HighQC™ Human IPSC From Fibroblast-Lesch-Nyhan Syndrome

Description

HighQC™ Human IPSC From Fibroblast-Lesch-Nyhan Syndrome is a disease-specific human induced pluripotent stem cell line derived from dermal fibroblasts of a donor diagnosed with Lesch-Nyhan Syndrome (LNS). This X-linked recessive disorder caused by mutations in the HPRT1 gene, leading to a deficiency of the enzyme hypoxanthine-guanine phosphoribosyltransferase (HGPRT). The resulting iPSCs (induced pluripotent stem cells) exhibit adherent growth with well-defined borders and a characteristic high nucleus-to-cytoplasm ratio. They preserve pluripotency and model LNS-related metabolic traits for research. 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.