Preclinical human models and emerging therapeutics for advanced systemic mastocytosis.

ABSTRACT: Mastocytosis is a term used to denote a group of rare diseases characterized by an abnormal accumulation of neoplastic mast cells in various tissues and organs. In most patients with systemic mastocytosis, the neoplastic cells carry activating mutations in KIT Progress in mastocytosis research has long been hindered by the lack of suitable in vitro models, such as permanent human mast cell lines. In fact, only a few human mast cell lines are available to date: HMC-1, LAD1/2, LUVA, ROSA and MCPV-1. The HMC-1 and LAD1/2 cell lines were derived from patients with mast cell leukemia. By contrast, the more recently established LUVA, ROSA and MCPV-1 cell lines were derived from CD34⁺ cells of non-mastocytosis donors. While some of these cell lines (LAD1/2, LUVA, ROSA

SUBMITTER: Arock M 

PROVIDER: S-EPMC6278969 | biostudies-literature | 2018 Nov

REPOSITORIES: biostudies-literature