Proteostasis in Huntington's disease: disease mechanisms and therapeutic opportunities.

Many neurodegenerative diseases are characterized by impairment of protein quality control mechanisms in neuronal cells. Ineffective clearance of misfolded proteins by the proteasome, autophagy pathways and exocytosis leads to accumulation of toxic protein oligomers and aggregates in neurons. Toxic protein species affect various cellular functions resulting in the development of a spectrum of different neurodegenerative proteinopathies, including Huntington's disease (HD).

Two new xanthones from Lomatogonium carinthiacum.

Aim: To study the chemical constituents of Lomatogonium carinthiacum (Wulfen) Rchb.

Method: The CHCl3-soluble fraction was separated by chromatography and the structures of the new compounds were elucidated by spectral experiments.

Results: Two new xanthones, 1, 8-dihydroxy-4, 5-dimethoxy-6, 7-methylenedioxyxanthone (1), 1, 4, 8-trimethoxyxanthone-6-O-β-D-glucoronyl-(1→6)O-β-D-glucoside (2) were isolated from the whole plant of Lomatogonium carinthiacum.

Activity difference between alpha-COOH and beta-COOH in N-phosphorylaspartic acids.

N-phosphorylamino acids are chemically active species that have many biomimic activities. alpha-COOH in amino acids and peptides behaviors rather differently than beta-COOH in many biochemical processes and takes a more important role in the origin of life. Activity differences between alpha-COOH and beta-COOH in the peptide formation of phosphoryl amino acids are studied by 1D, 2D NMR techniques and by ab initio and density functional theory (DFT) calculations in this paper.