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Stem Cells

HighQC™ Human IPSC From B-Lymphocyte-Ceroid Lipofuscinosis(Neuronal 2-Late Infantile Type)

  • For research use only

Cat No.

ABC-SC2068

Product Type

Human iPSCs

Cell Type

Induced Pluripotent Stem Cell

Species

Human

Growth Conditions

37 ℃, 5% CO2

Source Organ

B-Lymphocyte

Disease

Ceroid Lipofuscinosis

Storage

Liquid Nitrogen

HighQC™ Human IPSC From B-Lymphocyte-Ceroid Lipofuscinosis to model CLN2-related neurodegeneration and support drug discovery for lysosomal disorders.

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Description

HighQC™ Human IPSC From B-Lymphocyte-Ceroid Lipofuscinosis (Neuronal 2-Late Infantile Type) is a disease-specific induced pluripotent stem cell line generated from B-lymphocytes isolated from the peripheral blood mononuclear cells (PBMCs) of a donor diagnosed with Neuronal Ceroid Lipofuscinosis Type 2 (CLN2), a rare neurodegenerative lysosomal storage disorder. The resulting CLN2 disease iPSCs (induced pluripotent stem cells) exhibit adherent growth with a high nucleus-to-cytoplasm ratio. They preserve pluripotency and show CLN2-related cellular traits. These cells maintain the fundamental capacity for trilineage differentiation into ectoderm, mesoderm, and endoderm derivatives. Core pluripotency marker SSEA-4 is expressed. 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 B-Lymphocyte-Ceroid Lipofuscinosis (Neuronal 2-Late Infantile Type), HighQC™ hiPSC NCL2, hiPSC-CLN2, HighQC™ Human Induced Pluripotent Stem Cells From B-Lymphocyte-Neuronal Ceroid Lipofuscinosis Type 2

Species

Human

Cat.No

ABC-SC2068

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

B-Lymphocyte

Disease

Ceroid Lipofuscinosis

Storage

Liquid Nitrogen

Product Type

Human iPSCs

Application

  • HighQC™ Human IPSC From B-Lymphocyte-Ceroid Lipofuscinosis (Neuronal 2-Late Infantile Type) provides a patient-specific model for studying this pediatric neurodegenerative disorder. Its pluripotent capacity enables directed differentiation into neuronal lineages, particularly cortical neurons and glial cells affected by TPP1 enzyme deficiency. This system is used to model lysosomal dysfunction, investigate mechanisms of neurodegeneration and neuronal ceroid lipofuscin accumulation, and perform compound screening in neural organoids to identify potential therapeutic strategies.

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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