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症例は28歳,一年程前より上肢を長時間動かすと倦怠感と立ちくらみを自覚.今回台所仕事中に意識消失し搬送された.意識清明なるも脈は触知せず,血圧も測定不可であった.頸部に血管雑音聴取し,血管造影にて左右鎖骨下動脈は描出されず,右総頸動脈に狭窄を認めた.血液検査にて炎症反応を認め,大動脈炎症候群と診断した.左右鎖骨下動脈,右総頸動脈に計3個のステントを留置し症状は消失,プレドニン,トラニラスト,シロスタゾール投与下に経過良好である.

Background The intracellular deposition of misfolded proteins is a common neuropathological hallmark of most neurodegenerative disorders. Increasing evidence suggests that these pathogenic proteins may spread to neighboring cells and induce the propagation of neurodegeneration. Results In this study, we have demonstrated that α-synuclein (αSYN), a major constituent of intracellular inclusions in synucleinopathies, was taken up by neuronal and oligodendroglial cells in both a time- and concentration-dependent manner. Once incorporated, the extracellular αSYN was immediately assembled into high-molecular-weight oligomers and subsequently formed cytoplasmic inclusion bodies. Furthermore, αSYN uptake by neurons and cells of the oligodendroglial lineage was markedly decreased by the genetic suppression and pharmacological inhibition of the dynamin GTPases, suggesting the involvement of the endocytic pathway in this process. Conclusions Our findings shed light on the mode of αSYN uptake by neuronal and oligodendroglial cells and identify therapeutic strategies aimed at reducing the propagation of protein misfolding.

Although certain saponins in soybean seeds have been reported to have health benefits, group A acetyl saponins cause undesirable bitter and astringent tastes in soy products. Therefore, reduction or elimination of group A saponins is an important target for soybean breeders. A wide survey of cultivated and wild soybean germplasm identified a mutant line that lacked group A saponins. The absence of soyasapogenol A, a group A saponin aglycone, is controlled by a single recessive allele, sg-5 that mapped genetically near the SSR marker, Satt117, on soybean chromosome 15 (linkage group E). The locus is epistatic to Sg-1, which controls the terminal sugar variation on the C-22 sugar chain of soyasapogenol A, and allelic differences at this locus lead to changes in the amount of DDMP saponins and their derivatives group B and E products. These findings provide a new insight into the biosynthetic pathway of soybean saponins, and identify a genetic approach that can be applied to improve the quality of foods produced from soybean.

The authors describe the case of a 39-year-old man who presented to our hospital with easy fatigability and malaise. On physical examination, hypertension was noted without any cushingoid appearance. Laboratory testing revealed normochromic-normocytic anaemia with positive results of occult blood in the stool, hyperglycaemia and hypokalemia. Upper endoscopy revealed active gastric ulcer with Helicobacter pylori infection, likely causing gastrointestinal bleeding. Endocrine examinations showed that both serum adrenocorticotropic hormone and cortisol were elevated with loss of diurnal variation. A diagnosis of Cushing’s disease secondary to pituitary adenoma was made as results of brain MRI and blood sampling from inferior petrosal sinus. In a patient with peptic ulcer disease, physician should be alert to the possible endocrine background.

Triterpene saponins are a diverse group of biologically functional products in plants. Saponins usually are glycosylated, which gives rise to a wide diversity of structures and functions. In the group A saponins of soybean (Glycine max), differences in the terminal sugar species located on the C-22 sugar chain of an aglycone core, soyasapogenol A, were observed to be under genetic control. Further genetic analyses and mapping revealed that the structural diversity of glycosylation was determined by multiple alleles of a single locus, Sg-1, and led to identification of a UDP-sugar-dependent glycosyltransferase gene (Glyma07g38460). Although their sequences are highly similar and both glycosylate the nonacetylated saponin A0-αg, the Sg-1(a) allele encodes the xylosyltransferase UGT73F4, whereas Sg-1(b) encodes the glucosyltransferase UGT73F2. Homology models and site-directed mutagenesis analyses showed that Ser-138 in Sg-1(a) and Gly-138 in Sg-1(b) proteins are crucial residues for their respective sugar donor specificities. Transgenic complementation tests followed by recombinant enzyme assays in vitro demonstrated that sg-1(0) is a loss-of-function allele of Sg-1. Considering that the terminal sugar species in the group A saponins are responsible for the strong bitterness and astringent aftertastes of soybean seeds, our findings herein provide useful tools to improve commercial properties of soybean products.

