Target intelligence / Profile preview

Syntaxin-binding protein 2 (STXBP2)

Target
STXBP2
Molecular classification
SNARE complex regulator, Intracellular trafficking protein, Vesicular transport (Sec/Munc/SM protein family)
01

Overview

Syntaxin-binding protein 2 (STXBP2) is a member of the Sec/Munc (Sec1/Munc18, SM) protein family involved in the regulation of vesicle trafficking and membrane fusion in eukaryotic cells. STXBP2 acts as a chaperone and fusion regulator by binding syntaxin-11 and other SNARE proteins, facilitating the exocytosis of cytotoxic granules by natural killer (NK) cells and cytotoxic T lymphocytes. Mutations in STXBP2—including loss-of-function variants—disrupt granule secretion, leading to immune disorders such as familial hemophagocytic lymphohistiocytosis type 5 (FHL5), which is characterized by uncontrolled inflammatory responses due to impaired cytotoxicity in lymphocytes. STXBP2 is expressed in a range of tissues, with notable roles in immune cells. No drugs are currently known to specifically target STXBP2 in clinical use or development.

Other names
FHL5Hunc18bMUNC18-2Unc18-2UNC18-2UNC18Bpp10122unc-18Bsyntaxin-binding protein 2protein unc-18 homolog 2protein unc-18 homolog BSyntaxinBP2Syntaxin-BP2
02

Biological functions

Intracellular vesicle traffickingRegulation of membrane fusionCytotoxic granule exocytosisImmune cytotoxicity (in NK cells and cytotoxic lymphocytes)
03

Disease associations

Familial hemophagocytic lymphohistiocytosis type 5 (FHL5)Immunodeficiency-related syndromesOther rare immune dysregulation disorders
04

Safety considerations

Risk of severe immune dysregulation (e.g., HLH) with loss-of-function mutationsPotential critical effects on innate immune defense when inhibited
05

Biomarkers

Mutations (e.g., R190C) are biomarkers for familial hemophagocytic lymphohistiocytosis type 5 (FHL5)

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