Ablation of the scaffold protein JLP causes reduced fertility in male mice

金沢大学がん研究所がん分子細胞制御The specific and efficient activation of mitogen-activated protein kinase (MAPK) signaling modules is mediated, at least in part, by scaffold proteins. c-Jun NH2-terminal kinase (JNK)-associated leucine zipper protein (JLP) was identified as a scaffold protein for JNK and p38 MAPK signaling modules. JLP is expressed nearly ubiquitously and is involved in intracellular signaling pathways, such as the Gα13 and Cdo-mediated pathway, in vitro. To date, however, JLP expression has not been analyzed in detail, nor are its physiological functions well understood. Here we investigated the expression of JLP in the mouse testis during development. Of the tissues examined, JLP was strongest in the testis, with the most intense staining in the elongated spermatids. Since the anti-JLP antibody used in this study can recognize both JLP and sperm-associated antigen 9 (SPAG9), a splice variant of JLP that has been studied extensively in primates, we also examined its expression in macaque testis samples. Our results indicated that in mouse and primate testis, the isoform expressed at the highest level was JLP, not SPAG9. We also investigated the function of JLP by disrupting the Jlp gene in mice, and found that the male homozygotes were subfertile. Taken together, these observations may suggest that JLP plays an important role in testis during development, especially in the production of functionally normal spermatozoa. © 2008 Springer Science+Business Media B.V

Relationship between monoclonal gammopathy of undetermined significance and radiation exposure in Nagasaki atomic bomb survivors.

Radiation exposure is a possible predisposing factor for monoclonal gammopathy of undetermined significance (MGUS), but the association has been uncertain. We investigated the relationship between radiation exposure and MGUS prevalence by using data from the M-protein screening for Nagasaki atomic bomb survivors between 1988 and 2004. Radiation exposure was assessed by exposure distance from the hypocenter and exposure radiation dose. We computed prevalence ratios (PRs) and the 95% confidence intervals (CIs) adjusting for exposure age and sex. A total of 1082 cases of MGUS were identified from 52 525 participants. MGUS prevalence was significantly higher in people exposed at distance within 1.5 km than beyond 3.0 km (PR, 1.4; 95% CI, 1.1-1.9) among those exposed at age 20 years or younger, but it was not found among those exposed at age 20 years or older. MGUS prevalence was also significantly higher in people exposed to more than 0.1 Gy than those exposed to less than 0.01 Gy (PR, 1.7; 95% CI, 1.0-2.8) among those exposed at age 20 years or younger. Thus, people exposed at younger age exhibited a significantly high risk of MGUS when exposed to a high radiation dose. There was no clear association between radiation exposure and the malignant progression of MGUS. Further detailed analysis is needed

Swallowing function improvement using costal cartilage for severe dysphagia after supracricoid hemilaryngopharyngectomy

Supracricoid hemilaryngopharyngectomy (SCHLP) is a laryngeal preservation surgery for hypopharyngeal carcinoma confined to the pyriform sinus or laryngeal carcinoma with arytenoid infiltration. Postoperative dysphagia is inevitable, but both voice and swallowing functions can be preserved. Here, we present a case of severe dysphagia secondary to unexpected postoperative tissue loss and scarring. A 67-year-old man underwent left SCHLP for hypopharyngeal cancer, and on the third postoperative day, he developed necrotizing fasciitis of the left neck. He was unable to swallow and was transferred to a rehabilitation hospital 90 days after the surgery. Six months after surgery, the patient had significant glottic insufficiency due to laryngeal deformity, constant massive salivary aspiration, and difficulty releasing the cuffed cannula. Severe dysphagia due to glottic insufficiency and laryngeal elevation insufficiency due to scar formation were considered, and laryngoplasty and laryngeal suspension surgery using costal cartilage were performed on the 221st postoperative day. After the reoperation, he was able to wear a speech valve and could speak and spit out, although his glottis was still closed. On the 70th day after the reoperation, he was able to consume three meals of an oral dysphagia diet and was discharged home

The Age at Diagnosis of Autism Spectrum Disorder in Children in Japan

Background. No large-scale study of the timing of autism spectrum disorder (ASD) diagnosis has been performed in Japan to date. The aim of this study was to examine sex differences and annual trends in age at diagnosis of ASD using clinical data. Methods. Clinical data for children aged less than 18 years diagnosed with ASD between January 1, 2009, and December 31, 2013, and in whom follow-up was possible 1 year after diagnosis, were extracted. Results. The mean age at ASD diagnosis was 7.2 ± 4.2 years and the mode age was 3 years. No sex difference was observed for age at diagnosis (p=0.157). An annual trend of earlier diagnosis was observed when fiscal years were compared (p<0.001). Conclusion. This study highlighted the need to develop and provide appropriate early intervention methods and services for ASD children in Japan