The functions of UCH-L1 and its relation to neurodegenerative diseases

Neurochem Int. Jul-Sep 2007;51(2-4):105-11. doi: 10.1016/j.neuint.2007.05.007. Epub 2007 May 24.


Parkinson's disease (PD) and Alzheimer's disease (AD), the most common neurodegenerative diseases, are caused by both genetic and environmental factors. Ubiquitin carboxy-terminal hydrolase L1 (UCH-L1) is a deubiquitinating enzyme that is involved in the pathogenesis of both of these neurodegenerative diseases. Several functions of UCH-L1, other than as an ubiquitin hydrolase, have been proposed; these include acting as an ubiquitin ligase and stabilizing mono-ubiquitin. This review focuses on recent findings on the functions and the regulation of UCH-L1, in particular those that relate to PD and AD.

Publication types

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

MeSH terms

  • Alzheimer Disease / genetics
  • Alzheimer Disease / metabolism*
  • Animals
  • Humans
  • Mice
  • Mice, Neurologic Mutants / genetics
  • Mice, Neurologic Mutants / metabolism
  • Mutation / genetics
  • Nerve Degeneration / genetics
  • Nerve Degeneration / metabolism
  • Nerve Degeneration / physiopathology
  • Nerve Tissue Proteins / metabolism
  • Oxidative Stress / physiology
  • Parkinson Disease / genetics
  • Parkinson Disease / metabolism*
  • Ubiquitin / metabolism
  • Ubiquitin Thiolesterase / genetics
  • Ubiquitin Thiolesterase / physiology*


  • Nerve Tissue Proteins
  • UCHL1 protein, human
  • Ubiquitin
  • Ubiquitin Thiolesterase