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Stem Cells

HighQC™ Human IPSC From Fibroblast-Long QT Syndrome 3

  • For research use only

Cat No.

ABC-SC2054

Product Type

Human iPSCs

Cell Type

Induced Pluripotent Stem Cell

Species

Human

Growth Conditions

37 ℃, 5% CO2

Source Organ

Fibroblast

Disease

Long QT Syndrome 3

Storage

Liquid Nitrogen

Utilize HighQC™ Human IPSC From Fibroblast-Long QT Syndrome 3 for cardiac electrophysiology research, arrhythmia modeling, and drug safety evaluation.

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Description

HighQC™ Human IPSC From Fibroblast-Long QT Syndrome 3 is a disease-specific induced pluripotent stem cell line derived from dermal fibroblasts of a donor diagnosed with Long QT Syndrome Type 3 (LQT3), a cardiac arrhythmia disorder primarily caused by mutations in the SCN5A gene. The resulting iPSCs (induced pluripotent stem cells) exhibit adherent growth with a high nucleus-to-cytoplasm ratio. The cells reflect LQT3 cardiac electrophysiology for mechanism research. They retain the fundamental capacity for trilineage differentiation into cell types representing the ectoderm, mesoderm, and endoderm germ layers under defined protocols. These cells express the core pluripotency marker SSEA-4. The cells undergo rigorous screening and isolation procedures, and are rigorously tested to ensure they are free of contamination from HIV-1, HBV, HCV, Syphilis, Mycoplasma, Fungi, Yeast, and Bacteria.

Product Code

HighQC™ Human IPSC From Fibroblast-Long QT Syndrome 3, HighQC™ hiPSC LQT3, hiPSC-LQT3, HighQC™ Human Induced Pluripotent Stem Cells From Fibroblast-Long QT Syndrome Type 3

Species

Human

Cat.No

ABC-SC2054

Product Category

Stem Cells

Size/Quantity

1 vial

Cell Type

Induced Pluripotent Stem Cell

Growth Mode

Adherent

Shipping Info

Dry Ice

Growth Conditions

37 ℃, 5% CO2

Source Organ

Fibroblast

Disease

Long QT Syndrome 3

Storage

Liquid Nitrogen

Product Type

Human iPSCs

Gene Info

Gene: SCN5A; Mutation: ASN406LYS

Application

  • HighQC™ Human IPSC From Fibroblast-Long QT Syndrome 3 provides a patient-specific in vitro model for cardiac developmental and disease research. Its pluripotent capacity supports directed differentiation into cardiomyocytes affected by SCN5A mutations. This system enables the study of cardiac action potential prolongation, late sodium current abnormalities, and arrhythmogenic mechanisms, while facilitating drug screening and toxicity evaluation in engineered cardiac tissues or organoid models for channelopathy investigations.

Citation

When you publish your research, please cite our product as "AcceGen Biotech Cat.# XXX-0000". In return, we’ll give you a $200 coupon. Simply click here and submit your paper’s PubMed ID (PMID).

Frequently Asked Questions

  • Are the mutations in this iPSC line heterozygous or homozygous, and what is the specific mutation information?

    Sequencing revealed a de novo missense mutation in exon 10 of the SCN5A gene (1218C>A), located at chromosomal region 3p22.2. The identified mutation is ASN406LYS (N406K), a novel pathogenic variant situated in the IS6 region of the transmembrane-spanning domain of the NaV1.5 cardiac sodium channel encoded by SCN5A.

Inquiring HighQC™ Human IPSC From Fibroblast-Long QT Syndrome 3

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High Viability
To succeed in cell culture
Precision and Reliability
To support a consistent result
Customization Options
Tailed to your research

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