onr:"swepub:oai:prod.swepub.kib.ki.se:123024396"
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| Fältnamn | Indikatorer | Metadata |
|---|---|---|
| 000 | 02640naa a2200325 4500 | |
| 001 | oai:prod.swepub.kib.ki.se:123024396 | |
| 003 | SwePub | |
| 008 | 240701s2011 | |||||||||||000 ||eng| | |
| 024 | 7 | a http://kipublications.ki.se/Default.aspx?queryparsed=id:1230243962 URI |
| 024 | 7 | a https://doi.org/10.1073/pnas.11032951082 DOI |
| 040 | a (SwePub)ki | |
| 041 | a engb eng | |
| 042 | 9 SwePub | |
| 072 | 7 | a ref2 swepub-contenttype |
| 072 | 7 | a art2 swepub-publicationtype |
| 100 | 1 | a Sterky, FHu Karolinska Institutet4 aut |
| 245 | 1 0 | a Impaired mitochondrial transport and Parkin-independent degeneration of respiratory chain-deficient dopamine neurons in vivo |
| 264 | c 2011-07-18 | |
| 264 | 1 | b Proceedings of the National Academy of Sciences,c 2011 |
| 520 | a Mitochondrial dysfunction is heavily implicated in Parkinson disease (PD) as exemplified by the finding of an increased frequency of respiratory chain-deficient dopamine (DA) neurons in affected patients. An inherited form of PD is caused by impaired function of Parkin, an E3 ubiquitin ligase reported to translocate to defective mitochondria in vitro to facilitate their clearance. We have developed a reporter mouse to assess mitochondrial morphology in DA neurons in vivo and show here that respiratory chain deficiency leads to fragmentation of the mitochondrial network and to the formation of large cytoplasmic bodies derived from mitochondria. Surprisingly, the dysfunctional mitochondria do not recruit Parkin in vivo, and neither the clearance of defective mitochondria nor the neurodegeneration phenotype is affected by the absence of Parkin. We also show that anterograde axonal transport of mitochondria is impaired in respiratory chain-deficient DA neurons, leading to a decreased supply of mitochondria to the axonal terminals. | |
| 700 | 1 | a Lee, Su Karolinska Institutet4 aut |
| 700 | 1 | a Wibom, Ru Karolinska Institutet4 aut |
| 700 | 1 | a Olson, Lu Karolinska Institutet4 aut |
| 700 | 1 | a Larsson, NGu Karolinska Institutet4 aut |
| 710 | 2 | a Karolinska Institutet4 org |
| 773 | 0 | t Proceedings of the National Academy of Sciences of the United States of Americad : Proceedings of the National Academy of Sciencesg 108:31, s. 12937-12942q 108:31<12937-12942x 1091-6490 |
| 773 | 0 | t Proceedings of the National Academy of Sciencesd : Proceedings of the National Academy of Sciencesg 108:31, s. 12937-12942q 108:31<12937-12942x 0027-8424 |
| 856 | 4 | u http://www.pnas.org/content/108/31/12937.full.pdf |
| 856 | 4 8 | u http://kipublications.ki.se/Default.aspx?queryparsed=id:123024396 |
| 856 | 4 8 | u https://doi.org/10.1073/pnas.1103295108 |
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