Ubiquitin-positive, tau- and α-synuclein–negative inclusions are hallmarks of frontotemporal
lobar degeneration with ubiquitin-positive inclusions and amyotrophic lateral sclerosis. …

Expansion of a GGGGCC hexanucleotide repeat upstream of the C9orf72 coding region is the
most common cause of familial frontotemporal lobar degeneration and amyotrophic lateral …

Objective Amyotrophic lateral sclerosis (ALS) is a common, fatal motor neuron disorder with
no effective treatment. Approximately 10% of cases are familial ALS (FALS), and the most …

The amyloid precursor protein (APP) is critical in the pathogenesis of Alzheimer's disease.
The question of its normal biological function in neurons, in which it is predominantly located …

Frontotemporal dementia (FTD) is a clinical syndrome with a heterogeneous molecular
basis. The neuropathology associated with most FTD is characterized by abnormal cellular …

Mutations in the α-synuclein (αSYN) gene are associated with rare cases of familial Parkinson's
disease, and αSYN is a major component of Lewy bodies and Lewy neurites. Here we …

Scrapie is a slow degenerative encephalopathy of animals caused by unusual infectious
particles termed prions. A cDNA encoding the only apparent component of the prion, a protein …

Reports on the structure/function of an alpha-synuclein in health and disease and the
rational development of animal models for Parkinson's and related diseases. Amino-terminal …

The cellular function of the prion protein (PrP C ), a cell surface glycoprotein expressed in
neurones and astrocytes, has not been elucidated. Cell culture experiments reveal that …

Frontotemporal lobar degeneration (FTLD) is the second most common cause of presenile
dementia. The predominant neuropathology is FTLD with TAR DNA-binding protein (TDP-43) …