Melan-rp4 cell line

Immortal melanocyte cell lines derived from neonatal Rprp/Rprp Ink4aArf -/- mice

Contributors

Inventor Institute
Elena Sviderskaya St. George's University London
Cat. #: 162032
Organism: Mouse
Tissue: Skin
Model: Immortalised non-cancerous cell lines
Morphology: Small, oval-fusiform
Product description: The melan-rp3, -rp4 and -rp5 cell lines are immortal melanocyte cell lines derived from neonatal Rprp/Rprp Ink4aArf -/- mice. These cell lines lack Blos3, a subunit of the biogenesis of lysosome-related organelles complex 1 (BLOC-1). BLOC-1 is involved in cargo sorting from early-endosomes towards lysosome-related organelles, including melanosomes. Pathogenic variants in the human ortholog of Blos3 cause a form of Hermansky-Pudlak syndrome (HPS), HPS type 8. HPS is a group of disorders characterised by hypopigmentation, bleeding tendency and other variable symptoms. These cells are hypopigmented despite the expression of tyrosinase. These cells are a useful model for HPS and for the study of trafficking of cargoes towards lysosome-related organelles.
disease: Hermansky-Pudlak syndrome
Storage conditions: liquid N2
Growth medium: RPMI + FBS (10%) + TPA (200 nM) + cholera toxin (CT) (200 pM)

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