Pathological mechanisms underlying TDP-43 driven neurodegeneration in FTLD-ALS spectrum disorders.

Abstract excerpt

Aggregation of misfolded TAR DNA-binding protein 43 (TDP-43) is a striking hallmark of neurodegenerative processes that are observed in several neurological disorders, and in particular in most patients diagnosed with frontotemporal lobar degeneration (FTLD) or amyotrophic lateral sclerosis (ALS). A direct causal link with TDP-43 brain proteinopathy was provided by the identification of pathogenic mutations in...

Topics

• Amyotrophic Lateral Sclerosis
• Animals
• Brain
• C9orf72 Protein
• DNA-Binding Proteins
• Frontotemporal Lobar Degeneration
• Genetic Variation
• Humans
• Mutation
• Proteins
• TDP-43 Proteinopathies