Target intelligence / Profile preview

RIMS binding protein 2 (RIMBP2)

Target
RIMBP2
Molecular classification
Other (modular adaptor/scaffolding protein), Protein phosphatase 1 regulatory subunit
01

Overview

RIMS binding protein 2 (RIMBP2) is a modular adaptor and scaffolding protein found at the presynaptic active zone of neurons, where it links voltage-gated calcium channels and synaptic release machinery to organize and regulate neurotransmitter release[1][2][3][4][5][6]. It features multiple SH3-domains and fibronectin III repeats, supporting its role as a scaffold for coupling Ca²⁺ channel influx with vesicle exocytosis and ensuring precise synaptic signaling[1][2][5]. RIMBP2 is essential for organizing the presynaptic active zone, modulating calcium channel abundance and coupling to release sites, thus influencing release probability and synaptic plasticity[2][4][5][6]. Dysregulation or mutation of RIMBP2 has been linked to various neurological disorders, including Pitt-Hopkins syndrome, specific hereditary deafness, and centronuclear myopathy[3][6]. Despite its critical role in synaptic transmission, RIMBP2 itself is not recognized as a direct therapeutic target for available drugs[1][3][6].

Other names
RIM-BP2PPP1R133KIAA0318RBP2MGC15831protein phosphatase 1 regulatory subunit 133
02

Biological functions

Regulation of synaptic transmissionPresynaptic active zone scaffoldingRegulation of voltage-gated calcium channel localization and abundanceOrganization of neurotransmitter release machineryRegulation of presynaptic cytosolic calcium levelsRegulation of synaptic vesicle fusion
03

Disease associations

Neurodevelopmental disease (e.g., Pitt-Hopkins syndrome)Neurological disorderDeafness (autosomal dominant 41)Myopathy (centronuclear, type 6)Other

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