Sil1-Mutant Mice Elucidate Chaperone Function in Neurological Disorders.

ABSTRACT: Chaperone dysfunction leading to the build-up of misfolded proteins could frequently be linked to clinical manifestations also affecting the nervous system and the skeletal muscle. In addition, increase in chaperone function is beneficial to antagonize protein aggregation and thus represents a promising target for therapeutic concepts for many genetic and acquired chaperonopathies. However, little is known on the precise molecular mechanisms defining the cell and tissue abnormalities in the case of impaired chaperone function as well as on underlying effects in the case of compensatory up-regulation of chaperones. This scarcity of knowledge often arises from a lack of appropriate animal models that mimic closely the human molecular, cellular, and histological characteristics. Here, we intr

SUBMITTER: Buchkremer S 

PROVIDER: S-EPMC5271578 | biostudies-literature | 2016 May

REPOSITORIES: biostudies-literature