Ontology highlight
ABSTRACT:
SUBMITTER: Sellin J
PROVIDER: S-EPMC6025876 | biostudies-literature | 2018 Jun
REPOSITORIES: biostudies-literature
Sellin Julia J Wingen Christian C Gosejacob Dominic D Senyilmaz Deniz D Hänschke Lea L Büttner Sven S Meyer Katharina K Bano Daniele D Nicotera Pierluigi P Teleman Aurelio A AA Bülow Margret H MH
PLoS biology 20180619 6
Mutations in peroxin (PEX) genes lead to loss of peroxisomes, resulting in the formation of peroxisomal biogenesis disorders (PBDs) and early lethality. Studying PBDs and their animal models has greatly contributed to our current knowledge about peroxisomal functions. Very-long-chain fatty acid (VLCFA) accumulation has long been suggested as a major disease-mediating factor, although the exact pathological consequences are unclear. Here, we show that a Drosophila Pex19 mutant is lethal due to a ...[more]