Peroxisome biogenesis deficiency attenuates the BDNF-TrkB pathway-mediated development of the cerebellum.

ABSTRACT: Peroxisome biogenesis disorders (PBDs) manifest as neurological deficits in the central nervous system, including neuronal migration defects and abnormal cerebellum development. However, the mechanisms underlying pathogenesis remain enigmatic. Here, to investigate how peroxisome deficiency causes neurological defects of PBDs, we established a new PBD model mouse defective in peroxisome assembly factor Pex14p, termed Pex14 ^ΔC/ΔC mouse. Pex14 ^ΔC/ΔC mouse manifests a severe symptom such as disorganization of cortical laminar structure and dies shortly after birth, although peroxisomal biogenesis and metabolism are partially defective. The Pex14 ^ΔC/ΔC mouse also shows malformation of the cerebellum including the impaire

SUBMITTER: Abe Y 

PROVIDER: S-EPMC6277683 | biostudies-literature | 2018 Dec

REPOSITORIES: biostudies-literature