Integrated analysis of transcriptomic and proteomic alterations in mouse models of ALS/FTD identify early metabolic adaptions with similarities to mitochondrial dysfunction disorders

• Published in: Amyotrophic lateral sclerosis and frontotemporal degeneration Vol. 25; no. 1-2; p. 135
• Main Authors: Matveeva, Anna, Watters, Orla, Rukhadze, Ani, Khemka, Niraj, Gentile, Debora, Perez, Ivan Fernandez, Llorente-Folch, Irene, Farrell, Cliona, Lo Cacciato, Elide, Jackson, Joshua, Piazzesi, Antonia, Wischhof, Lena, Woods, Ina, Halang, Luise, Hogg, Marion, Muñoz, Amaya Garcia, Dillon, Eugène T, Matallanas, David, Arijs, Ingrid, Lambrechts, Diether, Bano, Daniele, Connolly, Niamh M C, Prehn, Jochen H M
• Format: Journal Article
• Language: English
• Published: England 01.02.2024
• Subjects: Amyotrophic Lateral Sclerosis - pathology; Animals; Frontotemporal Dementia - metabolism; Gene Expression Profiling; Humans; Mice; Mice, Transgenic; Mitochondrial Diseases; Neurodegenerative Diseases; Pick Disease of the Brain; Proteomics; RNA, Messenger; frontotemporal dementia (FTD); master regulator analysis; mitochondrial dysfunction; Amyotrophic lateral sclerosis (ALS); bioinformatics; lipid metabolism

Sporadic and familial amyotrophic lateral sclerosis (ALS) is a fatal progressive neurodegenerative disease that results in loss of motor neurons and, in some patients, associates with frontotemporal dementia (FTD). Apart from the accumulation of proteinaceous deposits, emerging literature indicates...