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Stem Cells

HighQC™ Human IPSC From Fibroblast-Krabbe Disease-Galactosylceramidase

  • For research use only

Cat No.

ABC-SC2052

Product Type

Human iPSCs

Cell Type

Induced Pluripotent Stem Cell

Species

Human

Growth Conditions

37 ℃, 5% CO2

Source Organ

Fibroblast

Disease

Krabbe Disease

Storage

Liquid Nitrogen

Cell Type: iPSC; Disease: Krabbe Disease, Galactosylceramidase; GALC.

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Description

HighQC™ Human IPSC From Fibroblast-Krabbe Disease-Galactosylceramidase
is a disease-specific induced pluripotent stem cell line generated from dermal fibroblasts of a donor diagnosed with Krabbe Disease (Globoid Cell Leukodystrophy), a lysosomal storage disorder caused by mutations in the GALC gene. The resulting iPSCs (induced pluripotent stem cells) exhibit adherent growth with a high nucleus-to-cytoplasm ratio. These cells maintain the fundamental pluripotent capacity for trilineage differentiation into ectoderm, mesoderm, and endoderm derivatives, harboring galactosylceramidase (GALC) deficiency traits and recapitulating in vivo neural myelin metabolism. They express the core pluripotency marker SSEA-4 and support disease modeling, mechanistic studies, and target identification for Krabbe disease. 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-Krabbe Disease, HighQC™ hiPSC Krabbe, hiPSC-Krabbe, HighQC™ Human Induced Pluripotent Stem Cells From Fibroblast-Krabbe Disease

Species

Human

Cat.No

ABC-SC2052

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

Krabbe Disease

Storage

Liquid Nitrogen

Product Type

Human iPSCs

Gene Info

Gene: GALC; Mutation: 30-KB DEL,IVS10

Application

  • HighQC™ Human IPSC From Fibroblast-Krabbe Disease-Galactosylceramidase provides a genetically relevant model for studying this lysosomal storage disorder. Its pluripotent capacity enables directed differentiation into oligodendrocytes and astrocytes, the primary cell types affected by GALC deficiency. This system is used to model demyelination pathology, investigate lipid metabolism dysfunction, and perform drug screening in neural organoids, facilitating research into glial cell development and therapeutic strategies for neurodegenerative lysosomal diseases.

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).

Inquiring HighQC™ Human IPSC From Fibroblast-Krabbe Disease-Galactosylceramidase

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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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