Target intelligence / Profile preview

Roundabout guidance receptor 3 (ROBO3)

Target
ROBO3
Molecular classification
Receptor, Immunoglobulin superfamily receptor, Transmembrane protein
01

Overview

Roundabout guidance receptor 3 (ROBO3) is a transmembrane receptor protein essential for proper development of the nervous system, particularly in directing neuronal migration and axon pathfinding across the midline in the brainstem and spinal cord[3][5][1]. ROBO3 is a member of the ROBO family within the immunoglobulin superfamily of receptors; unlike other ROBO family members, it does not bind canonical Slit ligands but can bind NELL2, mediating axonal repulsion and acting as a key signaling hub during commissural axon guidance[1][4]. Mutations in ROBO3 are causative for the human disorder known as horizontal gaze palsy with progressive scoliosis (HGPPS), characterized by congenital absence of horizontal eye movement and progressive spinal curvature due to failed crossing of motor and sensory neuronal tracts at the brainstem[3][5]. ROBO3 is not known to directly interact with any approved drugs, nor is it currently used as a biomarker for therapy or targeted by mechanism-based pharmaceuticals.

Other names
Roundabout homolog 3RBIG1FLJ21044HGPPSRoundabout-like protein 3HGPPS1RIG1retinoblastoma inhibiting gene 1roundabout, axon guidance receptor, homolog 3
02

Biological functions

Axon guidanceNeuronal migrationNeurite outgrowthGrowth cone guidanceAxon fasciculation
03

Disease associations

Neurodevelopmental disorderHorizontal gaze palsy with progressive scoliosis (HGPPS)
04

Safety considerations

Critical for developmental neural wiring; loss of function causes severe neurodevelopmental and motor disordersMutations result in failure of commissural axon crossing and congenital eye movement restrictions with scoliosis[3][5]

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