Many neurodegenerative diseases share a common pathological feature: the deposition of amyloid-like fibrils composed of misfolded proteins. Emerging evidence suggests that these proteins may spread from cell-to-cell and encourage the propagation of neurodegeneration in a prion-like manner. Here, we demonstrated that α-synuclein (αSYN), a principal culprit for Lewy pathology in Parkinson's disease (PD), was present in endosomal compartments and detectably secreted into the extracellular milieu. Unlike prion protein, extracellular αSYN was mainly recovered in the supernatant fraction rather than in exosome-containing pellets from the neuronal culture medium and cerebrospinal fluid. Surprisingly, impaired biogenesis of multivesicular body (MVB), an organelle from which exosomes are derived, by dominant-negative mutant vacuolar protein sorting 4 (VPS4) not only interfered with lysosomal targeting of αSYN but facilitated αSYN secretion. The hypersecretion of αSYN in VPS4-defective cells was efficiently restored by the functional disruption of recycling endosome regulator Rab11a. Furthermore, both brainstem and cortical Lewy bodies in PD were found to be immunoreactive for VPS4. Thus, VPS4, a master regulator of MVB sorting, may serve as a determinant of lysosomal targeting or extracellular secretion of αSYN and thereby contribute to the intercellular propagation of Lewy pathology in PD.

The variation in saponin composition in soybean seeds is explained by different combinations of five genes controlling the utilization of soyasapogenol glycosides as substrates. The function of these genes is variety-specific and organ-specific. Phenotypes of over 1000 soybeans were classified into eight saponin types, and the frequency of phenotypes was different between the cultivated [Glycine max (L.) Merr.] and the wild soybean (G. soja Sieb.Zucc.). The AaBc saponin type predominated in G. soja (58.4% of test collections), but was only found in 0.3% of G. max. Four unidentified arabinoside saponins were detected in the seeds of the AaBc type soybeans. The mode of inheritance of saponin types is explained by a combination of co-dominant, dominant and recessive acting genes. The combined chemical and genetic data show that the directed manipulation of soybean saponin composition is a possibility for the future.

Hyposmia is one of the cardinal early symptoms of Parkinson disease (PD). Accumulating clinical and pathological evidence suggests that dysfunction of the olfactory-related cortices may be responsible for the impaired olfactory processing observed in PD; however, there are no clear data showing a direct association between altered brain metabolism and hyposmia in PD. In this study, we evaluated brain glucose metabolism and smell-identification ability in 69 Japanese patients with nondemented PD. Olfactory function was assessed using the Odor Stick Identification Test for Japanese. The regional cerebral metabolic rate of glucose consumption at rest was measured using (18)F-fluorodeoxyglucose positron emission tomography and was analyzed using SPM-based group comparisons and the brain-behavior partial least-squares method. We found that olfactory dysfunction was closely related to cognitive dysfunction, including memory impairment. Moreover, brain-behavior partial least-squares analysis revealed that odor-identification performance was closely associated with broad cortical dysfunction, including dysfunction of the piriform cortex and amygdala. Our results suggest that the cognitive deficit in olfactory perception is an important aspect of hyposmia in PD and that this deficit is caused by altered brain metabolism in the amygdala and piriform cortex.

Group A acetyl saponin deficient mutant was discovered from wild soybeans ( Glycine soja ). The mutant contained deacetyl Af but not deacetyl Ab. F 1 seeds were obtained from a cross between the mutant and a soybean variety which carries the acetylation activity of deacetyl Af but lacks the glucosylation activity for producing saponin Ab. The F 1 seeds contained saponin Ab but deacetyl Af was not detected. It can be seen that the mutant lacks the ability to acetylate the hydroxyl groups of the terminal sugar of the sugar chain attached at the C-22 position of soyasapogenol A, and that saponin Ab is produced via saponin Af, not via deacetyl Ab. The mutant carried additional mutations in the seed protein composition, such as a deficiency in the ′α subunit of β-conglycinin.

