Mammalian cells remove misfolded proteins using various proteolytic systems, including the
ubiquitin (Ub)-proteasome system (UPS), chaperone mediated autophagy (CMA) and …

Selective degeneration and death of one or more classes of neurons is the defining feature
of human neurodegenerative disease. Although traditionally viewed as diseases mainly …

The ubiquitin-proteasome system targets numerous cellular proteins for degradation. In
addition, modifications by ubiquitin-like proteins as well as proteins containing ubiquitin …

The ubiquitin–proteasome pathway (UPP) is the primary cytosolic proteolytic machinery for
the selective degradation of various forms of damaged proteins. Thus, the UPP is an …

▪ Abstract Copper-zinc superoxide dismutase (CuZnSOD, SOD1 protein) is an abundant
copper-and zinc-containing protein that is present in the cytosol, nucleus, peroxisomes, and …

Amyotrophic lateral sclerosis (ALS) is a progressive degenerative disease of motor neurons.
The inherited form of the disease, familial ALS, represents 5–10% of the total cases, and the …

Deposition of aggregated protein into neurofilament-rich cytoplasmic inclusion bodies is a
common cytopathological feature of neurodegenerative disease. How—or indeed whether …

The mechanisms leading to neurodegeneration in ALS (amyotrophic lateral sclerosis) are
not well understood, but cytosolic protein aggregates appear to be common in sporadic and …

The molecular mechanisms by which mutations in the gene for Cu/Zn superoxide dismutase
(SOD1) lead to the selective death of motor neurones in familial amyotrophic lateral …

The inherited retinal degenerations are typified by retinitis pigmentosa (RP), a
heterogeneous group of inherited disorders that causes the destruction of photoreceptor …