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HighQC™ Human IPSC From Fibroblast (Niemann-Pick Disease, Type A Sphingomyelin Phosphodiesterase 1, Acid Lysosomal)

  • For research use only

Cat No.

ABC-SC2037
Product Type

Human iPSCs
Cell Type

Induced Pluripotent Stem Cell
Species

Human
Growth Conditions

37 ℃, 5% CO2
Source Organ

iPSC
Disease

Niemann-Pick Disease, Type A
Storage

Liquid Nitrogen

HighQC™ Human IPSC From Fibroblast (Niemann-Pick Disease Type A) supports sphingolipid metabolism and SMPD1-related lysosomal storage disorder modeling.

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  • HighQC™ Human IPSC From Fibroblast (Niemann-Pick Disease, Type A Sphingomyelin Phosphodiesterase 1, Acid Lysosomal)
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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 A Sphingomyelin Phosphodiesterase 1, Acid Lysosomal)

Description

HighQC™ Human IPSCs From Fibroblasts (Niemann-Pick Disease, Type A Sphingomyelin Phosphodiesterase 1, Acid Lysosomal) are generated from human skin fibroblasts obtained from an individual affected by Niemann-Pick disease type A, a heritable lysosomal storage disorder caused by deficiency of acid sphingomyelinase encoded by the Sphingomyelin Phosphodiesterase 1 gene. These cells display tight colony boundaries, high nuclear-to-cytoplasmic ratios, and clonal growth patterns. Reprogramming was performed using a non-integrating approach, which transiently expresses key human transcription factors to initiate the reprogramming process. 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 A, SMPD1), HighQC™ hiPSC NPA, hiPSC-NPA, HighQC™ Human Induced Pluripotent Stem Cells From Fibroblast-Niemann-Pick Disease Type A
Species

Human
Cat.No

ABC-SC2037
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

iPSC
Disease

Niemann-Pick Disease, Type A
Storage

Liquid Nitrogen
Product Type

Human iPSCs
Gene Info

Gene: SMPD1; Mutation: 1-BP DEL,PRO33FS

Application

  • HighQC™ Human IPSCs From Fibroblasts (Niemann-Pick Disease, Type A Sphingomyelin Phosphodiesterase 1, Acid Lysosomal), also known as Niemann-Pick Type A iPSCs, provide a robust platform for investigating lysosomal dysfunction and sphingolipid metabolism in a disease-relevant context. With strong pluripotency and developmental potential, these iPSCs can be directed to differentiate into multiple cell types implicated in Niemann-Pick disease type A pathology, including neurons, hepatocytes, and macrophage-like cells, supporting studies of tissue-specific vulnerability and cellular lipid accumulation.

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

  • What is the similarities and differences between Niemann-Pick Disease, Type A and Type B?

    Both Type A and Type B Niemann-Pick Disease are lysosomal storage disorders (LSDs) caused by mutations in the SMPD1 gene, resulting in a deficiency in acid sphingomyelinase (ASM) function. This deficiency leads to the abnormal accumulation of sphingomyelin in lysosomes.

    Type B: Caused by SMPD1 mutations, but it presents a partial deficiency of the enzyme. The disease primarily affects the non-CNS tissues, and patients generally experience a slower progression compared to Type A. Type B is suitable for studying lipid storage and metabolic disorders, but the Type A model remains preferred for studying classical LSD mechanisms.

    Type A: Also caused by mutations in the SMPD1 gene resulting in a severe deficiency of acid sphingomyelinase. Patients typically experience early onset, rapid progression, and severe central nervous system (CNS) involvement, with significant sphingomyelin accumulation in various cell types. This aligns with the clinical and cytological features of classic lysosomal storage disorders. If your research focuses on studying the pathological mechanisms of LSDs or drug screening, the Type A iPSC model is recommended.

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