Topic: Lamp2a

The primary and progressive degeneration of motor system is the main pathogenic process that characterizes amyotrophic lateral sclerosis (ALS), which is a fatal neurodegenerative disease of motor neurons (MN) in the primary motor cortex and spinal cord. Patients’ death occurs in 3–5 years after the diagnosis due to the progressive palsy and dysfunction of the respiratory system. The etiology is known in most of the patients with the most frequent sporadic ALS (90%), and clinical onset and symptoms are highly heterogeneous. Irrespective of the cause and initial onset the ALS is an almost select

Amyotrophic Lateral Sclerosis (ALS) is an almost selective motoneuron (MN) neurodegenerative disease in which the factors determining preferential involvement of MN are yet to be fully understood. TDP-43 nuclear clearance and aggregates are identified as a major component of the ubiquitinated neuronal cytoplasmic inclusions deposited in spinal motor neurons both in familiar and sALS patients. Since TDP43 protein contains the HSC70 recognition motif, and, therefore, chaperon mediated autophagy (CMA) function may represent a cellular mechanism to regulate TDP43 cytoplasmic turnover in cells. We