TMEM16E/GDD1 has been shown to be responsible for the bone-related late-onset disease gnathodiaphyseal dysplasia (GDD), with the dominant allele (TMEM16E(gdd) ) encoding a missense mutation at Cys356. Additionally, several recessive loss-of-function alleles of TMEM16E also cause late-onset limb girdle muscular dystrophy. In this study, we found that TMEM16E was rapidly degraded via the proteasome pathway, which was rescued by inhibition of the PI3K pathway and by the chemical chaperone, sodium butyrate. Moreover, TMEM16E(gdd) exhibited lower stability than TMEM16E, but showed similar propensity to be rescued. TMEM16E did not exhibit cell surface calcium-dependent chloride channel (CaCC) activity, which was originally identified in TMEM16A and TMEM16B, due to their intracellular vesicle distribution. A putative pore-forming domain of TMEM16E, which shared 39.8% similarity in 98 amino acids with TMEM16A, disrupted CaCC activity of TMEM16A via domain swapping. However, the Thr611Cys mutation in the swapped domain, which mimicked conserved cysteine residues between TMEM16A and TMEM16B, reconstituted CaCC activity. In addition, the GDD-causing cysteine mutation made in TMEM16A drastically altered CaCC activity. Based on these findings, TMEM16E possesses distinct function other than CaCC and another protein-stabilizing machinery toward the TMEM16E and TMEM16E(gdd) proteins should be considered for the on-set regulation of their phenotypes in tissues.

No abstract

The records of 70 patients with oral cancer who were treated at a single institution between 2008 and 2014 were reviewed. The body temperature, white blood cell count, and C-reactive protein (CRP) levels were compared between those who had received preoperative oral care (oral care group) and those who had not received any (non-oral care group). When the patients were divided into those who underwent minimally invasive surgery and those who underwent severely invasive surgery, the mean CRP level in the early postoperative period was lower in the oral care group as compared with the non-oral care group in those who underwent minimally invasive surgery as well as those who underwent severely invasive surgery. However, the mean CRP level was most evidently reduced in the severely invasive group on days 1 and 3-5. However, no significant differences were observed with regard to the percentage of postoperative infectious complications (for example, surgical site infection, anastomotic leak and pneumonia) between the oral care (13.6%) and non-oral care (20.8%) groups, though a reduced prevalence of postoperative complications following preoperative oral care was noted. The results of the present study suggest that preoperative oral care can decrease inflammation during the early postoperative stage in patients with oral cancer who undergo severely invasive surgery.

BackgroundOnlay bone grafting techniques have some problems related to the limited volume of autogenous grafted bone and need for surgery to remove bone fixing screws. Here, we report a case of horizontal alveolar ridge atrophy following resection of a maxillary bone cyst, in which autogenous onlay bone grafting with interconnected porous hydroxyapatite ceramics (IP-CHA) and bioresorbable poly-L-lactic/polyglycolic acid (PLLA-PGA) screws was utilized.Case presentationA 51-year-old man had aesthetic complications related to alveolar atrophy following maxillary bone cyst extraction. We performed onlay grafting for aesthetic alveolar bone recovery using IP-CHA to provide adequate horizontal bone volume and PLLA-PGA screws for bone fixing to avoid later damage to host bone during surgical removal. During the operation, an autogenous cortical bone block was collected from the ramus mandibular and fixed to the alveolar ridge with PLLA-PGA screws, then the gap between the bone block and recipient bone was filled with a granular type of IP-CHA. Post-surgery orthopantomograph and CT scan findings showed no abnormal resorption of the grafted bone, and increased radiopacity, which indicated new bone formation in the area implanted with IP-CHA.ConclusionOur results show that IP-CHA and resorbable PLLA-PGA screws are useful materials for autogenous onlay bone grafting.

Unilateral condylar hyperplasia often causes severe facial asymmetry, malocclusion and temporomandibular joint pain. We present two cases of unilateral condylar hyperplasia with significant facial asymmetry and severe malocclusion. The first case was a 46-year-old female whose complaint was temporomandibular joint pain, facial asymmetry and trismus. The clinical diagnosis was left condylar tumor because these symptoms were recognized in post-adolescence and continued to progress. Low condylectomy with extraction of condylar disk was performed. The pathological diagnosis was condylar hyperplasia. Orthodontic treatment was continued for 5 months after condylectomy, and orthognathic surgery were performed. The second case was a 34-year-old female whose complaint was facial asymmetry and temporomandibular joint pain. These symptoms were recognized in adolescence and progressed slowly. The clinical diagnosis was left condylar hyperplasia. During pre-operative orthodontic treatment, 99mTc scintigram revealed proliferative activity in the left condylar lesion, and resection of the lesion was performed. The residual condyle was trimmed and contoured. Orthodontic treatment was continued for 10 months after the condylar operation, followed by orthognathic surgery. Four years and one year have passed since the orthognathic surgery, and esthetic and functional improvements and patient satisfaction have been obtained in both cases. In cases of facial asymmetry with condylar hyperplasia, it is often difficult to predict the occlusal and esthetic status without the improvement of condylar shape and function.The two-stage approach was a valid procedure to predict and obtain post-operative stability for the jaw and temporomandibular joint deformity.: condylar hyperplasia( 下 顎 頭 過 形 成 ) ， facial asymmetry(顔面非対称) ，orthognathic surgery (顎矯正手術) ［Received Nov. 30, 2015］ 日顎変形誌 Jpn.