ADP-ribosylation factor-like protein 3 (ARL3)

ADP-ribosylation factor-like protein 3 (ARL3) is a member of the ARF-like family of small GTPases, functioning as a regulatory switch that cycles between inactive GDP-bound and active GTP-bound states[2][3][1]. ARL3 is predominantly involved in the regulation of ciliary trafficking of lipid-modified proteins and is essential for cilium function, photoreceptor development, and maintenance[2][1][4]. It localizes to the centrosome, primary cilium, Golgi apparatus, mitochondria, and endosomes, and can influence processes such as cell division and autophagy regulation[2][3][4]. ARL3 is activated by the guanine exchange factor ARL13B, with cofactors such as BART and ARL2BP facilitating full activation and proper function[1][2]. Dysregulation or mutations in ARL3 cause a spectrum of ciliopathies, including Joubert syndrome, retinal dystrophies (such as cone-rod dystrophy and retinitis pigmentosa), and other multisystem disorders due to defects in ciliary trafficking and photoreceptor degeneration[2]. No direct pharmacological modulators or clinical drugs acting on ARL3 are currently known.

Other names

ARF like GTPase 3ARFL3JBTS35RP83ARF-like 3ADP ribosylation factor like GTPase 3