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101.
Takashi Kurashige Tetsuya Takahashi Yu Yamazaki Yoshito Nagano Keita Kondo Takeshi Nakamura Takemori Yamawaki Rie Tsuburaya Yukiko K. Hayashi Ikuya Nonaka Ichizo Nishino Masayasu Matsumoto 《Neuromuscular disorders : NMD》2013,23(11):911-916
Here we report what is to our knowledge the first identified Japanese family afflicted by X-linked myopathy with excessive autophagy. The index case is a 52-year-old man with almost 40 years of progressive proximal muscle weakness. High urinary β2 microglobulin, normal serum β2 microglobulin, autophagic vacuoles with sarcolemmal features, and a hemizygous c.164–7T>G mutation in the VMA21 gene were found. His two maternal uncles had similar clinicopathological findings. High urinary β2 microglobulin without obvious renal dysfunction might result from decreased urine acidification in the distal convoluted tubules caused by the VMA21 gene mutation. These findings might prove to be useful as a preliminary marker suggestive of X-linked myopathy with excessive autophagy. 相似文献
102.
Murakoshi Miki Kamei Koichi Ogura Masao Sato Mai Nada Taishi Suzuki Ryutaro Kamae Chikako Nishi Kentaro Kanamori Toru Nagano China Nozu Kandai Nakanishi Koichi Iijima Kazumoto 《Clinical and experimental nephrology》2022,26(2):162-169
Clinical and Experimental Nephrology - The management of congenital nephrotic syndrome of the Finnish type (CNF) is challenging. It is difficult to withdraw intravenous albumin infusions, resulting... 相似文献
103.
The Actin‐Binding Protein Cofilin and Its Interaction With Cortactin Are Required for Podosome Patterning in Osteoclasts and Bone Resorption In Vivo and In Vitro 下载免费PDF全文
Detina Zalli Lynn Neff Kenichi Nagano Nah Young Shin Walter Witke Francesca Gori Roland Baron 《Journal of bone and mineral research》2016,31(9):1701-1712
The adhesion of osteoclasts (OCs) to bone and bone resorption require the assembly of specific F‐actin adhesion structures, the podosomes, and their dense packing into a sealing zone. The OC‐specific formation of the sealing zone requires the interaction of microtubule (MT) + ends with podosomes. Here, we deleted cofilin, a cortactin (CTTN)‐ and actin‐binding protein highly expressed in OCs, to determine if it acts downstream of the MT‐CTTN axis to regulate actin polymerization in podosomes. Conditional deletion of cofilin in OCs in mice, driven by the cathepsin K promoter (Ctsk‐Cre), impaired bone resorption in vivo, increasing bone density. In vitro, OCs were not able to organize podosomes into peripheral belts. The MT network was disorganized, MT stability was decreased, and cell migration impaired. Active cofilin stabilizes MTs and allows podosome belt formation, whereas MT disruption deactivates cofilin via phosphorylation. Cofilin interacts with CTTN in podosomes and phosphorylation of either protein disrupts this interaction, which is critical for belt stabilization and for the maintenance of MT dynamic instability. Accordingly, active cofilin was required to rescue the OC cytoskeletal phenotype in vitro. These findings suggest that the patterning of podosomes into a sealing zone involves the dynamic interaction between cofilin, CTTN, and the MTs + ends. This interaction is critical for the functional organization of OCs and for bone resorption. © 2016 American Society for Bone and Mineral Research. 相似文献
104.
The laparoscopic technique for repairing ventral and incisional hernias (VIH) is now well established. However, several issues related to laparoscopic VIH repair, such as the high recurrence rate for hernias with large fascial defects and in extremely obese patients, are yet to be resolved. Additional problems include seroma formation, mesh bulging/eventration, and non-restoration of the abdominal wall rigidity/function with only bridging of the hernial orifice using standard laparoscopic intraperitoneal onlay mesh repair (sIPOM). To solve these problems, laparoscopic fascial defect closure with IPOM reinforcement (IPOM-Plus) has been introduced in the past decade, and a few studies have reported satisfactory outcomes. Although detailed techniques for fascial defect closure and handling of the mesh have been published, standardized techniques are yet to be established. We reviewed the literature on IPOM-Plus in the PubMed database and identified 16 reports in which the recurrence rate, incidence of seroma formation, and incidence of mesh bulging were 0–7.7, 0–11.4, and 0 %, respectively. Several comparison studies between sIPOM and IPOM-Plus seem to suggest that IPOM-Plus is associated with more favorable surgical outcomes; however, larger-scale studies are essential. 相似文献
105.
