[HTML][HTML] Inactivation of the hereditary spastic paraplegia-associated Hspd1 gene encoding the Hsp60 chaperone results in early embryonic lethality in mice
JH Christensen, MN Nielsen, J Hansen… - Cell Stress and …, 2010 - Elsevier
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 describe the generation and
characterization of a mutant mouse line bearing an inactivating gene-trap insertion in the …
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 describe the generation and
characterization of a mutant mouse line bearing an inactivating gene-trap insertion in the …