Carrier Rate and Mutant Allele Frequency of GM1 Gangliosidosis in Miniature Shiba Inus (Mame Shiba): Population Screening of Breeding Dogs in Japan.

GM1 gangliosidosis is a progressive, recessive, autosomal, neurodegenerative, lysosomal storage disorder that affects the brain and multiple systemic organs due to an acid β-galactosidase deficiency encoded by the gene. This disease occurs in the Shiba Inu breed, which is one of the most popular traditional breeds in Japan, due to the :c.1649delC (p.

2D electron temperature diagnostic using soft x-ray imaging technique.

We have developed a two-dimensional (2D) electron temperature (T(e)) diagnostic system for thermal structure studies in a low-aspect-ratio reversed field pinch (RFP). The system consists of a soft x-ray (SXR) camera with two pin holes for two-kinds of absorber foils, combined with a high-speed camera. Two SXR images with almost the same viewing area are formed through different absorber foils on a single micro-channel plate (MCP).

Tangential soft-x ray imaging for three-dimensional structural studies in a reversed field pinch.

Tangential soft-x ray (SXR) imaging diagnostic has been developed and three-dimensional (3D) structure of the internal magnetic surface has been deduced by comparing the experimental and calculated two-dimensional SXR images in a reversed field pinch. The SXR imaging system, consisting of a MCP, a fluorescent plate, and an intensified charge coupled device camera, has been installed in REversed field pinch of Low-Aspect-ratio eXperiment (RELAX) machine. Major characteristics of an experimental SXR image could be reproduced by numerical calculations of the image using a single island model, suggesting a helical hot core in RELAX.

Self-reversal phenomena of toroidal current by reversing the external toroidal field in helicity-driven toroidal plasmas.

In order to understand self-organization in helicity-driven systems, we have investigated the dynamics of low-aspect-ratio toroidal plasmas by decreasing the external toroidal field and reversing its sign in time. Consequently, we have discovered that the helicity-driven toroidal plasma relaxes towards the flipped state. Surprisingly, it has been observed that not only toroidal flux but also poloidal flux reverses sign spontaneously during the relaxation process.

Mechanism of the double aldol reaction: the first spectroscopic characterization of a carbon-bound boron enolate derived from carboxylic esters.

The novel doubly borylated enolate is identified as an intermediate of the double aldol reaction of acetate esters. As a precursor to the formation of the doubly borylated enolate, carbon-bound boron enolates of carboxylic esters are spectroscopically characterized for the first time. When 2,6-diisopropylphenyl acetate (10d) is treated with c-Hex(2)BOTf (1.