Amylotrophic Lateral Sclerosis-Like Motor Impairment in Prion Diseases

ECSU Author/Contributor (non-ECSU co-authors, if there are any, appear on document)
Eyualem Abebe, Professor (Creator)
Elizabeth City State University (ECSU )
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Abstract: Neurodegenerative diseases are collective diseases that affect different parts of the brain with common or distinct disease phenotype. In almost all of the Prion diseases, motor impairments that are characterized by motor derange- ment, apathy, ataxia, and myoclonus are documented and again are shared by motor neuron diseases (MND). Proteins such as; B-Cell lymphoma 2 (BCL2), Copper chaperone for superoxide dismutase (CCS), Amyloid beta precursor protein (APP), Amyloid Precursor-Like Protein1/2 (APLP1/2), Catalase (CAT), and Stress induced phosphoprotein 1 (STIP1), are common interactomes of Prion and superoxide dismutase 1 (SOD1). Although there is no strong evi- dence to show the interaction of SOD1 and Prion, the implicated common interacting proteins indicate the potential bilateral interaction of those pro- teins in health and disease. For example, down-regulation of Heat shock pro- tein A (HSPA5), a Prion interactome, increases accumulation of misfolded SOD1 leading to MND. Loss of Cu uptake function disturbs normal function of CCS. Over-expressed proteasome subunit alpha 3 (PSMA3) could fatigue its normal function of removing misfolded proteins. Studies showed the in- crease in CAT and lipid oxidation both in Prion-knocked out animal and in catalase deficiency cases. Up regulation, down regulation or direct interaction with their interactomes are predicted molecular mechanisms by which Prion and SOD exert their effect. The loss of protective function or the gain of a novel toxic property by the principal proteins is shared in Prion and MND. Thus, it might be possible to conclude that the interplay of proteins displayed in both diseases could be a key phenomenon in motor dysfunction development.

Additional Information

Language: English
Date: 2019
Prion, Super Oxide Dismutase-1, Amyotrophic Lateral Sclerosis, Motor

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