Satoyoshi's syndrome in an adult: a review of the literature of adult onset cases

Satoyoshi syndrome consists of painful intermittent muscle spasms, alopecia and diarrhea. The age of onset is commonly less than 20 years of age. Adult onset of this syndrome is rare. We report a 65-year-old Japanese woman with Satoyoshi's syndrome of adult onset. Satoyoshi's syndrome of adult onset shows no skeletal abnormalities, in comparison with the classical pediatric cases. The clinical features in our patient revealed the benign and long-term course of muscle spasm and alopecia. This progression differed markedly from the other patients of adult onset. Thus, the severity of Satoyoshi's syndrome is variable in adult onset cases, and this diagnosis should be considered in adults with various and unexplained muscle spasms.     

A content search system considering the activity and context of a mobile user

People routinely carry mobile devices in their daily lives and obtain a variety of information from the Internet in many different situations. In searching for information (content) with a mobile device, a user’s activity (e.g., moving or stationary) and context (e.g., commuting in the morning or going downtown in the evening) often change, and such changes can affect the user’s degree of concentration on his or her mobile device’s display and information needs. Therefore, a search system should provide the user with an amount of information suitable for the current activity and a type of information suitable for the current context. In this study, we present the design and implementation of a content search system that considers a mobile user’s activity and context, with the goal of reducing the user’s operation load for content search. The proposed system switches between two kinds of content search systems according to the user’s activity: the location-based content search system is activated when the user is stationary (e.g., standing and sitting), while a menu-based content search system is activated when the user is moving (e.g., walking). Both systems present information according to user context. The location-based system presents detailed information via menus and a map according to location-based categories. The menu-based system presents only a few options to enable users to get content easily. Through user experiments, we confirmed that participants could get desired information more easily with this system than with a commercial search system.     

Ionic effects on the transport characteristics of nanowire-based FETs in a liquid environment

For the development of ultra-sensitive electrical bio/chemical sensors based on nanowire field effect transistors （FETs）, the influence of the ions in the solution on the electron transport has to be understood. For this purpose we establish a simulation platform for nanowire FETs in the liquid environment by implementing the modified Poisson-Boltzmann model into Landauer transport theory. We investigate the changes of the electric potential and the transport characteristics due to the ions. The reduction of sensitivity of the sensors due to the screening effect from the electrolyte could be successfully reproduced. We also fabricated silicon nanowire Schottky-barrier FETs and our model could capture the observed reduction of the current with increasing ionic concentration. This shows that our simulation platform can be used to interpret ongoing experiments, to design nanowire FETs, and it also gives insight into controversial issues such as whether ions in the buffer solution affect the transport characteristics or not.     

Analysis of the Catalytic Mechanism of Bifunctional Triterpene/Sesquarterpene Cyclase: Tyr167 Functions To Terminate Cyclization of Squalene at the Bicyclic Step

Onoceroids are a group of triterpenes biosynthesized from squalene or dioxidosqualene by cyclization from both termini. We previously identified a bifunctional triterpene/sesquarterpene cyclase (TC) that constructs a tetracyclic scaffold from tetraprenyl‐β‐curcumene (C₃₅) but a bicyclic scaffold from squalene (C₃₀) in the first reaction. TC also accepts the bicyclic intermediate as a substrate and generates tetracyclic and pentacyclic onoceroids in the second reaction. In this study, we analyzed the catalytic mechanism of an onoceroid synthase by using mutated enzymes. TC^Y167A produced an unnatural tricyclic triterpenol, but TC^Y167L, TC^Y167F, and TC^Y167W formed small quantities of tricyclic compounds, which suggested that the bulk size at Y167 contributed to termination of the cyclization of squalene at the bicyclic step. Our findings provide insight into the unique catalytic mechanism of TC, which triggers different cyclization modes depending on the substrate. These findings may facilitate the large‐scale production of an onoceroid for which natural sources are limited.     

Oxidative Neurodegeneration Is Prevented by UCP0045037, an Allosteric Modulator for the Reduced Form of DJ-1, a Wild-Type of Familial Parkinson��s Disease-Linked PARK7

Although a loss-of-function mutation has been identified in familial Parkinson’s disease PARK7, the wild-type of DJ-1 is known to act as an oxidative stress sensor in neuronal cells. Recently, we identified UCP0045037 as a compound that bound to the reduced form of DJ-1 by in silico virtual screening. In this study, we determined the neuroprotective effects of UCP0045037 against focal cerebral ischemia-induced neurodegeneration in rats. Hydrogen peroxide-induced cell death was significantly inhibited by UCP0045037 in both rat mesencephalic dopaminergic neurons and human normal SH-SY5Y cells. In contrast, DJ-1-knockdown SH-SY5Y cells lost the protective activity of UCP0045037. These results suggest that UCP0045037 interacts with endogenous DJ-1 and produces a neuroprotective response.     