Fragrance materials such as perfumes have long been known to exert effects on the mind and body. Throughout histry, perfumes have been used as sedatives and stimulants. However, there are very few psychophysiological studies in aromachology with regard to the effect of odor itself on human subjects.In this study, the effects of odors on cardiac response patterns were investigated during the foreperiod of the two-stimulus paradigm in a simple reaction time task. Changes in the cardiac response pattern were typically triphasic during the foreperiod between the worning stimulus (WS) and imperative stimulus (IS).Heart rate decelelation (DE) usually seen just prior to IS is thought to reflect the process of anticipation or attention. Thus, stimulative odors are expected to activate those processes while sedative odors are thought to exert a suppressive effect.This experiment was designed to estimate the effect of some kinds of odors by the DE amplitude. Olfactory stimulation (some different aromatic air samples and odorless air) was provided to subjects by the blast method.A trial consisted of a 5-second rest period, followed by a 20-second period in which the olfactory stimulus was presented, a 10-second foreperiod lasting from the WS tone to the IS tone signaling the subject to press the response key, and a 15-second post-foreperiod after the IS tone.The odor of lemon, traditionally thought to be stimulative, had the effect of activating anticipation or attention process. This effect tended to be stronger by increasing odor intensity. On the other hand, the rose odor, thought to be sedative, had the effect of suppressing this process, and these effect was observed across all intensity levels.Futhermore, We carried out an experiment in the same method, to estimate the effects of 3 kinds of perfumes for the development of odor using for certain skincare products.We believe this investigational method with two-stimulus paradigm could be a useful one to determine the psychophysiological effects of odor especially in stimulative-sedative continuum of its effect.

A numerical analysis is conducted without introducing any turbulence model for the turbulent flow in a square pipe. The direct numerical calculation approach generally requires a huge computational time and memory size, and is possibly performed on a relatively coarse grid system. Therefore, evaluation of the solution for the coarse grid system is important in practical applications. Comparison with the experiment and the large eddy simulation (LES) reveals that the present calculation successfully predicts the characteristic feature of the turbulent flow even with the coarse grid system. Quantitative improvement of the solution is achieved nearly in proportion to the grid resolution squared.

Hyposmia in Parkinson's disease (PD) was evaluated by using neuroimaging techniques. It is well known that olfactory impairments are one of the cardinal non-motor symptoms in PD. However, all smell tests used in previous studies depend on subjective answers by examinees and on sniffing of odorants, the latter of which may be impaired in PD as a consequence of motor impairments. We developed an fMRI system, which can visualize brain activation by olfactory stimuli during natural breathing. Although 7 age-matched controls demonstrated significant activations in various brain areas including precentral gyrus (BA6/6) and middle temporal gyrus (BA19/39) by the odorant stimuli, 9 patients with PD showed little activations by the same stimuli. These data suggest that the olfactory dysfunction in PD is not a simple reflection of impaired sniffing. Recent epidemiological studies demonstrate that the olfactory impairments may precede the onset of motor symptoms. Moreover, several pathological studies suggest that amygdala is one of the most frequently affected regions and is closely related to hyposmia in PD. Further brain imaging studies of hyposmia will shed light on the early pathological changes in PD.

Neuromyelitis optica (NMO) is characterized by severe optic neuritis and longitudinally extended transverse myelitis (LETM). The clinical and laboratory features of NMO are different from multiple sclerosis (MS).1 An autoantibody to aquaporin-4 (AQP4) has been detected exclusively in the NMO sera.1 Moreover, we demonstrated an extensive loss of AQP4 and glial fibrillary acidic protein immunoreactivities in the perivascular regions with complement and immunoglobulin deposition in NMO that suggests astrocytic impairment. However, when AQP4 antibody is produced in patients with NMO is unknown, and thus it remains unresolved whether there are long-term asymptomatic AQP4 antibody–positive carriers, whether AQP4 antibody alone can be pathogenic, and whether AQP4 antibody is produced secondarily as a result of tissue destruction in attacks of NMO. We herein report a case of NMO in which AQP4 antibody was detected years before the NMO onset. ### Case report. A 34-year-old healthy woman without previous history of inflammatory or neurologic diseases noticed temporary skin eruptions on her chest and shoulders in June 2007. A dermatologist made the diagnosis of eczema. Three weeks later, when the skin eruptions subsided, she noted progressive paresthesia in the chest and toes. Within a few days, she could not walk well due to right leg weakness, and then she was hospitalized. Neurologic examination on …

