The AAA ATPase spastin links microtubule severing to membrane modelling

Biochim Biophys Acta. 2012 Jan;1823(1):192-7. doi: 10.1016/j.bbamcr.2011.08.010. Epub 2011 Aug 25.

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.

Publication types

  • Research Support, Non-U.S. Gov't
  • Review

MeSH terms

  • Adenosine Triphosphatases / chemistry
  • Adenosine Triphosphatases / genetics
  • Adenosine Triphosphatases / metabolism*
  • Animals
  • Axons / enzymology
  • Axons / pathology
  • Cell Membrane / metabolism*
  • Endosomal Sorting Complexes Required for Transport / metabolism
  • Endosomes / metabolism
  • Humans
  • Microtubules / metabolism*
  • Mutation
  • Protein Structure, Tertiary
  • Spastic Paraplegia, Hereditary / enzymology
  • Spastic Paraplegia, Hereditary / genetics
  • Spastin

Substances

  • Endosomal Sorting Complexes Required for Transport
  • Adenosine Triphosphatases
  • Spastin
  • SPAST protein, human