TBC1D24 is a multifunctional GTPase-activating protein primarily involved in neuronal development and synaptic function. The protein acts as a GAP for Rab family proteins 1 and negatively modulates ARF6 function to regulate neuronal projection development 1. TBC1D24 controls synaptic vesicle trafficking 2 and critically regulates intraorganellar pH homeostasis by interacting with and positively regulating the vacuolar ATPase (v-ATPase) 3. Loss of TBC1D24 function impairs axonal specification, growth cone endocytosis, and action potential firing during early neuronal development 4. Clinically, TBC1D24 mutations cause a phenotypically diverse spectrum of neurological disorders 5. The most common manifestation is myoclonic epilepsy (38% of patients), though presentations range from isolated deafness to early-onset epileptic encephalopathy with severe developmental delay 5. Most patients develop drug-resistant epilepsy 5. TBC1D24 mutations also cause DOORS syndrome, nonsyndromic deafness (DFNA65/DFNB86), and alternating hemiplegia of childhood 67. Disease severity correlates with the degree of axonal defects 4. The protein's roles in pH homeostasis and v-ATPase regulation suggest that disrupted organellar acidification may underlie pathology 3.