SAMEA118794523

General Information
* Is the cell line readily obtainable for third parties?
Yes
Research use: allowed
Clinical use: not allowed
Commercial use: not allowed
Subclone of

Donor Information

General Donor Information
Sex male
Ethnicity White/Caucasian

Phenotype and Disease related information (Donor)
Diseases A disease was diagnosed.
Ehlers-Danlos syndrome, vascular type
The donor is a carrier of a disease-associated mutation and affected.
Synonyms
  • EDS IV
  • EDS type 4
  • Ehlers-Danlos syndrome type 4
  • Ehlers-Danlos syndrome type IV
  • Ehlers-Danlos syndrome, type IV
  • Ehlers-Danlos syndrome, vascular type
  • sack-Barabas syndrome
  • EDS IV (formerly)
  • EDS type 4 (formerly)
  • EDS4 (formerly)
  • Ehlers Danlos syndrome, arterial type
  • Ehlers Danlos syndrome, ecchymotic type
  • Ehlers Danlos syndrome, sack-Barabas type
  • Ehlers-Danlos syndrome type 4 (formerly)
  • Ehlers-Danlos syndrome type IV (formerly)
  • vEDS
  • vascular EDS
  • vascular Ehlers-Danlos syndrome
show more synonyms
Genetic variants
Collagen, type III, alpha 1 (target)
2q32.2
NM_000090.4(COL3A1):c.755G>T (p.Gly252Val)
NP_000081.1:p.G252V
Heterozygous
Sanger sequencing confirmation of mutation in hiPSC lines
0195-col3a1.jpg
Sequencing data showing mutation in genomic sequences of i0195 hiPSC (CTL0195 represents the initial fibroblasts)
Family history Unknown
Is the medical history available upon request? No
Is clinical information available? No

Other Genotyping (Donor)
Is there genome-wide genotyping or functional data available?
No

External Databases (Donor)
BioSamples SAMEA118322522

Ethics

Also have a look at the ethics information for the parental line JHUi007-A .
Is there an MTA available for the cell line? No
For generation of the cell line, who was the supplier of any recombined DNA vectors or commercial kits used? Synthego was contracted to perform the gene editing of line JHUi007-A to generate this normal isogenic control line
Are you aware of any constraints on the use or distribution of the cell line from the owner or any parties identified in the query above? No

hIPSC Derivation

General
The source cell information can be found in the parental cell line JHUi007-A.

Reprogramming method

Vector free reprogramming

Other
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 Accutase, TrypLE or 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 Flow Cytometry Enzymatic Assay Expression Profiles
POU5F1 (OCT-4)
Yes
–
–
NANOG
Yes
–
–
SOX2
Yes
–
Morphology pictures
i0195 D8 BF 20x.tif
Brightfield image of colonies of this gene edited (normal) hiPSC line
Differentiation Potency
Endoderm
Ont Id: UBERON_0000925
In vitro directed differentiation
Marker Expressed
GATA4 zinc finger transcription factor
Yes
Sox17 Sry box transcription factor
Yes
Hepatocyte nuclear factor 3-alpha
Yes
Protocol or reference
i0195 D8 Endoderm.jpg
Graphic representation of qPCR results showing data for 3 endodermal markers
Mesoderm
Ont Id: UBERON_0000926
In vitro directed differentiation
Marker Expressed
Heart and Neural Crest Derivatives Expressed 1
Yes
Brachyury - T-box transcription factor T
Yes
NKX2-5 homeobox transcription factor
Yes
Protocol or reference
i1095 D8 Mesoderm.jpg
Graphic representation of qPCR results showing data for 3 mesodermal markers
Ectoderm
Ont Id: UBERON_0000924
In vitro directed differentiation
Marker Expressed
OTX2 homeobox
Yes
Pax6 paired box TF
Yes
Protocol or reference
i0195 D8 Ectoderm.jpg
Graphic representation of qPCR results showing data for 3 ectodermal markers

Microbiology / Virus Screening
Mycoplasma Negative

Genotyping

Karyotyping (Cell Line)
Has the cell line karyotype been analysed?
Yes
46, XY
i0195_Clone_D8-Karyostat.pdf
Passage number: 28
Karyotyping method: KaryoStat (ThermoFisher)

Other Genotyping (Cell Line)

Genetic Modification

Disease/phenotype related modifications
Vascular type Ehlers-Danlos syndrome
This line serves as an isogenic normal control line for JHUi007-A, which contains a gene mutation. This gene edited control is normal, and details are provided below, since this gene mutation should not lead to a disease phenotype
Synonyms
  • Ehlers-Danlos syndrome type 4
  • Ehlers-Danlos syndrome type IV
  • autosomal dominant type IV Ehlers-Danlos syndrome
Genetic modifications
Isogenic modification
Genetic modifications not related to a disease
Isogenic modification
2q32.2
NM_000090.4(COL3A1):c.755T>G (p.Val252Gly)
NP_000081.1:p.V252G
Homozygous
A COL3A1-G252V mutation present in JHUi007-A was corrected using CRISPR-Cas9 techniques to generate a wildtype (normal) COL3A1 gene with a COL3A1-V252G sequence: V252G (GTT>GGT)
Repaired