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 selective motoneuron (MN) neurodegenerative disease in which the factors determining preferential involvement of MN are yet to be fully understood. Therefore, to improve our understanding of ALS selective pathogenesis of human MNs it is necessary to develop more adequate experimental models. Human adipose-derived stem cells (hADSCs) isolated from an sALS donor, and a control donor were induced into neural stem cells (iNSCs) using a 3-step NSC induction protocol Park et al., 2017). These iNSCs cells derived from hADSCs develop neuronal morphology and express the Choline acetyltransferase (ChAT), that is a characteristic marker of the spinal cord motoneurons, in both sALS and control derived neuros. Now, we are studying the reproduction of pathogenic processes described in ALS motoneurons in our adipose-derived motoneurons model using markers such as TDP43 to determine the presence of sALS characteristic proteinopathy, Lamp2a and LC3 to analyze autophagy, as a possible mechanism underlaying selective pathogeny of ALS in motoneurons.