Prickle planar cell polarity protein 3 (PRICKLE3)

Prickle planar cell polarity protein 3 (PRICKLE3) is a LIM domain-containing modular protein involved in the core module of the planar cell polarity (PCP) pathway, essential for the polarization of epithelial cells during morphogenesis, including gastrulation and neurulation[3][4][6]. Its three LIM domains mediate protein-protein interactions, enabling complex assembly and signaling. PRICKLE3 localizes to the basal body and is involved in cilia growth, positioning, and cellular organization[1][3][4]. It also plays a role in the assembly and stability of mitochondrial ATP synthase, influencing bioenergetics[2][3][4]. Disease associations include being a genetic modifier in Leber hereditary optic neuropathy and ocular motility disease[3]; however, it is not currently a recognized therapeutic target nor does it directly interact with drugs, nor serve as a clinical biomarker. Disruption of PRICKLE3 impairs cilia-related developmental processes and can affect cell polarity and mitochondrial function.

Other names

LIM domain only protein 6LMO6LMO-6Triple LIM domain protein 6Prickle-like protein 3LOAMLOASPk3Prickle homolog 3