| pubmed-article:16816367 | rdf:type | pubmed:Citation | lld:pubmed |
| pubmed-article:16816367 | lifeskim:mentions | umls-concept:C0026809 | lld:lifeskim |
| pubmed-article:16816367 | lifeskim:mentions | umls-concept:C0031760 | lld:lifeskim |
| pubmed-article:16816367 | lifeskim:mentions | umls-concept:C0162638 | lld:lifeskim |
| pubmed-article:16816367 | lifeskim:mentions | umls-concept:C0035304 | lld:lifeskim |
| pubmed-article:16816367 | pubmed:issue | 1 | lld:pubmed |
| pubmed-article:16816367 | pubmed:dateCreated | 2006-7-3 | lld:pubmed |
| pubmed-article:16816367 | pubmed:abstractText | UCH-L3 belongs to the ubiquitin C-terminal hydrolase family that deubiquitinates ubiquitin-protein conjugates in the ubiquitin-proteasome system. A murine Uchl3 deletion mutant displays retinal degeneration, muscular degeneration, and mild growth retardation. To elucidate the function of UCH-L3, we investigated histopathological changes and expression of apoptosis- and oxidative stress-related proteins during retinal degeneration. In the normal retina, UCH-L3 was enriched in the photoreceptor inner segment that contains abundant mitochondria. Although the retina of Uchl3-deficient mice showed no significant morphological abnormalities during retinal development, prominent retinal degeneration became manifested after 3 weeks of age associated with photoreceptor cell apoptosis. Ultrastructurally, a decreased area of mitochondrial cristae and vacuolar changes were observed in the degenerated inner segment. Increased immunoreactivities for manganese superoxide dismutase, cytochrome c oxidase I, and apoptosis-inducing factor in the inner segment indicated mitochondrial oxidative stress. Expression of cytochrome c, caspase-1, and cleaved caspase-3 did not differ between wild-type and mutant mice; however, immunoreactivity for endonuclease G was found in the photoreceptor nuclei in the mutant retina. Hence, loss of UCH-L3 leads to mitochondrial oxidative stress-related photoreceptor cell apoptosis in a caspase-independent manner. Thus, Uchl3-deficient mice represent a model for adult-onset retinal degeneration associated with mitochondrial impairment. | lld:pubmed |
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| pubmed-article:16816367 | pubmed:language | eng | lld:pubmed |
| pubmed-article:16816367 | pubmed:journal | http://linkedlifedata.com/r... | lld:pubmed |
| pubmed-article:16816367 | pubmed:citationSubset | AIM | lld:pubmed |
| pubmed-article:16816367 | pubmed:chemical | http://linkedlifedata.com/r... | lld:pubmed |
| pubmed-article:16816367 | pubmed:status | MEDLINE | lld:pubmed |
| pubmed-article:16816367 | pubmed:month | Jul | lld:pubmed |
| pubmed-article:16816367 | pubmed:issn | 0002-9440 | lld:pubmed |
| pubmed-article:16816367 | pubmed:author | pubmed-author:WadaKeijiK | lld:pubmed |
| pubmed-article:16816367 | pubmed:author | pubmed-author:FurutaAkikoA | lld:pubmed |
| pubmed-article:16816367 | pubmed:author | pubmed-author:NodaMamiM | lld:pubmed |
| pubmed-article:16816367 | pubmed:author | pubmed-author:KwonJungkeeJ | lld:pubmed |
| pubmed-article:16816367 | pubmed:author | pubmed-author:SetsuieRiekoR | lld:pubmed |
| pubmed-article:16816367 | pubmed:author | pubmed-author:WangYu-LaiYL | lld:pubmed |
| pubmed-article:16816367 | pubmed:author | pubmed-author:SakuraiMikako... | lld:pubmed |
| pubmed-article:16816367 | pubmed:author | pubmed-author:KikuchiHisaeH | lld:pubmed |
| pubmed-article:16816367 | pubmed:author | pubmed-author:SanoYaeY | lld:pubmed |
| pubmed-article:16816367 | pubmed:issnType | Print | lld:pubmed |
| pubmed-article:16816367 | pubmed:volume | 169 | lld:pubmed |
| pubmed-article:16816367 | pubmed:owner | NLM | lld:pubmed |
| pubmed-article:16816367 | pubmed:authorsComplete | Y | lld:pubmed |
| pubmed-article:16816367 | pubmed:pagination | 132-41 | lld:pubmed |
| pubmed-article:16816367 | pubmed:dateRevised | 2009-11-18 | lld:pubmed |
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| pubmed-article:16816367 | pubmed:meshHeading | pubmed-meshheading:16816367... | lld:pubmed |
| pubmed-article:16816367 | pubmed:year | 2006 | lld:pubmed |
| pubmed-article:16816367 | pubmed:articleTitle | Photoreceptor cell apoptosis in the retinal degeneration of Uchl3-deficient mice. | lld:pubmed |
| pubmed-article:16816367 | pubmed:affiliation | Department of Degenerative Neurological Diseases, National Institute of Neuroscience, National Center of Neurology and Psychiatry, 4-1-1, Ogawahigashi, Kodaira, Tokyo 187-8502, Japan. | lld:pubmed |
| pubmed-article:16816367 | pubmed:publicationType | Journal Article | lld:pubmed |
| pubmed-article:16816367 | pubmed:publicationType | Research Support, Non-U.S. Gov't | lld:pubmed |
| entrez-gene:50933 | entrezgene:pubmed | pubmed-article:16816367 | lld:entrezgene |
| http://linkedlifedata.com/r... | pubmed:referesTo | pubmed-article:16816367 | lld:pubmed |
