Mucopolysaccharidosis VI (MPS VI) is an autosomal recessive lysosomal storage disease caused by mutations in the arylsulfatase B gene (ARSB) and consequent deficient activity of ARSB, a lysosomal enzyme involved in glycosaminoglycan (s) (GAGs) metabolism. Here we present the results study of ARSB DNA analysis in MPS VI patients in the Russian Federation (RF) and other republics of Former Soviet Union. In a cohort of 68 patients (57 families) with MPS VI, a total of 28 different pathogenic alleles were found. The most prevalent nucleotide changes included c.194C>T (Ser65Phe) and c.454C>T (Arg152Trp). Five pathogenic alleles were novel, not previously reported (с.304C>G, c.533A>G, c.941T>C, c.447 456del10, С.990 1003del14). The nucleotide variant c.454C>T (Arg152Trp) was prevalent allele among Slavic Russian patients. The nucleotide variant c.194C>T (Ser65Phe) was found only in MPS VI families from the Republic of Dagestan. Based on the analysis of dry blood spots
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Industrial Workhorses 20 May 2021
Stuart Anderson, president of Chortsey Barr Associates discusses business and technical developments in the pick-and-carry mobile crane industry and analyses trends in the fast-growing Italian market.
Almost every European and American mobile crane manufacturer of any note had its beginnings building industrial mobile or ‘yard’ cranes of one variety or another. Time was when there were well in excess of 100 producers.
It will surprise some to know that even today there are around 40 manufacturers of hydraulic telescopic boom pick-and-carry cranes but in contrast with former times, its an industry where the manufacturers are largely-concentrated in just two countries – Italy and the U.S.
Cometto SPMTs played a key role in the installation of an 820t steel bridge for a railway project in Germany.
The bridge needed to be positioned at the junction in Wendlingen, as part of the construction of the ICE railway line between Stuttgart and Ulm.
This job required the simultaneous use of three systems. Marco Wilhelm, project manager from Wiesbauer, explained: We had to coordinate the Cometto SPMT, the slipway from Schachtbau Nordhausen and our 1000t crawler crane.
Apart from the 12 Cometto axle lines and two power packs from Wiesbauer s fleet, 12 MSPE units from Schares of Bocholt were also deployed. Wiesbauer coupled the SPMT combination in a 12+12 side-by-side arrangement.