MCOLN1 and amyotrophic lateral sclerosis: Abnormal autophagy resulting from disrupted lysosomal calcium homeostasis is considered highly significant in the pathogenesis of ALS.[12] In a neuronal model of the ALS/Parkinson-Dementia complex, established by exposure to the neurotoxin β-methylamino-L-alanine (L-BMAA), it was observed that TRPML1 expression was decreased, leading to impaired calcium homeostasis in both lysosomes and the ER.