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

A continuous threat to cell function and viability is the contain components of the ubiquitin-
proteasome degraaccumulation in cells of proteins with highly abnormal dative pathway and …

The cause of neurodegeneration in Huntington's disease (HD) is unknown. Patients with HD
have an expanded NH2-terminal polyglutamine region in huntingtin. An NH2-terminal …

The action of tissue Transglutaminase1 (TGase) on specific protein-bound glutamine
residues plays a critical role in numerous biological processes2–5. Here we provide …

The data we report in this study concern the types, location, numbers, forms, and
composition of microscopic huntingtin aggregates in brain tissues from humans with different …

The mechanism of neurodegeneration in CAG/polyglutamine repeat expansion diseases is
unknown but is thought to occur at the protein level. Here, in studies of spinocerebellar …

The protein cross-linking enzyme tissue transglutaminase binds in vitro with high affinity to
fibronectin via its 42-kD gelatin-binding domain. Here we report that cell surface …

Cysteamine is an amino thiol with the chemical formula HSCH 2 CH 2 NH 2. Endogenously,
cysteamine is derived from coenzyme A degradation, although its plasma concentrations are …

Loss of neurotransmitter receptors, especially glutamate and dopamine receptors, is one of
the pathologic hallmarks of brains of patients with Huntington disease (HD). Transgenic …

Huntington's disease (HD) is an autosomal dominant neurodegenerative disorder caused by
polyglutamine expansion in the disease protein, huntingtin. In HD patients and transgenic …