Gómez-Almería M, Burgaz S, Costas-Insua C, Rodríguez-Cueto C, Santos-García I, Rodríguez-Crespo I, García C, Guzmán M, de Lago E, Fernández-Ruiz J.

BiP Heterozigosity Aggravates Pathological Deterioration in Experimental Amyotrophic Lateral Sclerosis

Int J Mol Sci. 2021 Nov 20;22(22):12533

DOI: 10.3390/ijms222212533

RESUMEN

BiP is a molecular chaperone that aids in protein folding crucial for cellular homeostasis. Since most neurodegenerative diseases involve protein aggregation events, we asked if BiP deficiency worsens the course of amyotrophic lateral sclerosis (ALS) and Parkinson’s disease (PD). Using an in vivo loss of function approach, we have found that BiP deficiency accelerates the decline observed in ALS, whereas the inactivation of BiP in a toxin-based PD model did not increase the susceptibility to the disease. Thus, BiP could represent a novel pharmacological target for ALS.