Anti-NCL monoclonal antibody, clone 475-6 [Alexa Fluor® 488]

The neuronal ceroid lipofuscinoses (NCL) are a group of disorders defined by shared clinical and pathological features, including seizures and progressive decline in vision, neurocognition, and motor functioning, as well as accumulation of autofluorescent lysosomal storage material, or 'ceroid lipofuscin'. Research has revealed thirteen distinct genetic subtypes. Precisely how the gene mutations lead to the clinical phenotype is still incompletely understood, but recent research progress is starting to shed light on disease mechanisms, in both gene-specific and shared pathways. As the application of new sequencing technologies to genetic disease diagnosis has grown, so too has the spectrum of clinical phenotypes caused by mutations in the NCL genes. Most genes causing NCL have probably been identified, underscoring the need for a shift towards applying genomics approaches to achieve a deeper understanding of the molecular basis of the NCLs and related disorders. Here, we summarize the current understanding of the thirteen identified NCL genes and the proteins they encode, touching upon the spectrum of clinical manifestations linked to each of the genes, and we highlight recent progress leading to a broader understanding of key pathways involved in NCL disease pathogenesis and commonalities with other neurodegenerative diseases.

To develop a process for simultaneous extraction and recovery of nitric acid and gold from gold-bearing aqua regia solutions, solvent extraction study was conducted by using tributyl phosphate (TBP) as the extractant. The extraction behaviors of acid from the pure aqua regia, nitric acid, and hydrochloric acid were investigated with varying concentration of extractant, and the concentration ratio of HNO3 and NCl. The simultaneous recovery of HNO3 and Au with the recycling of HCl from the gold-bearing aqua regia solutions was also examined. Selective stripping was then carried out for co-extracted HNO3 and Au. Equilibrium conditions for HNO3 and Au recovery optimized from the experimental results have been found to be: extraction using 80 vol% TBP at O/A ratio of 1, four contacts of stripping for HNO3 at O/A ratio of 1 using distilled water, and two contacts of stripping for Au at an O/A ratio of 1 using a mixed solution at 1:1 (vol.) ratio of 0.1 M sodium thiosulfate (Na2O2S3) and 0.1 M ammonium hydroxide (NH4OH). It was thus possible to simultaneously recover HNO3 and Au from the gold bearing aqua regia solution through co-extraction and selective stripping of HNO3 and Au leaving HCl in the raffinate for recycling.