Soybean seeds contain isoflavone compounds which are responsible for the undesirable flavor of soybean products, namely bitterness and astringency. The analysis of isoflavones of soybean cultivars using high performance liquid chromatography revealed that in some early maturing cultivars, so-called "summer-type soybeans", the isoflavone contents were remarkably low. The low isoflavone content in the soybean cultivars grown under standard culture conditions was found to be stable.

A 50-year-old man without family history of metabolic bone disease was referred to our hospital with a 5-year history of progressively worsening spinal and bilateral diffuse leg pain and proximal muscle weakness. Two years before admission, he was diagnosed as ankylosing spondylitis by a rheumatologist and was maintained on low-dose prednisone therapy without benefit. He developed progressive spinal and thoracic deformities, resulting in a 10 cm loss in height in the preceding 2 years. On physical examination, marked thoracic kyphosis and pectus carinatum was noted. Plain radiograph revealed pseudofracture in the right femoral neck. Laboratory findings showed a normal level of serum calcium, elevated level of serum alkaline phosphatase and inappropriately increased urinary phosphate excretion despite extreme hypophosphatemia. He was diagnosed as adult-onset hypophosphatemic osteomalacia caused by renal phosphate wasting. Serum fibroblast growth factor 23 was the upper limit of normal despite extreme hypophosphatemia and no neoplastic lesion potentially inducing hypophosphatemic osteomalacia could be identified in a thorough search including imaging studies of his entire body. Oral administration of phosphate and activated vitamin D together with dipyridamole relieved the persistent pain and weakness, and he became fully ambulatory.

A 56-year-old woman was admitted because of a high fever, right ptosis, chemosis, proptosis and ocular muscle palsy. Cranial MRI revealed a cavernous sinus thrombosis and a subarachnoid abscess. Carotid angio-gram demonstrated marked stenosis as well as aneurismal formation of the right internal carotid artery at the intracavernous portion. Chest radiograph showed bilateral multiple pulmonary nodules, some of which contained a cavity. Blood culture was positive for Streptococcus constellatus. She was diagnosed with septic cavernous sinus thrombosis complicated by narrowing of the internal carotid artery, subarachnoid abscess and multiple pulmonary septic emboli. She recovered with partial ocular sequelae as a result of seven weeks of intravenous antimicrobial therapy.

Objectives The objective of the present study was to evaluate the association between physical activity (PA) levels and urinary incontinence (UI) in a community-based elderly population aged ≥70 yr. Methods This population-based cross-sectional survey was conducted in 2003 using an extensive health interview for each participant. A self-reported single-item questionnaire was used to estimate different levels of PA in each subject. The prevalence of UI was estimated by the self-administered International Consultation on Incontinence Questionnaire. The study population included 676 Japanese men and women. Results The prevalence of UI was 25% (34% in women and 16% in men). After adjustment for potential confounding factors, the odds ratio (95% confidence interval) of UI compared with the lowest PA group was 0.71 (0.47–1.09) and 0.58 (0.35–0.96) in subjects exhibiting middle and high levels of PA, respectively ( p for trend=0.02). Conclusions High PA level was independently related to a lower self-reported prevalence of UI in a community-dwelling elderly population aged ≥70 yr. Although this cross-sectional study cannot demonstrate a temporal relationship between PA and the onset of UI, the findings suggest that PA may have a potentially beneficial effect on the prevention of UI. A prospective study or randomized trials are required to clarify the causality.

Free radicals, including dopamine (DA)-oxidized metabolites, have long been implicated in pathogenesis of Parkinson's disease (PD). However, the relationships between such oxidative stresses and alpha-synuclein (alpha-S), a major constituent of Lewy bodies, remain unknown. In this study, we established neuronal cells that constitutively express alpha-S and tetracycline-regulated tyrosinase. While tyrosinase overexpression induced apoptosis, co-expression of wild type or A53T mutant human alpha-S with tyrosinase further exacerbated cell death. In this process, the formation of alpha-S oligomers and the reduction in mitochondrial membrane potential were demonstrated. This cellular model may reconstitute the pathological metabolism of alpha-S in the synucleinopathy and provide a useful tool to explore possible pathomechanisms of nigral degeneration in PD.

