BOD1, short for Biorientation Defective 1, is a protein-coding gene essential for proper chromosome segregation. It regulates phosphorylation of PLK1 substrates by modulating Protein Phosphatase 2A (PP2A) activity during mitosis [2]. Bod1 localizes with kinetochores and spindle poles during mitosis and is required for the detection or resolution of syntelic attachments in mitotic spindles [3]. It also belongs to a family of specific PP2A inhibitors that regulate specific PP2A holoenzymes at distinct locations and points in the cell cycle [4].

In a mouse model, BOD1 deficit in Purkinje cells of the cerebellar IV/V lobe attenuated the excitability and spine density of these cells, leading to ataxia behaviors. Conversely, BOD1 enrichment in these cells reversed the hyperexcitability of CaMKIIα+ neurons in the fastigial nucleus and ameliorated ataxia behaviors in L7-Cre; BOD1f/f mice, suggesting its role in the cerebellar IV/V lobe-fastigial nucleus circuit associated with motor coordination [1]. In Drosophila, neuron-specific knockdown of BOD1 caused pronounced learning deficits and significant abnormalities in synapse morphology, indicating its role in cognitive function [2].

In conclusion, BOD1 is crucial for chromosome biorientation during cell division and has cell-cycle independent functions in the nervous system. Studies using KO or CKO mouse models and Drosophila models have revealed its significance in motor coordination and cognitive function, providing insights into potential therapeutic targets for ataxia and intellectual disability [1,2].