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Target intelligence / Profile preview
Protein unc-93 homolog B1 (UNC93B1)
- Target
- UNC93B1
- Molecular classification
- Other (12-pass transmembrane trafficking chaperone)
01
Overview
Protein unc-93 homolog B1 (UNC93B1) is a polytopic transmembrane protein that acts as a critical trafficking chaperone for nucleic acid–sensing toll-like receptors (TLRs), specifically TLR3, TLR7, TLR8, TLR9, TLR11, TLR12, TLR13, and also TLR5. UNC93B1 is required for the correct trafficking of these TLRs from the endoplasmic reticulum to their respective signaling compartments (endosomes or plasma membrane), enabling proper recognition of pathogen-associated molecular patterns and appropriate innate immune responses. Deficiency or mutation in UNC93B1 disrupts TLR trafficking and signaling, predisposing individuals to severe infections (notably HSV-1 encephalitis) and, in some cases, autoimmune or autoinflammatory disease. UNC93B1 acts not as a direct receptor or enzyme but as an accessory/trafficking protein essential for TLR function and immune defense regulation[1][2][3][4].
- Other names
- Unc-93 homolog B1UNC93UNC93BUnc-93B1hUNC93B1IIAE1TLR signaling regulatorunc-93 related proteinunc93 homolog B1
02
Biological functions
Immune responseSignal transductionRegulation of toll-like receptor traffickingProtein stabilization
03
Disease associations
Infection (especially susceptibility to Herpes simplex virus type 1 encephalitis)Autoimmunity/autoinflammatory diseaseInflammation
04
Safety considerations
Loss or mutation can lead to severe viral infections (e.g., HSV-1 encephalitis)[1]Mutations may provoke autoinflammatory disorders due to dysregulated TLR signaling[1]
05
Biomarkers
Susceptibility to HSV-1 encephalitis (loss-of-function mutations used diagnostically)
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