Fabry disease is an inborn error of glycosphingolipid metabolism caused by the deficiency of lysosomal a-galactosidase A (a-Gal A). We have established transgenic mice that exclusively express human mutant a-Gal A (R301Q) in an a-Gal A knock-out background (TgM/KO mice). This serves as a biochemical model to study and evaluate active-site specific chaperone (ASSC) therapy for Fabry disease, whi...

To improve surgical results of duodenal ulcer, transgastric myotomy (TGM) was added to the traditional selective proximal vagotomy (SPV) and its efficacy was evaluated clinically in 40 patients. 35 men and 5 women were involved, with a mean age of 33.07 +/- 14.25 years. Pyloroplasty was added in 12 operations for stenosis and perforation. 28 patients in this series underwent TGM with SPV withou...

Mice transgenic with the human poliovirus receptor gene develop clinical signs and neuropathology similar to those of human poliomyelitis when neurovirulent polioviruses are inoculated into the central nervous system (CNS). Factors contributing to disease severity and the frequencies of paralysis and mortality include the poliovirus strain, dose, and gender of the mouse inoculated. The more neu...

Total gaseous mercury (TGM) was monitored during 2013 at the rural monitoring site, Harwell, England using the Tekran 2537A monitoring system. Average TGM for the year was 1.45 ± 0.24 ng m(-3). This is comparable to other northern hemisphere studies, but on average 0.5 ng m(-3) higher than at its sister monitoring station at Auchencorth Moss, Scotland, but 14% lower than that found in a similar...

In this study, total gaseous mercury (TGM) concentrations were measured on Yongheung Island off the coast of Korea between mainland Korea and Eastern China in 2013. The purpose of this study was to qualitatively evaluate the impact of local mainland Korean sources and regional Chinese sources on local TGM concentrations using multiple tools including the relationship with other pollutants, mete...

C-type natriuretic peptide (CNP) plays a critical role in endochondral ossification through guanylyl cyclase-B (GC-B), a natriuretic peptide receptor subtype. Cartilage-specific overexpression of CNP enhances skeletal growth and rescues the dwarfism in a transgenic achondroplasia model with constitutive active mutation of fibroblast growth factor receptor-3. For future clinical application, the...

The weak expansive soil subgrade of the railway in operation is prone to settlement a high-temperature climate, which results rapid deterioration geometric dimensions track, and affecting safety train operation. This paper uses Track Geometry Measurements (TGM) analyze characteristics track geometry parameters soil. Also establishes Settlement Prediction Model (SPM) identify time predict value ...

The ability to stratify patients with pancreatic neuroendocrine tumors (p-NETs) into prognostic groups has been hindered by the absence of a commonly accepted staging system. Both the 7th tumor-node-metastasis (TNM) staging guidelines by the American Joint Committee on Cancer (AJCC) and the 2010 grading classifications by the World Health Organization (WHO) were validated to be unsatisfactory.W...

Abstract While research on the prevalence of co-occurring autism spectrum conditions (ASC) and trans gender modality (TGM) is available, less known about underlying mechanism this association. Insight needed to improve treatment autistic people. This review provides an overview theories ASC-TGM link available evidence for/against them published between January 2016 October 2020. A systematic se...