PPFIBP1 (PPFIB scaffold protein 1), encoding liprin-β1, is a multifunctional protein with critical roles in neurodevelopment and cellular adhesion. Primarily, PPFIBP1 regulates focal adhesion dynamics and may participate in their disassembly 1. The protein localizes to focal adhesions, presynaptic active zones, and the cell cortex, where it regulates cortical microtubule organization and neuromuscular junction development 2. Mechanistically, PPFIBP1 activates focal adhesion kinase (FAK), Src, and JNK signaling pathways, promoting matrix metalloproteinase-2 expression, likely through interaction with SRCIN1/p140Cap 3. It plays essential roles in neuronal outgrowth and synapse formation in model organisms 2. Bi-allelic loss-of-function variants in PPFIBP1 cause severe autosomal recessive neurodevelopmental disorder characterized by moderate to profound developmental delay, early-onset refractory epilepsy, progressive microcephaly, and periventricular calcifications 2. Additional features include failure to thrive, feeding difficulties, and congenital heart defects 2. Homozygous truncating variants result in clinically variable presentations with intellectual disability and speech delay 4. Conversely, PPFIBP1 overexpression promotes glioma cell invasion and migration, with elevated expression correlating to poor prognosis and treatment resistance in glioblastoma 35. Clinically, PPFIBP1 represents both a developmental disorder gene and potential oncogenic target in glioma therapy.