Limb-girdle muscular dystrophy type 2A (LGMD2A) is a genetic disease that is caused by mutations in the calpain 3 gene (CAPN3), which encodes the skeletal muscle-specific calpain, calpain 3 (also known as p94). However, the precise mechanism by which p94 functions in the pathogenesis of this disease remains unclear. Here, using p94 knockin mice (termed herein p94KI mice) in which endogenous p94 was replaced with a proteolytically inactive but structurally intact p94:C129S mutant protein, we have demonstrated that stretch-dependent p94 distribution in sarcomeres plays a crucial role in the pathogenesis of LGMD2A.
View Article and Find Full Text PDFWhile adaptation to complex auditory stimuli has traditionally been reported for linguistic properties of speech, the present study demonstrates non-linguistic high-level aftereffects in the perception of voice identity, following adaptation to voices or faces of personally familiar speakers. In Exp. 1, prolonged exposure to speaker A's voice biased the perception of identity-ambiguous voice morphs between speakers A and B towards speaker B (and vice versa).
View Article and Find Full Text PDFSunitinib malate (Sutent, SU11248) is a small-molecule multitargeted tyrosine kinase inhibitor (TKI) used for the treatment of renal cell carcinoma and imatinib-resistant gastrointestinal stromal tumors. Some TKIs can overcome multidrug resistance conferred by ATP-binding cassette transporter, P-glycoprotein (P-gp)/ABCB1, multidrug resistance-associated protein 1 (MRP1)/ABCC1, and breast cancer resistance protein (BCRP)/ABCG2. Here, we analyzed the effects of sunitinib on P-gp and on wild-type and germ-line mutant BCRPs.
View Article and Find Full Text PDFBackground: Plaque erosion is a cause of atherothrombosis that preferentially occurs on smooth muscle cell (SMC)- and proteoglycan-rich rather than lipid-rich plaques. However, its underlying mechanisms remain unknown.
Objective: To determine whether disturbed blood flow induces erosive injury and thrombus formation on SMC-rich neointima.
Background/aims: For laparoscopic anterior resection, a small incision is placed in the left lower abdomen or suprapubic area, for which ideal location remains to be clarified. This study evaluated the validity of anthropometric placement of such an incision for colorectal anastomosis in laparoscopic anterior resection.
Methodology: Thirty consecutive patients with advanced rectal cancer (Age 30- 82 {mean 60.
Vocal attractiveness has a profound influence on listeners-a bias known as the "what sounds beautiful is good" vocal attractiveness stereotype [1]-with tangible impact on a voice owner's success at mating, job applications, and/or elections. The prevailing view holds that attractive voices are those that signal desirable attributes in a potential mate [2-4]-e.g.
View Article and Find Full Text PDFSodium butyrate (NaB) induced the membrane enclosed cell size vesicles from several IgM producing cell lines. We considered the application of the cell-derived vesicles (CDVs) to drug delivery system (DDS) using the lung cancer specific IgM producing AE6 cell line. Microscopic observation showed that the DiI fluorescence labeled AE6 vesicles were incorporated into the lung cancer cell line A549.
View Article and Find Full Text PDFBackground/purpose: Thoracoscopic repair has recently been attempted in newborns with esophageal atresia (EA), but it remains unclear whether thoracoscopic dissection reduces pathological gastroesophageal reflux. We investigated the influence of a thoracoscopic approach on esophageal motor function in patients with EA.
Methods: Clinical and gastrointestinal data of 10 patients with EA with open repair (group A) and 7 with thoracoscopic repair (group B) were analyzed retrospectively.
Between 2000 and 2008, 11 major orthopaedic surgeries for 7 congenital haemophilia patients with inhibitors were performed by the first author as the primary doctor using recombinant activated factor VII (rFVIIa). Orthopaedic surgical treatments were performed for six surgeries for four high-responder haemophilia A patients, three surgeries for two high-responder haemophilia B patients and two surgeries for one low-responder haemophilia B patient. This low-responder patient is allergic to factor IX products, so he usually uses rFVIIa as a haemostatic agent.
View Article and Find Full Text PDFA marine-derived actinomyces strain (NPS554) isolated from a marine sediment sample collected from Miyazaki Harbor, Japan, at a depth of 38 m yielded two trialkyl-substituted aromatic acids, lorneic acid A (1) and lorneic acid B (2). The structures of the lorneic acids, which were elucidated by spectroscopic analysis, differed only in the side-chain, which contained either a conjugated double bond or a benzylic alcohol. Their structural differences affected inhibition activities against phosphodiesterase 5.
View Article and Find Full Text PDFUnlabelled: Osteoporosis represents a significant side effect of glucocorticoid therapy, and alendronate has been reported to prevent this glucocorticoid-induced osteoporosis. Functional imaging with (18)F-fluoride PET allows quantitative analysis of bone metabolism in specific skeletal regions. However, only a few studies have quantitatively determined bone turnover and metabolism in glucocorticoid-induced osteoporosis by radiologic imaging techniques including PET.
View Article and Find Full Text PDFDifferential and integral cross sections for electron-impact excitation of the dipole-allowed C (1)Pi and D (1)Sigma(+) electronic states of nitrous oxide have been measured. The differential cross sections were determined by analysis of normalized energy-loss spectra obtained using a crossed-beam apparatus at six electron energies in the range 15-200 eV. Integral cross sections were subsequently derived from these data.
