Mitochondrial dysfunction is a common feature of C9orf72 amyotrophic lateral sclerosis/
frontotemporal dementia (ALS/FTD); however, it remains unclear whether this is a cause or …

Somatic maintenance and cell survival rely on proper protein homeostasis to ensure reliable
functions across the cell and to prevent proteome collapse. Maintaining protein folding and …

A fundamental question regarding the etiology of amyotrophic lateral sclerosis (ALS) is
whether the various gene mutations associated with the disease converge on a single …

The abnormal repetition of the hexanucleotide GGGGCC within the C9orf72 gene is the
most common genetic cause of both Amyotrophic Lateral Sclerosis (ALS) and …

Mitochondrial dysfunction such as excess production of reactive oxygen species (ROS) and
defective mitochondrial dynamics are common features of C9orf72 Amyotrophic Lateral …

Many human diseases are associated with the expansion of repeat sequences within the
genes. It has become clear that expressed disease transcripts bearing such long repeats …

In 2011, a hexanucleotide repeat expansion (HRE) in the noncoding region of C9orf72 was
associated with the most frequent genetic cause of frontotemporal dementia (FTD) and …

The most common genetic cause of familial and sporadic amyotrophic lateral sclerosis (ALS)
is a GGGGCC hexanucleotide repeat expansion (HRE) in the C9orf72 gene. While direct …

The degeneration of axons and their terminals occurs following traumatic, toxic, or
genetically-induced insults. Common molecular mechanisms unite these disparate triggers …

Amyotrophic lateral sclerosis is a devastating neurodegenerative disease with a complex
genetic basis, presenting both in familial and sporadic forms. The hexanucleotide (G 4 C 2) …