Perturbations of the proteome in primary astrocytes from the hSOD1(G93A) ALS mouse model

ABSTRACT: Amyotrophic lateral sclerosis (ALS) is a progressive neurodegenerative disease whose pathophysiology is largely unknown. Despite motor neuron death is recognized as the key event in ALS, astrocytes dysfunctionalities and neuroinflammation were demonstrated to accompany and probably even drive motor neuron loss. Nevertheless, the mechanisms priming astrocyte failure and hyperactivation are still obscure. In this work, altered pathways in ALS astrocytes were unveiled by investigating the proteomic profile of primary spinal-cord astrocytes derived from transgenic ALS mouse model overexpressing the human (h)SOD1(G93A) protein, in comparison with the transgenic counterpart expressing hSOD1(WT) protein. In this research, we showed that hSOD1(G93A) astrocytes present a profound alterations in the expression of proteins involved in proteostasis and glutathione metabolism.

INSTRUMENT(S): Q Exactive

ORGANISM(S): Mus Musculus (mouse)

TISSUE(S): Astrocyte Of The Spinal Cord, Primary Cell

DISEASE(S): Amyotrophic Lateral Sclerosis

SUBMITTER: Roberto Stella  

LAB HEAD: Alessandro Bertoli

PROVIDER: PXD026576 | Pride | 2022-02-17

REPOSITORIES: Pride