title: Hereditary spastic paraplegia: respiratory choke or unactivated substrate?
abstract: 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.
authors.affiliation: Department of Chemistry and Biochemistry, University of California, Los Angeles, 90095, USA.
authors.forename: Steven M
authors.initials: SM
authors.lastname: Claypool
authors.forename: Carla M
authors.initials: CM
authors.lastname: Koehler
mesh_terms: D000074183:ATPases Associated with Diverse Cellular Activities; D000818:Animals; D018833:Chaperonins / Q000378:metabolism; D006868:Hydrolysis; D008666:Metalloendopeptidases / Q000737:chemistry / Q000172:deficiency / Q000235:genetics / Q000378:metabolism*; D051379:Mice; D008928:Mitochondria / Q000201:enzymology*; D051336:Mitochondrial Membranes / Q000378:metabolism; D008954:Models, Biological; D009154:Mutation; D014176:Protein Biosynthesis; D017510:Protein Folding; D012270:Ribosomes / Q000378:metabolism*; D015419:Spastic Paraplegia, Hereditary / Q000235:genetics*; D013379:Substrate Specificity
chemical_list: D008666:Metalloendopeptidases; C491813:Spg7 protein, mouse; C114583:m-AAA proteases; D018833:Chaperonins; D000074183:ATPases Associated with Diverse Cellular Activities