Ablation of polyamine catabolic enzymes provokes Purkinje cell damage, neuroinflammation, and severe ataxia.

ABSTRACT:

Background

Polyamine catabolism plays a key role in maintaining intracellular polyamine pools, yet its physiological significance is largely unexplored. Here, we report that the disruption of polyamine catabolism leads to severe cerebellar damage and ataxia, demonstrating the fundamental role of polyamine catabolism in the maintenance of cerebellar function and integrity.

Methods

Mice with simultaneous deletion of the two principal polyamine catabolic enzymes, spermine oxidase and spermidine/spermine N¹-acetyltransferase (Smox/Sat1-dKO), were generated by the crossbreeding of Smox-KO (Smox^-/-) and Sat1-KO (Sat1^-/-) animals. Development and progression of tissue injury was monitored using imaging, behavioral, and molecular analyses.

Results

SUBMITTER: Zahedi K 

PROVIDER: S-EPMC7559641 | biostudies-literature | 2020 Oct

REPOSITORIES: biostudies-literature