KATNB1 encodes the regulatory p80 subunit of katanin, a microtubule-severing enzyme complex that functions in ATP-dependent microtubule reorganization 1. The protein serves multiple critical roles in cellular processes by modulating the enzymatic activity and subcellular localization of the catalytic KATNA1 subunit 2. KATNB1 is essential for normal brain development, with mutations causing severe microlissencephaly through disruption of asymmetrically dividing neural progenitors and defective mitotic spindle formation 3. The protein regulates centriole and cilia number, affecting Sonic hedgehog signaling pathways crucial for neocortical development 4. During spermatogenesis, KATNB1 localizes to microtubule-based structures including the manchette and is involved in sperm head shaping and flagellum formation 15. Additionally, KATNB1 plays roles in cardiac development, with mutations causing left-right asymmetry defects and heart malformations in mouse models 6. The gene's expression is transcriptionally regulated by Elk1 and post-transcriptionally modulated by miR-124-3p 27. Clinically, KATNB1 mutations are associated with complex cerebral malformations and have been implicated in cancer progression through the MALAT1/miR-328/KATNB1 pathway 8.