| pubmed-article:16647881 | rdf:type | pubmed:Citation | lld:pubmed |
| pubmed-article:16647881 | lifeskim:mentions | umls-concept:C0040710 | lld:lifeskim |
| pubmed-article:16647881 | lifeskim:mentions | umls-concept:C0026237 | lld:lifeskim |
| pubmed-article:16647881 | lifeskim:mentions | umls-concept:C0037773 | lld:lifeskim |
| pubmed-article:16647881 | lifeskim:mentions | umls-concept:C0542341 | lld:lifeskim |
| pubmed-article:16647881 | lifeskim:mentions | umls-concept:C0757738 | lld:lifeskim |
| pubmed-article:16647881 | pubmed:issue | 6 | lld:pubmed |
| pubmed-article:16647881 | pubmed:dateCreated | 2006-6-19 | lld:pubmed |
| pubmed-article:16647881 | pubmed:abstractText | Hereditary spastic paraplegia (HSP) is a genetically heterogeneous neurodegenerative disorder that is characterized by progressive and cell-specific axonal degeneration. An autosomal recessive form of the disease is caused by mutations in paraplegin, which is a conserved subunit of the ubiquitous and ATP-dependent m-AAA protease in mitochondria. The m-AAA protease carries out protein quality control in the inner membrane of the mitochondria, suggesting a pathogenic role of misfolded proteins in HSP. A recent study demonstrates that the m-AAA protease regulates ribosome assembly and translation within mitochondria by controlling proteolytic maturation of a ribosomal subunit. Here, we will discuss implications of the dual role of the m-AAA protease in protein activation and degradation for mitochondrial dysfunction and axonal degeneration. | lld:pubmed |
| pubmed-article:16647881 | pubmed:language | eng | lld:pubmed |
| pubmed-article:16647881 | pubmed:journal | http://linkedlifedata.com/r... | lld:pubmed |
| pubmed-article:16647881 | pubmed:citationSubset | IM | lld:pubmed |
| pubmed-article:16647881 | pubmed:chemical | http://linkedlifedata.com/r... | lld:pubmed |
| pubmed-article:16647881 | pubmed:chemical | http://linkedlifedata.com/r... | lld:pubmed |
| pubmed-article:16647881 | pubmed:chemical | http://linkedlifedata.com/r... | lld:pubmed |
| pubmed-article:16647881 | pubmed:chemical | http://linkedlifedata.com/r... | lld:pubmed |
| pubmed-article:16647881 | pubmed:chemical | http://linkedlifedata.com/r... | lld:pubmed |
| pubmed-article:16647881 | pubmed:chemical | http://linkedlifedata.com/r... | lld:pubmed |
| pubmed-article:16647881 | pubmed:status | MEDLINE | lld:pubmed |
| pubmed-article:16647881 | pubmed:month | Jun | lld:pubmed |
| pubmed-article:16647881 | pubmed:issn | 1471-4914 | lld:pubmed |
| pubmed-article:16647881 | pubmed:author | pubmed-author:RugarliElena... | lld:pubmed |
| pubmed-article:16647881 | pubmed:author | pubmed-author:LangerThomasT | lld:pubmed |
| pubmed-article:16647881 | pubmed:issnType | Print | lld:pubmed |
| pubmed-article:16647881 | pubmed:volume | 12 | lld:pubmed |
| pubmed-article:16647881 | pubmed:owner | NLM | lld:pubmed |
| pubmed-article:16647881 | pubmed:authorsComplete | Y | lld:pubmed |
| pubmed-article:16647881 | pubmed:pagination | 262-9 | lld:pubmed |
| pubmed-article:16647881 | pubmed:meshHeading | pubmed-meshheading:16647881... | lld:pubmed |
| pubmed-article:16647881 | pubmed:meshHeading | pubmed-meshheading:16647881... | lld:pubmed |
| pubmed-article:16647881 | pubmed:meshHeading | pubmed-meshheading:16647881... | lld:pubmed |
| pubmed-article:16647881 | pubmed:meshHeading | pubmed-meshheading:16647881... | lld:pubmed |
| pubmed-article:16647881 | pubmed:meshHeading | pubmed-meshheading:16647881... | lld:pubmed |
| pubmed-article:16647881 | pubmed:meshHeading | pubmed-meshheading:16647881... | lld:pubmed |
| pubmed-article:16647881 | pubmed:meshHeading | pubmed-meshheading:16647881... | lld:pubmed |
| pubmed-article:16647881 | pubmed:meshHeading | pubmed-meshheading:16647881... | lld:pubmed |
| pubmed-article:16647881 | pubmed:meshHeading | pubmed-meshheading:16647881... | lld:pubmed |
| pubmed-article:16647881 | pubmed:meshHeading | pubmed-meshheading:16647881... | lld:pubmed |
| pubmed-article:16647881 | pubmed:meshHeading | pubmed-meshheading:16647881... | lld:pubmed |
| pubmed-article:16647881 | pubmed:meshHeading | pubmed-meshheading:16647881... | lld:pubmed |
| pubmed-article:16647881 | pubmed:meshHeading | pubmed-meshheading:16647881... | lld:pubmed |
| pubmed-article:16647881 | pubmed:meshHeading | pubmed-meshheading:16647881... | lld:pubmed |
| pubmed-article:16647881 | pubmed:meshHeading | pubmed-meshheading:16647881... | lld:pubmed |
| pubmed-article:16647881 | pubmed:year | 2006 | lld:pubmed |
| pubmed-article:16647881 | pubmed:articleTitle | Translating m-AAA protease function in mitochondria to hereditary spastic paraplegia. | lld:pubmed |
| pubmed-article:16647881 | pubmed:affiliation | Istituto Nazionale Neurologico C. Besta, Division of Biochemistry and Genetics, 20126 Milan, Italy. | lld:pubmed |
| pubmed-article:16647881 | pubmed:publicationType | Journal Article | lld:pubmed |
| pubmed-article:16647881 | pubmed:publicationType | Review | lld:pubmed |
| pubmed-article:16647881 | pubmed:publicationType | Research Support, Non-U.S. Gov't | lld:pubmed |
| http://linkedlifedata.com/r... | pubmed:referesTo | pubmed-article:16647881 | lld:pubmed |
| http://linkedlifedata.com/r... | pubmed:referesTo | pubmed-article:16647881 | lld:pubmed |
| http://linkedlifedata.com/r... | pubmed:referesTo | pubmed-article:16647881 | lld:pubmed |
| http://linkedlifedata.com/r... | pubmed:referesTo | pubmed-article:16647881 | lld:pubmed |
| http://linkedlifedata.com/r... | pubmed:referesTo | pubmed-article:16647881 | lld:pubmed |
| http://linkedlifedata.com/r... | pubmed:referesTo | pubmed-article:16647881 | lld:pubmed |
| http://linkedlifedata.com/r... | pubmed:referesTo | pubmed-article:16647881 | lld:pubmed |
| http://linkedlifedata.com/r... | pubmed:referesTo | pubmed-article:16647881 | lld:pubmed |
| http://linkedlifedata.com/r... | pubmed:referesTo | pubmed-article:16647881 | lld:pubmed |
| http://linkedlifedata.com/r... | pubmed:referesTo | pubmed-article:16647881 | lld:pubmed |
