Selective Neuromuscular Denervation in Taiwanese Severe SMA Mouse Can Be Reversed by Morpholino Antisense Oligonucleotides.

Spinal muscular atrophy (SMA) is an autosomal recessive motor neuron disease caused by deficiency of the survival of motor neuron (SMN) protein, which leads to synaptic defects and spinal motor neuron death. Neuromuscular junction (NMJ) abnormalities have been found to be involved in SMA pathogenesis in the SMNΔ7 SMA mouse model. However, whether similar NMJ pathological findings present in another commonly used mouse model, the Taiwanese SMA mouse, has not been fully investigated. To examine the NMJs of the Taiwanese severe SMA mouse model (Smn-/-; SMN2tg/0), which is characterized by severe phenotype and death before postnatal day (P) 9, we investigated 25 axial and appendicular muscles from P1 to P9. We labelled the muscles with anti-neurofilament and anti-synaptophysin antibodies for nerve terminals and α-bungarotoxin for acetylcholine receptors (AChRs). We found that severe NMJ denervation (<50% fully innervated endplates) selectively occurred in the flexor digitorum brevis 2 and 3 (FDB-2/3) muscles from P5, and an increased percentage of fully denervated endplates correlated with SMA progression. Furthermore, synaptophysin signals were absent at the endplate compared to control littermate mice, suggesting that vesicle transport might only be affected at the end stage. Subsequently, we treated the Taiwanese severe SMA mice with morpholino (MO) antisense oligonucleotides (80 µg/g) via subcutaneous injection at P0. We found that MO significantly reversed the NMJ denervation in FDB-2/3 muscles and extended the survival of Taiwanese severe SMA mice. We conclude that early NMJ denervation in the FDB-2/3 muscles of Taiwanese severe SMA mice can be reversed by MO treatment. The FDB-2/3 muscles of Taiwanese severe SMA mice provide a very sensitive platform for assessing the effectiveness of drug treatments in SMA preclinical studies.

[Cited status and its correlated factors of articles published in Chinese Journal of Pediatrics from 2001 to 2010].

OBJECTIVE: The present study was designed to explore the cited status and its correlated factors of articles published in Chinese Journal of Pediatrics. METHOD: Articles published in Chinese Journal of Pediatrics from 2001 to 2010 were searched using Wanfang Medical Online database, and the relationship between cited number and column and funding status were analyzed. RESULTS: Totally 3209 articles were published by Chinese Journal of Pediatrics from 2001 to 2010. Two thousand and seventy-three articles (64.60%) were cited. The total cited number was 18 546 (mean rate: 5.78 per paper). Standard/protocol/guideline was the most often cited column (mean rate: 62.92 per paper). Featured articles including editorials (mean rate: 7.12 per paper), special articles (mean rate: 6.50 per paper) and original articles (mean rate: 7.90 per paper) had higher cited rate. Cited rate of original papers in featured articles was higher than other original articles (mean rate: 6.03 per paper). Those with academic perspectives, such as Opinion/Debate/Discussion, were well cited (mean rate: 7.09 per paper). All of original articles in Rapid Pathway were well cited (mean rate: 16.20 per paper). Mean cited number of grant-supported articles was 4.81 per paper, lower than that of all kinds of papers (mean rate: 5.78 per paper) and non-grant-supported articles (mean rate: 6.06 per paper). However, it was slightly higher than that of articles except Standard/protocol/guideline (mean rate: 4.36 per paper). CONCLUSION: Cited status varies among columns. The invited papers should be increased in number to raise commentary papers and original articles with high quality.Grant-supported or non-grant-supported papers should be reviewed based on the same standard after submission.

Morphology, crystal structure and adsorption performance of hydrothermally synthesized titania and titanate nanostructures.

Titania (TiO2) and sodium titanate nanostructures with controllable phases and morphologies were synthesized by a hydrothermal method with titanium disulfide (TiS2) as the starting material. Sodium titanate nanobelts could be synthesized under a relatively low alkaline concentration (1 mol L(-1) NaOH) and short duration (6 h). At 3 mol L(-1) HCl, rutile TiO2 nanorods were synthesized. Anatase TiO2 nanoparticles were obtained at pH values ranging between 2 and 13. FTIR analysis confirmed the phase change as the pH of the reacting medium increased from highly acidic to highly alkaline. The adsorption of Methylene Blue (MB) on the as-synthesized sodium titanate nanobelts fitted well with the Langmuir monolayer model, with an adsorption capacity as high as 312.5 mg g(-1). The kinetics of MB adsorption was found to be a pseudo-second-order kinetic model. In brief, this study demonstrates a simple method to control the phase and morphology of titanium-based oxides. Excellent performance has been shown in the MB adsorption test by the sodium titanate nanostructures.

Magneto-optic microscope for visually detecting subsurface defects.

A visual test method for detecting microdefects under fine surfaces is described. A new MO microscope that has a laser source, a CCD camera, and an exciting coil is developed for this work. A pulse generator supplies an intermittent square pulse to the exciting coil, which can intensify eddy currents yet reduce the working temperature of the exciting coil and sample. The magnetic field variation produced by the imbedded defect causes a rotation of the polarization plane of the reflected beam. Therefore the reflected beam carries an image of the defect, which is received by a CCD camera. The optical arrangement guarantees that no light is reflected back to the laser. The system was tested with a calibrator, which has an artificial subsurface defect; such a test attains a visual detected image. To our knowledge this is the first time an image of a subsurface defect has been distinctly detected with a MO sensor system.

[Effects of nitric oxide synthase inhibitor on dentate gyrus neurogenesis after diffuse brain injury in adult rats].

OBJECTIVE: To investigate the effects of selective nitric oxide synthase (NOS) inhibitors on dentate gyrus (DG) neurogenesis after diffuse brain injury (DBI) in adult rats. METHODS: DBI models were established in adult male SD rats, followed by systemic bromodeoxyuridine (BrdU) labeling of the dividing cells and immunohistochemical assay of the proliferation rates of neural precursor cells in the DG for comparison between NOS inhibitor (7-nitroindazole and Aminoguanidine) groups and the corresponding control groups at various time points after DBI. RESULTS: Intraperitoneal administration of 7-nitroindazole significantly reduced the number of BrdU-labeled cells in the DG of adult rats 2, 4 and 6 d after DBI (P<0.05). Aminoguanidine also significantly inhibited the proliferation of neural precursor cells in the DG induced by DBI at various time points (P<0.01). CONCLUSIONS: The activation of NOS after DBI may be an important regulatory factor for DG neurogenesis in adult rats, and NO generated by nNOS is probably involved mainly in the early stage of enhanced neurogenesis after DBI, while the NO from iNOS might participated primarily in the later stages.