Hereditary spastic paraplegia: Respiratory choke or unactivated substrate?

Steven M. Claypool, Carla M. Koehler

Research output: Contribution to journalShort surveypeer-review

4 Scopus citations


Defects in the mitochondrial AAA protease family member, paraplegin, result in an autosomal recessive form of hereditary spastic paraplegia (HSP). In this issue of Cell, Nolden et al. (2005) report a new molecular mechanism for HSP based on the requirement of paraplegin for the proteolysis of a specific mitochondrial ribosomal protein. The processing of this substrate is required for robust translation in mitochondria.

Original languageEnglish (US)
Pages (from-to)183-185
Number of pages3
Issue number2
StatePublished - Oct 21 2005
Externally publishedYes

ASJC Scopus subject areas

  • Biochemistry, Genetics and Molecular Biology(all)


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