Hereditary spastic paraplegia: respiratory choke or unactivated substrate?
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.
Claypool
Steven M
SM
Department of Chemistry and Biochemistry, University of California, Los Angeles, 90095, USA.
Koehler
Carla M
CM
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
D008666:Metalloendopeptidases; C491813:Spg7 protein, mouse; C114583:m-AAA proteases; D018833:Chaperonins; D000074183:ATPases Associated with Diverse Cellular Activities