Associated secretory biogenesis and mast cell phenotype | Type of SNARE protein responsible for vesicular trafficking | Ref. |
1. Familial Lymphohystiocytosis (FLH) type |
| |
a. FLH1 | Unknown |
|
b. FLH2 | Defect in cytotoxic granule protein perforin/pore forming protein | [87] |
c. FLH3 | Defects in SNARE accessory protein Munc13-4 | [88] |
d. FLH4 | Defects in t-SNARE syntaxin-11 | [89] |
e. FLH5 | Defects in SNARE accessory protein syntaxin Binding Protein-2 (Munc18-2) that belonging to SM family of fusion accessory protein | [90] [91] |
2. Hermansky-Pudlak syndrome (HPS) | Pulmonary fibrosis―endosomes to lysosomes related defects | [92] [93] |