Cat. #162032
Melan-rp4 cell line
Cat. #: 162032
Organism: Mouse
Tissue: Skin
Disease: Hermansky-Pudlak syndrome
Model: Immortalised non-cancerous cell lines
£575.00
This fee is applicable only for non-profit organisations. If you are a for-profit organisation or a researcher working on commercially-sponsored academic research, you will need to contact our licensing team for a commercial use license.
Contributor
Inventor: Elena Sviderskaya
Institute: St. George's University London
Tool Details
*FOR RESEARCH USE ONLY (for other uses, please contact the licensing team)
- Name: Melan-rp4 cell line
- Organism: Mouse
- Tissue: Skin
- Disease: Hermansky-Pudlak syndrome
- Morphology: Small, oval-fusiform
- Model: Immortalised non-cancerous cell lines
- 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.
- Application: Melanocyte cell biology
Applications
- Application: Melanocyte cell biology
Handling
- Growth medium: RPMI + FBS (10%) + TPA (200 nM) + cholera toxin (CT) (200 pM)
- Storage conditions: liquid N2