Analgesic Mechanisms of Steroid Ointment against Oral Ulcerative Mucositis in a Rat Model

Despite the long history of use of steroid ointments for oral mucositis, the analgesic mechanism has not been fully elucidated. In this study, we examined the effects of triamcinolone acetonide (Tmc) on oral ulcerative mucositis-induced pain in conscious rats by our proprietary assay system. Based on evaluations of the physical properties and retention periods in the oral mucosa of human volunteers and rats, we selected TRAFUL^® ointment as a long-lasting base. In oral ulcerative mucositis model rats, TRAFUL^® with Tmc suppressed cyclooxygenase-dependent inflammatory responses with upregulations of glucocorticoid receptor-induced anti-inflammatory genes and inhibited spontaneous nociceptive behavior. When an ointment with a shorter residual period was used, the effects of Tmc were not elicited or were induced to a lesser extent. Importantly, TRAFUL^® with Tmc also improved oral ulcerative mucositis-induced mechanical allodynia, which has been reported to be independent of cyclooxygenase. Ca²⁺ imaging in dissociated trigeminal ganglion neurons showed that long-term preincubation with Tmc inhibited the hypertonic stimulation-induced Ca²⁺ response. These results suggest that the representative steroid Tmc suppresses oral ulcerative mucositis-induced pain by general anti-inflammatory actions and inhibits mechanical sensitivity in peripheral nerves. For drug delivery, long-lasting ointments such as TRAFUL^® are needed to sufficiently induce the therapeutic effects.     

Insight into Isoprenoid Biosynthesis by Functional Analysis of Isoprenyl Diphosphate Synthases from Mycobacterium vanbaalenii and Mycobacterium tuberculosis

Comprehensive functional analyses of E-isoprenyl diphosphate synthases (E-IDSs) from nonpathogenic Mycobacterium vanbaalenii have been performed. Mv0992 and Mv1577 represent a nonaprenyl diphosphate (E-C₄₅) synthase and a geranylgeranyl diphosphate (E-C₂₀) synthase, respectively. Although Mv3536 was identified as an E-C₂₀ synthase using a single enzyme, co-incubation of Mv3536 and Z-IDSs (Mv4662 and Mv3822) strongly suggested it releases an intermediate geranyl diphosphate (E-C₁₀) during a continuous condensation reaction. Mv0992 and Mv3536 functions differed from those of the previously reported pathogenic Mycobacterium tuberculosis homologues Rv0562 and Rv2173, respectively. Re-analysis of Rv0562 and Rv2173 demonstrated that their functions were similar to those of Mv0992 and Mv3536 (Rv0562: E-C₄₅ synthase; Rv2173: E-C_10–15 synthase). The newly proposed functions of Rv0562 and Rv2173 would be in the biosynthesis of menaquinone and glycosyl carrier lipids essential for growth. Furthermore, a reduced allylic diphosphate could be used as the Z-IDS of the Mv3822 substrate, thereby introducing a potentially novel pathway of cyclic sesquarterpene biosynthesis.     

Biosynthesis of Sesterterpenes,Head‐to‐Tail Triterpenes,and Sesquarterpenes in Bacillus clausii: Identification of Multifunctional Enzymes and Analysis of Isoprenoid Metabolites

We performed functional analysis of recombinant enzymes and analysis of isoprenoid metabolites in Bacillus clausii to gain insights into the biosynthesis of rare terpenoid groups of sesterterpenes, head‐to‐tail triterpenes, and sesquarterpenes. We have identified an (all‐E)‐isoprenyl diphosphate synthase (E‐IDS) homologue as a trifunctional geranylfarnesyl diphosphate (GFPP)/hexaprenyl diphosphate (HexPP)/heptaprenyl diphosphate (HepPP) synthase. In addition, we have redefined the function of a tetraprenyl‐β‐curcumene synthase homologue as that of a trifunctional sesterterpene/triterpene/sesquarterpene synthase. This study has revealed that GFPP, HexPP, and HepPP, intermediates of two isoprenoid pathways (acyclic terpenes and menaquinones), are biosynthesized by one trifunctional E‐IDS. In addition, GFPP/HexPP and HepPP are the primary substrates for the biosynthesis of acyclic terpenes and menaquinone‐7, respectively.     

Energy cost of pushing a wheelchair on various gradients in young men

This study investigated the effects of pushing a wheelchair on the energy cost of walking (C_w; defined as the ratio of the steady-state oxygen consumption to the walking speed) and economical speed (ES) on the level and ±5% gradients. Eight pairs were formed from twelve young men to minimize variation in body weight between pushing and assisted participants. The State Trait Anxiety Inventory (STAI) test was conducted to evaluate wheelchair occupants' anxiety before and after each trial. The C_w values were significantly higher when pushing a wheelchair on the uphill gradient at more than 45 m/min. ES was significantly lower when pushing a wheelchair on the level (−8.5%) and uphill gradient (−9.1%), but not on the downhill gradient (−0.3%). Individual ES was also estimated using the concept of “Froude number”, and “estimated” ES was significantly correlated with “measured” ES even when pushing a wheelchair on the downhill gradient. The STAI score was not significantly increased except at 105 m/min, regardless of gradient. These results indicated that the fastest walking speed without an enhancement in wheelchair occupants' anxiety corresponds to ES when pushing a wheelchair with a seated occupant on all gradients, at least in young fit men.