GRN and hyperinsulinemic hypoglycemia, familial, 4: In this work, we have investigated the effect of PGRN deficit on mitochondrial function, dynamics and degradation, using a cellular model of FTLD-TDP with PGRN deficiency (SH-SY5Y GRN KD cells) and lymphoblasts from FTLD-TDP patients carrying a LOF mutation in the GRN gene (c.709-1G > A).