Biosynthesis of the polymeric gel-forming mucin MUC5B.

MUC5B is a major polymeric mucin in the airway mucus gel and is an essential component of innate defense of the respiratory epithelium. Knowledge of the synthesis and intracellular processing of MUC5B is incomplete. We investigated the molecular details of MUC5B assembly in primary human bronchial epithelial cells (HBECs) grown at an air-liquid interface (ALI).

A simple cell based assay to measure Parkin activity.

Parkin is an ubiquitin-protein ligase mutated in Autosomal Recessive - Juvenile Parkinsonism. Here, we describe a cell-based assay to measure Parkin's ubiquitin-protein ligase activity. It relies on the ability of Parkin to recognise depolarised mitochondria and exploits a cell line where Parkin expression is inducible.

Absence of alpha-synuclein affects dopamine metabolism and synaptic markers in the striatum of aging mice.

Despite numerous evidences for neurotoxicity of overexpressed alpha-synuclein, a protective function was suggested for endogenous alpha-synuclein and other members of the synuclein family. This protective role is most important for and evident in presynaptic terminals, where synucleins are normally accumulated. However, mice lacking synucleins display no adverse phenotype.

Alpha-synuclein and the pathogenesis of Parkinson's disease.

Lesions known as Lewy bodies (LBs) and Lewy neurites (LNs) characterise brains of Parkinson's disease (PD) patients. Intracellular aggregation of alpha-synuclein (alpha-syn) appears to play a key role in the generation of LBs and LNs. Such aggregation in the presence of redox metals may initiate Fenton reaction-mediated generation of reactive oxygen species (ROS).

Fe(II)-induced DNA damage in alpha-synuclein-transfected human dopaminergic BE(2)-M17 neuroblastoma cells: detection by the Comet assay.

Lewy bodies in the brains of patients with Parkinson's disease (PD) contain aggregates of alpha-synuclein (alpha-syn). Missense mutations (A53T or A30P) in the gene encoding alpha-syn are responsible for rare, inherited forms of PD. In this study, we explored the susceptibility of untransfected human dopaminergic BE(2)-M17 neuroblastoma cells, cells transfected with vector only, or cells transfected with wild-type alpha-syn, A30P alpha-syn or A53T alpha-syn to Fe(II)-induced DNA damage in the form of single-strand breaks (SSBs).