Studies were carried out to enhance the development of isoflavone aglycones in soybean. Grains of two soybean cultivars, BR 36 and IAS 5, 115 and 278 mg/100g of total isoflavone, respectively, were treated hydrothermalyl at 45, 60 and 85° C for 5, 30 and 60 minutes. Pre-treatments of grains at 60° C for 60 minutes allowed a considerable increase of the isoflavone aglycones. Non-treated grains of BR 36 and IAS 5 showed 1.2 mg/100g of genistein, after hydrothermal treatments,which increased to 12 and 53 mg/100g, in each variety, respectively. At higher temperature (85 ° C) there was a decrease of the aglycones due to inactivation of beta-glycosidases. Malonyl compounds were also reduced at higher temperatures. In processing functional soybean foods, hydrothermal treatments of the soybean grains, as well as high isoflavone content soybean cultivars will enhance development of aglycone forms.Estudos foram conduzidos para aumentar isoflavonas agliconas (compostos mais biodisponíveis e mais efetivos na prevenção de doenças crônicas) em grãos de soja. Pré-tratamentos hidrotérmicos dos grãos foram conduzidos a 45, 60 e 85 ºC por 5, 30, e 60 minutos. Duas cultivares de soja BR 36 e IAS 5 (115, e 278 mg/100g de isoflavonas totais, respectivamente), foram usadas nos experimentos. Pré-tratamentos dos grãos a 60 ºC por 60 minutos permitiram um considerável aumento das isoflavonas agliconas. Grãos não tratados de BR 36 e IAS 5 apresentaram 1,2 mg/100g de genisteína. Depois dos tratamentos hidrotérmicos, este composto aumentou para 12 e 53 mg/100g, em cada variedade, respectivamente. Em altas temperatures (85 ºC) houve diminuição das agliconas devido a inativação das enzimas beta-glicosidases. Os compostos malonil (térmicamente instáveis), também foram reduzidos sob altas temperaturas. No processamento de alimentos funcionais de soja, pré-tratamentos hidrotérmicos dos grãos, bem como a utilização de cultivares com alto teor de isoflavonas permitirão maior desenvolvimento das formas agliconas.

Alpha-synuclein (alpha-syn) is a major component of inclusion bodies in Parkinson's disease (PD) and other synucleinopathies. To clarify the possible roles of alpha-syn in the molecular pathogenesis of neurodegenerative diseases, we have established a novel cellular model based on the differentiation of SH-SY5Y cells that overexpress alpha-syn. In the presence of ferrous iron, differentiation of the cells led to the formation of large perinuclear inclusion bodies, which developed from scattered small aggregates seen in undifferentiated cells. The iron-induced alpha-syn-positive inclusions co-localized largely with ubiquitin, and some of them were positive for nitrotyrosine, lipid, gamma-tubulin and dynein. Notably, treatment with nocodazole, a microtubule depolymerizing agent, interrupted the aggregate formation but led to a concomitant increase of apoptotic cells. Therefore, it appears that an intracellular retrograde transport system via microtubules plays a crucial role in the aggregate formation and also that the aggregates may represent a cytoprotective response against noxious stimuli. This cellular model will enable better understanding of the molecular pathomechanisms of synucleinopathy.

alpha-Synuclein is a major component of intracytoplasmic inclusions including Lewy bodies (LB), Lewy neurites (LN) and glial cytoplasmic inclusions, and plays a key role in neurodegenerative processes in Parkinson's disease (PD) and other synucleinopathies. Although the molecular mechanisms of the disease process still remain to be elucidated, recent studies have suggested that an interaction between reactive oxygen species (ROS) and alpha-synuclein may be closely associated with the initiation and/or the progression of synucleinopathies. In this study, we established human dopaminergic SH-SY5Y cell lines overexpressing wild-type or mutant alpha-synuclein and exposed them to various ROS generators. After the exposure to ROS, alpha-synuclein aggregates were formed in the cytoplasm of these cells, and these were immunopositive for ubiquitin, nitrotyrosine and dityrosine, and positive for thioflavin S staining. Thus, the obtained cytoplasmic aggregates shared many features with inclusion bodies in synucleinopathies. The gamma-tubulin and molecular chaperones coexisted as well, suggesting that the aggregate formation is associated with the intracellular transport along microtubules and may reflect protective responses against neuronal insults. This cellular model not only will be informative for our understanding of the pathophysiological process in synucleinopathies, but also can be applied to the screening of neuroprotective molecules with therapeutic potential.