Further evidence that major outer membrane proteins homologous to OmpA in Porphyromonas gingivalis stabilize bacterial cells 总被引:1,自引:0,他引:1
Iwami J Murakami Y Nagano K Nakamura H Yoshimura F 《Oral microbiology and immunology》2007,22(5):356-360
INTRODUCTION: Porphyromonas gingivalis is one of the most important bacteria in the progression of chronic periodontal disease. We hypothesized that the major outer membrane proteins Pgm6/7, which are homologous to the OmpA protein in Escherichia coli, might contribute to the stabilization of the cell surface. In this study, the effects of Pgm6/7 on the cell surface were examined morphologically. METHODS: Deletion mutants of Pgm6/7 (Delta694, Delta695 and Delta695-694) were constructed using the polymerase chain reaction-based overlap extension method. Wild-type ATCC 33277 and Pgm6/7 mutants were grown under anaerobic conditions. Whole cells and thin sections of fixed cells were stained and examined by transmission electron microscopy. RESULTS: Compared with the wild-type, numerous vesicles released from cells were observed in each deletion mutant. The outer membrane appeared wavy and irregular. Increased numbers of vesicles were confirmed after their preparation from the culture supernatant. Total gingipain activity in vesicles was increased five- to 10-fold in the deletion mutants. CONCLUSION: This report provides further evidence that Pgm6/7 proteins in P. gingivalis play an important role in the maintenance of bacterial outer membrane integrity. 相似文献
106.
Kimura Yuta Tsunedomi Ryouichi Yoshimura Kiyoshi Matsukuma Satoshi Shindo Yoshitaro Matsui Hiroto Tokumitsu Yukio Yoshida Shin Iida Michihisa Suzuki Nobuaki Takeda Shigeru Ioka Tatsuya Hazama Shoichi Nagano Hiroaki 《Annals of surgical oncology》2022,29(12):7423-7433
Annals of Surgical Oncology - Poor prognosis in liver cancer is due to its high frequency of intrahepatic metastasis. Cancer stem-like cells (CSLCs), which possess the properties of stemness, tumor... 相似文献
107.
Annika Treyball Audrey C. Bergeron Daniel J. Brooks Audrie L. Langlais Hina Hashmi Kenichi Nagano Deborah Barlow Ryan J. Neilson Tyler A. Roy Kathleen T. Nevola Karen L. Houseknecht Roland Baron Mary L. Bouxsein Anyonya R. Guntur Katherine J. Motyl 《Journal of bone and mineral research》2022,37(5):954-971
Although the nonselective β-blocker, propranolol, improves bone density with parathyroid hormone (PTH) treatment in mice, the mechanism of this effect is unclear. To address this, we used a combination of in vitro and in vivo approaches to address how propranolol influences bone remodeling in the context of PTH treatment. In female C57BL/6J mice, intermittent PTH and propranolol administration had complementary effects in the trabecular bone of the distal femur and fifth lumbar vertebra (L5), with combination treatment achieving microarchitectural parameters beyond that of PTH alone. Combined treatment improved the serum bone formation marker, procollagen type 1 N propeptide (P1NP), but did not impact other histomorphometric parameters relating to osteoblast function at the L5. In vitro, propranolol amplified the acute, PTH-induced, intracellular calcium signal in osteoblast-like cells. The most striking finding, however, was suppression of PTH-induced bone resorption. Despite this, PTH-induced receptor activator of nuclear factor κ-B ligand (RANKL) mRNA and protein levels were unaltered by propranolol, which led us to hypothesize that propranolol could act directly on osteoclasts. Using in situ methods, we found Adrb2 expression in osteoclasts in vivo, suggesting β-blockers may directly impact osteoclasts. Consistent with this, we found propranolol directly suppresses osteoclast differentiation in vitro. Taken together, this work suggests a strong anti-osteoclastic effect of nonselective β-blockers in vivo, indicating that combining propranolol with PTH could be beneficial to patients with extremely low bone density. © 2022 American Society for Bone and Mineral Research (ASBMR). 相似文献
108.
109.