title: The AAA ATPase spastin links microtubule severing to membrane modelling.
abstract: In 1999, mutations in the gene encoding the microtubule severing AAA ATPase spastin were identified as a major cause of a genetic neurodegenerative condition termed hereditary spastic paraplegia (HSP). This finding stimulated intense study of the spastin protein and over the last decade, a combination of cell biological, in vivo, in vitro and structural studies have provided important mechanistic insights into the cellular functions of the protein, as well as elucidating cell biological pathways that might be involved in axonal maintenance and degeneration. Roles for spastin have emerged in shaping the endoplasmic reticulum and the abscission stage of cytokinesis, in which spastin appears to couple membrane modelling to microtubule regulation by severing.
authors: Department of Medical Genetics and Cambridge Institute for Medical Research, University of Cambridge, UK.
authors: Jennifer H
authors: JH
authors: Lumb
authors: James W
authors: JW
authors: Connell
authors: Rachel
authors: R
authors: Allison
authors: Evan
authors: E
authors: Reid
mesh_terms: D000251:Adenosine Triphosphatases / Q000737:chemistry / Q000235:genetics / Q000378:metabolism*; D000818:Animals; D001369:Axons / Q000201:enzymology / Q000473:pathology; D002462:Cell Membrane / Q000378:metabolism*; D056827:Endosomal Sorting Complexes Required for Transport / Q000378:metabolism; D011992:Endosomes / Q000378:metabolism; D006801:Humans; D008870:Microtubules / Q000378:metabolism*; D009154:Mutation; D017434:Protein Structure, Tertiary; D015419:Spastic Paraplegia, Hereditary / Q000201:enzymology / Q000235:genetics; D000074181:Spastin
chemical_list: D056827:Endosomal Sorting Complexes Required for Transport; D000251:Adenosine Triphosphatases; D000074181:Spastin; C400283:SPAST protein, human