Quick Reference

A family of GTPases with homology to guanylate-binding protein-1 (GBP1), one of the dynamin family of large GTPases. It is expressed at much higher level in brain, predominantly in vesicular tubular complexes and cis-Golgi cisternae, and atlastin-1 (558 aa) is mutated in hereditary spastic paraplegia type 3A (SPG3A). The N-terminal domain of spastin (mutated in SPG4) binds directly to the C-terminal cytoplasmic domain of atlastin in yeast two-hybrid assays, suggesting that the two act in concert. Atlastin-2 (ADP-ribosylation-like factor 6-interacting protein 2, 583 aa) and atlastin-3 (541 aa) are closely related to atlastin-1, but are localized to the endoplasmic reticulum in nonbrain tissue.

Subjects: Medicine and Health.

Reference entries