Atlastin GTPase 1 (ATL1)

Atlastin GTPase 1 (ATL1) is a membrane-anchored dynamin-related enzyme primarily expressed in the central nervous system, particularly in neurons of the corticospinal tract[4][5]. It catalyzes GTP-dependent homotypic fusion of endoplasmic reticulum (ER) membranes, playing a crucial role in maintaining the interconnected network of ER tubules[1][2][3][5]. The protein contains a GTPase domain and a unique three-helix bundle critical for its fusion mechanism, with nucleotide-dependent dimerization and conformational changes driving membrane merging[1][2]. ATL1 is genetically linked to hereditary spastic paraplegia type 3A (SPG3A), where pathogenic mutations lead to axonal degeneration and progressive spasticity in the lower limbs[4][5]. No drugs directly target ATL1, and its clinical relevance is mainly as a genetic biomarker and player in neurodegenerative disease[3][4][5].

Other names

Atlastin-1ATL1GBP3SPG3ATL-1GBP-3hGBP3FSP1AD-FSPbrain-specific GTP-binding proteinGTP-binding protein 3guanine nucleotide-binding protein 3spastic paraplegia 3 protein AHSN1DSPG3Aatlastin1atlastin-1guanylate-binding protein 3