General

Cell Line
hPSCreg name	INEUi007-A-1
Cite as: When citing this cell line, please use the hPSCreg name (see Naming Tool ) and the corresponding Research Resource ID (RRID).	INEUi007-A-1
Alternative name(s)	IV-B-HO hiPSC line, DESY-HO
Cell line type	Human induced pluripotent stem cell (hiPSC)
Similar lines	No similar lines found.
Last update	17th March 2026
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Provider
Generator	Instituto de Neurociencias Conicet (INEU) Contact: Instituto de Neurociencias Conicet (INEU)
Owner	Instituto de Neurociencias Conicet (INEU)
Distributors	Instituto de Neurociencias Conicet (INEU)
Derivation country	Argentina
External Databases
BioSamples	SAMEA121974448
General Information
* Is the cell line readily obtainable for third parties?	No
Subclone of	INEUi007-A

Donor Information

General Donor Information
Sex	male
Ethnicity	Lebanese
Phenotype and Disease related information (Donor)
Diseases	A disease was diagnosed. Desminopathy The donor is a carrier of a disease-associated mutation and affected. Synonyms Desmin-related myofibrillar myopathy Genetic variants Nucleotide sequence variant in HGVS format NM_001927.4:c.1059_1061dup Protein sequence variant in HGVS format NP_001918.3:p.(Glu353dup) Is the variant homozygous or heterozygous? Heterozygous ClinVar SCV004565348 Publication Pubmed ID https://doi.org/10.1136/jmg-2025-110852
Family history	https://doi.org/10.1136/jmg-2025-110852
Is the medical history available upon request?	Yes
Is clinical information available?	Yes
Other Genotyping (Donor)
Is there genome-wide genotyping or functional data available?	No
Donor Relations
All cell lines of this donor's relatives	Has sister: INEUi006-A Has brother: INEUi008-A
External Databases (Donor)
BioSamples	SAMEA121971888

Ethics

Also have a look at the ethics information for the parental line INEUi007-A .

For generation of the cell line, who was the supplier of any recombined DNA vectors or commercial kits used?

hIPSC Derivation

General
The source cell information can be found in the parental cell line INEUi007-A.
Reprogramming method
Vector type	Integrating
Vector	Virus (Lentivirus)
Genes	POU5F1 KLF4 SOX2 MYC
Is the used vector excisable?	Yes
Absence of reprogramming vector(s)?	No
Reprogramming vectors silenced?	Yes
Methods used	RT-PCR
Notes on reprogramming vector silencing	STEMCCA lentivirus silencing was verified in the parental hiPSC line INEUi007-A.
Vector free reprogramming
Other
Derived under xeno-free conditions	Unknown
Derived under GMP?	Unknown
Available as clinical grade?	Unknown

Culture Conditions

Surface coating	Matrigel/Geltrex
Feeder cells	No
Passage method	Enzyme-free cell dissociation EDTA
CO2 Concentration	5 %
Medium	Other medium: Base medium: Gibco StemFlex Medium Main protein source: Serum concentration: %
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 Expression

Marker	Expressed	Immunostaining	RT-PCR	Flow Cytometry	Enzymatic Assay	Expression Profiles
POU5F1 (OCT-4)	Yes	–

Genotyping

Karyotyping (Cell Line)
Has the cell line karyotype been analysed?	Yes 46,XY DESY-HO_40_L1_5K.tif Passage number: 25 Karyotyping method: G-Banding
Other Genotyping (Cell Line)

Genetic Modification

Disease/phenotype related modifications

Desminopathy Synonyms Desmin-related myofibrillar myopathy Genetic modifications Type of modification Isogenic modification Nucleotide sequence variant in HGVS format NM_001927.4:c.1059_1061dup Protein sequence variant in HGVS format NP_001918.3:p.(Glu353dup) Is the modification homozygous or heterozygous? Homozygous How has the target locus been modified? The wild type allele was modified to have the variant NM_001927.4:c.1059_1061dup and obtain an hiPSC line homozygous for the variant NM_001927.4:c.1059_1061dup.