Article
Inactivation of the hereditary spastic paraplegia-associated Hspd1 gene encoding the Hsp60 chaperone results in early embryonic lethality in mice.
Cell stress & chaperones - 1 Nov 2010
Christensen Jane H, Nielsen Marit N, Hansen Jakob, Füchtbauer Annette, Füchtbauer Ernst-Martin, West Mark, Corydon Thomas J, Gregersen Niels, Bross Peter
Abstract excerpt
The mitochondrial Hsp60 chaperonin plays an important role in sustaining cellular viability. Its dysfunction is related to inherited forms of the human diseases spastic paraplegia and hypomyelinating leukodystrophy. However, it is unknown whether the requirement for Hsp60 is neuron specific or whether a complete loss of the protein will impair mammalian development and postnatal survival. In this study, we...
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