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Additional file 1: Figure S1. Transcriptome sequencing quality. Figure S2. Silence efficiency of CRISP1 at VIGS 10 dpi. Figure S3. Expression level of PR1a in response to CGMMV and melatonin treatment. Figure S4. Original picture for Fig.1. Figure S5. Original picture for Fig.2. Figure S6. Original picture for Fig.5. Figure S7. Original picture for Fig.7C. Figure S8. Original picture for Fig.7F.

Pyrophosphate synthetase-1(PRS-1) is a crucial enzyme that catalyzes the synthesis of phosphoribosylpyrophosphate (PRPP) with substrate: adenosine triphosphate (ATP) and ribose-5-phophate(R5P) in the de novo pathways of purine and pyrimidine nucleotide synthesis. Mutation in PRPS1 can result in a series of diseases of purine metabolism, which includes PRS-1 superactivity. The common clinical phenotypes are hyperuricemia and hyperuricosuria. We identified a novel missense mutation in X-chromosomal gene PRPS1 in a young Chinese woman while her mother has heterogeneous genotype and phenotype. A 24-year-old Chinese female patient suffered hyperuricemia, gout, and recurrent hyperpyrexia for more than 6 years, and then was diagnosed with hyperandrogenism, insulin resistance (IR), and polycystic ovary syndrome (PCOS). A novel missense mutation, c.521(exon)G>T, p.(Gly174Val) was detected by next-generation sequencing (NGS) and confirmed by Sanger sequencing in the patient and her parents. Interestingly, her mother has the same heterozygous missense mutation but without uric acid overproduction which can be explained by the phenomenon of the skewed X-chromosome inactivation. The substituted amino acid Val for Gly174 is positioned in the pyrophosphate (PPi) binding loop, and this mutation impacts the binding rate of Mg2+-ATP complex to PRS-1, thus the assembling of homodimer is affected by changed Val174 leading to the instability of the allosteric site. Our report highlights the X-linked inheritance of gout in females caused by mutation in PRPS1 accompanied with severe metabolic disorders and recurrent hyperpyrexia.

SMBI (supersonic molecular beam injection), as an effective fueling method for fusion plasmas, has been widely used on the HL-2A tokamak and other fusion devices. Two different types of SMBI system are now installed on HL-2A. One is an electromagnetic valve injector on the low field side (LFS), and the other is a pneumatic valve injector on the high field side (HFS). A new electron density record n?e = 4.7 ? 1019 m?3, which exceeds both the Greenwald density limit and the maximum density obtained by gas puffing (GP), was obtained on HL-2A with single-null-divertor operation. The HFS injection system is still under test, however, its outstanding fueling characteristics have already been observed, e.g. it has higher fueling efficiency compared to the LFS system. This excellent feature is still preserved during the process of ECRH.

Interactions between neurons and glial cells in the brain have important roles in brain functions such as development and plasticity of neural circuits or functions. Glial cells are much more actively involved in brain functions than previously thought. Here, we used vibrissal stimuli to induce sensoryevoked responses and multiunit spikes in the contralateral barrel cortex in a rat model. Local application of the gliotoxin DL-alpha-aminoadipate (AA) revealed that glial cells were involved in the sensoryevoked responses. The increases in the amplitude of somatosensory-evoked potential (SEP) and multiunit sensory-evoked spike rates in barrel cortex after AA injection were dramatic. Immunohistochemical staining of brain lipid binding protein (BLBP) and NeuN showed AA decreased cell number of astrocytes but not neurons in the barrel cortex. In conclusion, our results suggested an important role for astrocyte metabolism in normal synaptic activities.

There is intensive gap-junctional coupling between glial processes, but their significance in sensory functions remains unknown. Connexin-43 (Cx43), a major component of astrocytic gap-junction channels, is abundantly expressed in astrocytes. To investigate the role of Cx43-mediated gap junctions between astrocytes in sensory functions, we generated Cx43 knockout (KO) mice with a mouse line carrying loxP sites flanking exon 2 of the Cx43 gene and the transgenic line expressing Cre recombinase under control of the glial fibrillary acidic protein promoter, which exhibited a significant loss of Cx43 in astrocytes in the barrel cortex. Although Cx43 expression between the astrocytes measured by immunohistochemistry was virtually abolished in Cx43 KO mice, they had normal architecture in the barrel cortex but the intensity of cytochrome oxide histochemistry decreased significantly. In vivo electrophysiological analysis revealed that the long-term potentiation of the vibrissal evoked responses in the barrel cortex evoked by high-frequency rhythmic vibrissal stimuli (100 Hz, 1 s) was abolished in Cx43 KO mice. Current source density analysis also revealed that astrocytic Cx43 was important to the flow of excitation within the laminar connections in barrel cortex. Behavioral tests showed that the ability of Cx43 KO mice to sense the environment with their whiskers decreased. Even so, the jump-stand experiment showed that they could still discriminate rough from smooth surfaces. Our findings suggest that Cx43-mediated gap-junctional coupling between astrocytes is important in the neuron-glia interactions required for whisker-related sensory functions and plasticity.

A recent improvements in charge exchange recombination spectroscopy (CXRS) diagnostic operated at the tokamak HL-2A is reported. With the presented diagnostic half the plasma minor radius at a spatial resolution of ~1.3 cm is covered and temporal resolution is 11 ms. Two spectrometers operating at different wavelengths for impurity ion and beam neutrals emission. Some first results are presented.

Gap junction is the aggregate of some intercellular channels, which allows ions and small molecules to transport or transfer between cells. There are about 20 proposed members of the connexin family found in mammalian tissues now, and more than 10 reported are expressed in the nervous system. The astrocytes and oligodendrocytes express some specific connexins. In the present article, we review the recent literatures to illustrate the importance of gap junction for the intercellular communication between glial cells, astrocytes and neurons, and neuronal cells, which is crucial for brain functions.

HL-2A plasmas heated by neutral beam injection (NBI) regularly exhibit n = 1 long-lived saturated magnetohydrodynamic instabilities. A reduction in the electron density and plasma stored energy and an increase in fast ion losses are usually observed in the presence of such perturbations. The observed long-lived saturated internal mode (LLM) occurs when the safety factor profile has a weak shear in a broad range of the plasma centre with qmin around unity. It is found that the ideal interchange mode can become marginally stable due to the weak magnetic shear reaching a critical value. The LLM, due to its pressure-driven feature, is destabilized by the strong interaction with fast ions in the low-shear region during the NBI. Furthermore, for the first time it is clearly observed that the LLMs can be suppressed by electron cyclotron resonant heating (ECRH), or by supersonic molecular beam injection in HL-2A plasmas. Low-n sidebands observed during the LLM are also suppressed by increasing the ECRH power. The control of LLMs is due to the change in the magnetic shear or in the pressure profile induced by the local heating or fuelling.