Oxidized metabolites of dopamine, known as dopamine quinone derivatives, are thought to play a pivotal role in the degeneration of dopaminergic neurons. Although such quinone derivatives are usually produced via the autoxidation of catecholamines, tyrosinase, which is a key enzyme in melanin biosynthesis via the production of DOPA and subsequent molecules, may potentially accelerate the induction of catecholamine quinone derivatives by its oxidase activity. In the present study, we developed neuronal cell lines in which the expression of human tyrosinase was inducible. Overexpression of tyrosinase in cultured cell lines resulted in (i) increased intracellular dopamine content; (ii) induction of oxidase activity not only for DOPA but also for dopamine; (iii) formation of melanin pigments in cell soma; and (iv) increased intracellular reactive oxygen species. Interestingly, the expressed tyrosinase protein was initially distributed in the entire cytoplasm and then accumulated to form catecholamine-positive granular structures by 3 days after the induction. The granular structures consisted of numerous rounded, dark bodies of melanin pigments and were largely coincident with the distribution of lysosomes. This cellular model that exhibits increased dopamine production will provide a useful tool for detailed analyses of the potentially noxious effects of oxidized catecholamine metabolites.

The changes of regional cerebral blood flow (rCBF) in Parkinson's disease (PD) were investigated. Because of individual differences in brain volume and the extent of brain atrophy, previous functional imaging studies involved potential methodological difficulties. In this study, using the statistical parametric mapping technique, 99mTechnetium-labeled hexamethylpropyleneamineoxime brain single-photon emission computed tomography images from 18 patients with PD were transformed into standard brain-based stereotaxic coordinate spaces and then compared with such images for 11 control subjects matched for age and extent of brain atrophy. A rCBF decrement in the supplementary motor area (SMA) and such decrement in the dorsolateral prefrontal cortex (DLPFC) were observed in the summarized PD images as compared with controls (p

Some pathological similarities between Alzheimer’s disease and muscle diseases with rimmed vacuoles (RV) have been pointed out. For example, several pathological hallmark proteins have been reported to be immunopositive in the lesions of both diseases. Since apoptotic processes or primary DNA damage are suggested to play a role in the pathomechanism of Alzheimer’s disease, we examined DNA double-strand breaks (DSB) and single-strand breaks (SSB) in the muscle biopsy specimens of several diseases, including muscle diseases with RV. Although no DSB-positive myonuclei were detected in any muscles examined, the number of SSB-positive myonuclei markedly increased in the muscles from cases with polymyositis and muscle diseases with RV. In polymyositis, SSB-positive myonuclei were observed in regenerating fibers and muscle fibers in the vicinity of inflammatory infiltrates, suggesting that the increase of SSB is due to muscle fiber regeneration following necrosis and inflammation. In muscle diseases with RV, however, SSB-positive myonuclei were observed in small angulated fibers and in morphologically normal fibers, regardless of necrosis, regeneration or inflammation. These findings suggest that muscle diseases with RV may share a common pathological process involving DNA damage.

