Mutations in the autophagy gene WDR45 cause -propeller protein-associated neurodegeneration (BPAN); however the molecular and cellular mechanism of the disease process is largely unknown. Here we generated constitutive Wdr45 knockout (KO) mice that displayed cognitive impairments, abnormal synaptic transmission and lesions in hippocampus and basal ganglia. Immunohistochemistry analysis shows loss of neurons in prefrontal cortex and basal ganglion in aged mice, and increased apoptosis in these regions, recapitulating a hallmark of neurodegeneration. Quantitative proteomic analysis shows accumulation of endoplasmic reticulum (ER) proteins in KO mouse. Furthermore, we show that a defect in autophagy results in impaired ER turnover and ER stress. The unfolded protein response (UPR) is elevated through IRE1 and possibly other kinase signaling pathways, and eventually leads to neuronal apoptosis. Suppression of ER stress, or activation of autophagy through inhibition of mTOR pathway rescues neuronal death. Thus, our study not only provides mechanistic insights for BPAN, but also suggests that a defect in macroautophagy machinery leads to impairment in selective organelle autophagy.

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A recombinant laccase from Trametes versicolor in Pichia methanolica was produced constitutively in a defined medium. The recombinant laccase was purified using ultrafiltration, anion-exchange chromatography, and gel filtration. The molecular weight of the purified laccase was estimated as 64 kDa by SDS-PAGE. The purified recombinant laccase decolorized more than 90% of Remazol Brilliant Blue R (RBBR) initially at 80 mg l(-1) after 16 h at 45 degrees C and pH 5 when 25 U laccase ml(-1) was used. The purified recombinant laccase could efficiently decolorize RBBR without additional redox mediators.

Waste white foam, toxic dyes, and metal ions are difficult to degrade in the ecological environment, posing a serious threat to human health and natural beauty, it is of great significance to design a functional material to jointly solve these problems. This study provided a facile approach to convert waste polystyrene foam into a new acid anhydride modified polystyrene adsorbent (PS-MA) for the dyes and heavy metals removal from aqueous solution. The as-obtained absorbent was characterized by Fourier transform infrared spectroscopy, thermogravimetry, scanning electron microscopy, energy dispersive spectroscopy, Brunauer Emmett Teller method, and X-ray photoelectron spectroscopy. Influencing factors are including, pH, dye concentration, contact time and temperature on the adsorption of methylene blue (MB), safranine T (ST), and Cu(II) were systematically studied. Boehm titration showed that the content of surface carboxyl groups reached 4.53 mmol/g. The maximum adsorption capacity had reached 1405.37 mg/g for MB, 2269.33 mg/g for ST, 65.03 mg/g for Cu(II). Moreover, the kinetic data were in good agreement with the pseudo-second-order model and closed to adsorption equilibrium at 20 min. After five times of recycling experiments and the application of mixed dye wastewater with salinity, PS-MA showed excellent stability and removal ability. Abundant efforts revealed a broad application prospect of PS-MA materials in water remediation.