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HighQC™ Human IPSC From Fibroblast-Niemann-Pick Disease, Type C1

  • For research use only

Cat No.

ABC-SC2038
Product Type

Human iPSCs
Cell Type

Induced Pluripotent Stem Cell
Species

Human
Growth Conditions

37 ℃, 5% CO2
Source Organ

Fibroblast
Disease

Niemann-Pick Disease, Type C1
Storage

Liquid Nitrogen

Cell Type: iPSC; Disease: Niemann-Pick Disease, Type C1; NPC1 NPC1 GENE; NPC1.

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  • HighQC™ Human IPSC From Fibroblast-Niemann-Pick Disease, Type C1
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Home | Stem Cells | Induced Pluripotent Stem Cells (iPSCs) | Induced Pluripotent Stem Cells (iPSCs) | HighQC™ Human IPSC From Fibroblast-Niemann-Pick Disease, Type C1

Description

HighQC™ Human IPSCs From Fibroblasts-Niemann-Pick Disease, Type C1 are a disease-specific induced pluripotent stem cell line generated from human dermal fibroblasts carrying pathogenic alterations in the Niemann-Pick disease type C1 gene, a key regulator of intracellular cholesterol and lipid trafficking associated with Niemann-Pick disease type C1, a hereditary lysosomal storage disorder. The parental fibroblasts are reprogrammed into a pluripotent state using Sendai virus–mediated delivery of defined transcription factors. These iPSCs exhibit pluripotency, with the ability to differentiate into all three germ layers under defined conditions, and express the 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-Niemann-Pick Disease Type C1, HighQC™ hiPSC NPC1, hiPSC-NPC1, HighQC™ Human Induced Pluripotent Stem Cells From Fibroblast-Niemann-Pick Disease Type C1
Species

Human
Cat.No

ABC-SC2038
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

Niemann-Pick Disease, Type C1
Storage

Liquid Nitrogen
Product Type

Human iPSCs
Gene Info

Gene: NPC1; Mutation: ILE1061THR PRO237SER

Application

  • HighQC™ Human IPSCs From Fibroblasts-Niemann-Pick Disease, Type C1, also known as Niemann-Pick Type C1 iPSCs, serve as a robust in vitro research model for investigating the molecular and cellular mechanisms underlying lysosomal lipid trafficking dysfunction caused by NPC1 deficiency. This iPSC line enables the differentiation into disease-relevant cell types to study intracellular cholesterol accumulation, endolysosomal transport pathways, and lipid homeostasis at a mechanistic level. It is well suited for exploring NPC1-associated gene regulation, protein function, and downstream signaling networks, as well as for establishing genotype–phenotype correlations in lysosomal storage disorders.

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).

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