RFP Conditionally Immortalized Human Kidney Podocytes (Fabry Patient)
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Product Overview
Description
Conditionally Immortalized Human Kidney Podocytes, which have been derived from a patient with Fabry disease, represent a highly relevant model for investigating the complexities of Fabry nephropathy. This condition is characterized by podocyte injury and lysosomal dysfunction, both of which are crucial factors in the progression of the disease and its impact on renal function.
Fabry disease arises from a genetic mutation in the GLA gene, which leads to a significant deficiency in the enzyme ?-galactosidase A. This deficiency results in the harmful accumulation of glycosphingolipids, particularly globotriaosylceramide (Gb3/GL3), within various tissues, including the kidneys.
The podocytes, specialized epithelial cells lining the kidney's filtration system, are particularly vulnerable and play a central role in the pathophysiology of Fabry nephropathy, making them a key focus for research into potential therapeutic interventions.
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Key Features
Catalog Number: NBP-0064FP-RFP
Product Format: Frozen
Vial Cell Number: > 5x10[5] cells/vial
Passage:1(P1)
Storage: Liquid Nitrogen, Vapor Phase
Intended Use: Research use only (RUO)
Characteristics:
These specialized cells are engineered to thrive and multiply under permissive culture conditions, allowing for robust growth and expansion. When exposed to non-permissive conditions, these cells have the unique ability to differentiate into specific cell types, which is essential for various research applications.
This innovative approach utilizes temperature-sensitive SV40 large T antigen systems, making it a popular choice for developing conditionally immortalized podocyte models. These models can be crucial for studying kidney function and disease mechanisms.
Quick Facts
Product Type
Immortalized Endothelial
Viability
>95% viable (Trypan Blue exclusion)
Applications
-Fabry nephropathy research -Podocyte injury and…
Availability
In Stock • Global Shipping
Technical Specifications
Primary Applications
Applications & Use Cases
Primary Applications
-Fabry nephropathy research -Podocyte injury and loss studies -Lysosomal storage disease modeling -Gb3/GL3 accumulation studies -Drug screening and therapeutic-response testing -Enzyme replacement or substrate-reduction research models -Fluorescence imaging using RFP signal -Co-culture renal disease models with endothelial or mesangial cells
Suitable for -fabry nephropathy research -podocyte injury and loss studies -lysosomal storage disease modeling -gb3/gl3 accumulation studies -drug screening and therapeutic-response testing -enzyme replacement or substrate-reduction research models -fluorescence imaging using rfp signal -co-culture renal disease models with endothelial or mesangial cells studies and research applications
Research Areas
In Vitro Studies
Cell culture and screening applications
Molecular Biology
Gene expression & signaling studies
Drug Discovery
Screening & validation studies
Disease Modeling
Pathophysiology research
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