Target intelligence / Profile preview

Radial spoke head protein 3 homolog (RSPH3)

Target
RSPH3
Molecular classification
Other, Ciliary structural protein, Protein kinase A-anchoring protein (AKAP)
01

Overview

Radial spoke head protein 3 homolog (RSPH3) is a structural protein that localizes to axonemal radial spoke complexes and plays a critical role in the motility of motile cilia and sperm flagella[1][5]. It serves as a protein kinase A-anchoring protein (AKAP), scaffolding the cAMP-dependent protein kinase holoenzyme and contributing to local cAMP/PKA signal regulation within cilia[2][4]. RSPH3 also interacts with ERK1/2 kinases and may integrate MAPK and PKA signaling in cilia[2][4]. Mutations in RSPH3 cause primary ciliary dyskinesia, a disorder of impaired movement of motile cilia resulting in chronic respiratory disease, infertility, and related symptoms[1][4]. RSPH3 is not currently considered a typical therapeutic target such as a receptor or enzyme, but is of clinical importance due to its essential structural and signaling roles in ciliary function[1][5].

Other names
Radial spoke head 3RSPH3RSHL2RSP3dJ111C20.1A-kinase anchor protein RSPH3Radial spoke head-like protein 2CILD32radial spoke head protein 3 homologradial spoke 3 homolog
02

Biological functions

Ciliary and flagellar motilityProtein kinase A anchoring (scaffolding cAMP-dependent protein kinase)Regulation of ERK/MAPK signaling pathways
03

Disease associations

Primary ciliary dyskinesia (specifically, Primary ciliary dyskinesia 32)Other motile ciliopathies
04

Biomarkers

Mutations in RSPH3 can indicate risk or diagnosis of primary ciliary dyskinesia

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