View Article and Find Full Text PDFPurpose: Rikkunshito is used to treat functional dyspepsia in adults. This study investigated the effects of rikkunshito on delayed gastric emptying in handicapped patients.
Methods: A retrospective review was performed in nine profoundly handicapped patients (aged 1-19 years).
While high-level adaptation to faces has been extensively investigated, research on behavioural and neural correlates of auditory adaptation to paralinguistic social information in voices has been largely neglected. Here we replicate novel findings that adaptation to voice gender causes systematic contrastive aftereffects such that repeated exposure to female voice adaptors causes a subsequent test voice to be perceived as more male (and vice versa), even minutes after adaptation [S.R.
View Article and Find Full Text PDFThe dysfunction of proteasomes and mitochondria has been implicated in the pathogenesis of Parkinson disease. However, the mechanism by which this dysfunction causes neuronal cell death is unknown. We studied the role of cyclin-dependent kinase 5 (Cdk5)-p35 in the neuronal cell death induced by 1-methyl-4-phenylpyrinidinium ion (MPP+), which has been used as an in vitro model of Parkinson disease.
View Article and Find Full Text PDFTo establish the effects of type I antifreeze protein (AFP) on E. coli cells, we have focused on the survival rate of the E. coli cells using type I AFP at various concentrations under rapid cooling conditions using liquid N2 at atmospheric or low pressure.
View Article and Find Full Text PDFBackground/aims: We reported a novel surgical technique, sliding functional end-to-end anastomosis (SFEEA), which abrogates the need for the transanal procedure for colorectal anastomosis. The aim of this study is to evaluate to the usefulness of SFEEA in high anterior resection as compared with double stapling technique (DST).
Methodology: From 2001 to 2005, high anterior resection was performed in 78 consecutive patients with advanced sigmoid colon cancer whose anatomoses with stapler were located under their promontories using SFEEA (n=35) or DST (n=43).
Six antitumor antibiotics of a new structure class, indoxamycins A-F (1-6), were isolated from a saline culture group of marine-derived actinomyces whose strains showed approximately 96% sequence homology of 16S rDNA with the family streptomycetaceae. The structures of these indoxamycins, which are unusual polyketides composed of six consecutive chiral centers, were assigned by combined spectral and chemical methods. In feeding experiments using a stable isotope label, indoxamycin A was assembled from propionate units initially forming the "aglycon" pentamethyl indeno furan.
View Article and Find Full Text PDFPrenatal diagnosis of concomitant duodenal atresia (DA) and esophageal atresia (EA) without tracheoesophageal fistula (TEF) (Gross type A) is very rare. We describe prenatal findings of one such case. Sonographic examination of a 26-week fetus showed a double cystic structure and an intrathoracic cyst.
View Article and Find Full Text PDFThe aim of this study is to develop a prototype system for noncontact, noninvasive and unconstrained vital sign monitoring using microwave radar and to use the system to measure the respiratory rate of a Japanese black bear (Ursus thibetanus japonicus) during hibernation for ensuring the bear's safety. Ueno Zoological Gardens in Tokyo planned to help the Japanese black bear (female, approximately 2 years of age) going into hibernation. The prototype system has a microwave Doppler radar antenna (10-GHz frequency, approximately 7 mW output power) for measuring motion of the body surface caused by respiratory activity without making contact with the body.
View Article and Find Full Text PDFIn Noh, a traditional performing art of Japan, extremely expressive voice quality is used to convey an emotional message. A periodicity of voice appears responsible for these special effects. Acoustic signals were recorded for selected portions of dramatic singing in order to study the acoustic effects of delicate voice control by a master of the Konparu school.
View Article and Find Full Text PDFEpidermal growth factor receptor tyrosine kinase inhibitors (EGFR-TKIs) inhibit the function of certain adenosine triphosphate (ATP)-binding cassette transporters, including P-glycoprotein/ABCB1 and breast cancer resistance protein (BCRP)/ABCG2. We previously reported an antagonistic activity of gefitinib towards BCRP. We have now analyzed the effects of erlotinib, another EGFR-TKI, on P-glycoprotein and BCRP.
View Article and Find Full Text PDFTooth loss accompanied by a massive defect of the alveolar bone can cause serious problems such as food deposit and esthetic impairment. This report describes procedures for the fabrication of an osseous defect obturator prosthesis connected to a fixed partial denture by a magnetic attachment along with the clinical outcome.
View Article and Find Full Text PDFJapanese radish tuber and leaf produced antifreeze proteins (AFPs) having thermal hysteresis activity (TH) and ice recrystallization inhibiting activity (RI). Upon cold acclimation, the apoplastic fluid of the Japanese radish exhibited hexagonal crystal growth, indicating the presence of an antifreeze protein. The induction patterns of protein and the TH activity of apoplastic fraction from both samples were different.
View Article and Find Full Text PDFGhrelin induces orexigenic behavior by activation of growth hormone secretagogue 1 receptors (GHSRs) in the ventral tegmental area (VTA) as well as hypothalamus, suggesting the involvement of mesolimbic dopamine system in the action of ghrelin. The present study aimed to identify neuronal mechanisms by which peripherally administered ghrelin regulates the mesolimbic dopamine system under different food-consumptive states. Ghrelin was administered to rats peripherally (3 nmol, i.
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