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HighQC™ Human IPSC From Fibroblast-Congenital Disorder Of Deglycosylation

  • For research use only

Cat No.

ABC-SC2064
Product Type

Human iPSCs
Cell Type

Induced Pluripotent Stem Cell
Species

Human
Growth Conditions

37 ℃, 5% CO2
Source Organ

Fibroblast
Disease

Congenital Disorder Of Deglycosylation
Storage

Liquid Nitrogen

Employ HighQC™ Human IPSC From Fibroblast-Congenital Disorder Of Deglycosylation for rare genetic disease modeling, drug testing, and molecular studies.

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  • HighQC™ Human IPSC From Fibroblast-Congenital Disorder Of Deglycosylation
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Home | Stem Cells | Induced Pluripotent Stem Cells (iPSCs) | Induced Pluripotent Stem Cells (iPSCs) | HighQC™ Human IPSC From Fibroblast-Congenital Disorder Of Deglycosylation

Description

HighQC™ Human IPSC From Fibroblast-Congenital Disorder of Deglycosylation is a disease-specific human induced pluripotent stem cell line derived from dermal fibroblasts of a donor diagnosed with a Congenital Disorder of Deglycosylation (CDDG), a group of rare inherited metabolic diseases characterized by defects in glycoprotein synthesis. The resulting iPSCs (induced pluripotent stem cells) exhibit adherent growth with a high nucleus-to-cytoplasm ratio. They maintain pluripotency and model CDDG-related glycosylation traits. These cells retain the fundamental capacity for trilineage differentiation into cell types of the ectoderm, mesoderm, and endoderm germ layers. They 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-Congenital Disorder Of Deglycosylation, HighQC™ hiPSC CDG, hiPSC-CDG, HighQC™ Human Induced Pluripotent Stem Cells From Fibroblast-Congenital Disorder Of Deglycosylation
Species

Human
Cat.No

ABC-SC2064
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

Congenital Disorder Of Deglycosylation
Storage

Liquid Nitrogen
Product Type

Human iPSCs
Gene Info

CACNA1S NGLY1

Application

  • HighQC™ Human IPSC From Fibroblast-Congenital Disorder of Deglycosylation provides a patient-specific model for in vitro studies of glycoprotein metabolism and disease pathogenesis. Its pluripotent capacity enables directed differentiation into diverse lineages such as hepatocytes, cardiomyocytes, and neuronal cells to investigate the cellular and tissue-specific consequences of glycosylation defects. This system is used to model disease mechanisms, study early developmental pathways affected by protein glycosylation, and serve as a platform for compound screening to identify potential therapeutic agents targeting this metabolic disorder.

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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