It is proposed that alpha-synucleinopathy initially affects the medulla oblongata and then progresses to more rostral brain areas ("Braak hypothesis"). According to this hypothesis, substantia nigra is affected in the later stages of PD. Another region affected in the earlier stages was reported to be olfactory bulb, although the following processes were not described in detail. On the other hand, several lines of evidence suggest that non-motor symptoms including constipation, depression, REM-sleep behavior disorder (RBD) and hyposmia may be prodromal symptoms in PD. The pathological staging postulated by the Braak hypothesis is in good agreement with the fact that these non-motor symptoms precede motor symptoms in PD, because affected brain areas in the early stages, such as dorsal vagal nucleus, locus ceruleus and olfactory bulb, are related to these non-motor features. Recently, it was reported that although half of brains corresponded to the Braak hypothesis, there were a high proportion of cases which did not fit the Braak's staging system and majority of the latter demonstrated amygdale-predominant alpha-synucleinopathy. It was also demonstrated that the Lewy pathology in olfactory bulb was closely related to the presence of alpha-synuclein pathology in amygdala. The amygdala is one of the main systems in odor perception and in PD, cortical neurons in corticomedial complex of amygdale, which have major olfactory connections, are selectively affected even in the early stages of the disease. We recently obtained the data suggesting that metabolic changes in the amygdala were associated with low scores in odor identification test. These data suggest that not only the olfactory bulb, but also the amygdala is also responsible for hyposmia in PD and that there may be another pathological process, which starts from the olfactory bulb and involves the amygdala.

To explore pathogenesis of synucleinopathy including Parkinson's disease and multiple system atrophy, we developed cellular model for synucleinopathy. In this experimental model, alpha-synuclein was overexpressed in SH-SY5Y cells, which were then exposed to mitochondrial toxins. The data thus obtained suggested the followings. (1) By the treatment with rotenone, wild type alpha-synuclein overexpressing cells demonstrated intracellular aggregations, which shared a number of features with Lewy bodies. (2) The aggregate formation of alpha-synuclein may be cytoprotective. (3) The catechol-derived quinones are candidate molecules to facilitate the oligomer formation of a-synuclein. (4) The cells overexpressing S129A mutant showed few aggregations. It is suggested that phosphorylation at serine 129 is essential for aggregate formation. (5) In wild-type alpha-synuclein cells treated with rotenone, unfolded protein response (UPR) markers were induced prior to the induction of mitochondrial disruption and caspase-3 activation. (6) On the other hand, the S129A mutant failed to activate these UPRs. Thus it seems plausible that alpha-synuclein toxicity is dependent on the phosphorylation at S129.

A 61-year-old man presented with diffuse myalgia and arthralgia. The physical examination showed diffuse musculoskeletal tenderness and joint stiffness without the presence of synovitis or arthritis. Nerve conduction study showed combined mild axonal degenerative and demyelinating change. Muscle biopsy analysis showed unspecific mild muscle atrophy with myopathic changes found on electromyography. A diagnosis of isolated adrenocorticotropic hormone deficiency was made on the basis of endocrinological evaluation. The widespread musculoskeletal pain resolved as a result of administration of physiological dose of hydrocortisone replacement. In a patient with unexplained rheumatic symptoms, adrenocortical insufficiency should be considered in the possible aetiology.

Organising pneumonia (OP) is an inflammatory lung disease with distinctive clinicopathological features. OP can be evident during the course of rheumatoid arthritis (RA) with increased disease activity. The authors report an OP associated with RA case in which pulmonary symptoms preceded the onset of joint symptoms. An OP patient with elevated serum anticyclic citrullinated peptide antibody is likely to manifest RA in the near future, reflecting its high disease activity. Thus, an early rheumatologic consultation should be taken into consideration to make an early decision to initiate disease-modifying antirheumatic drugs therapy.

The Irabu Bridge connects Miyako Island to Irabu Island and it is now under construction. A causeway was constructed between the foot of the bridge and its central part using land reclamation method, resulting in formation of wave shelter zone on both sides of the causeway. After the construction of the causeway, marked beach changes occurred on the reef coast, and egg-laying sites of sea turtles were lost. The BG model was applied to investigate the stabilization method of the beach. Most suitable measure was to use groins as well as the beach nourishment.

This is a report on the clinical courses and pathological findings in two gay male patients with acquired immunodeficiency syndrome (AIDS) infected in Japan. Case 1. A 39 year-old Japanese homosexual male was diagnosed as amebic dysentery complicated with liver abscess on admission. He was placed on Metronidazole with complete relief. Serological tests was positive for AIDS. On second admission, he was found to have pneumocystis carinii pneumonia (PCP) and cytomegalo-viral uveitis. Administration of Pentamidine was partially effective, however the therapy with Azidothimidine was discontinued by bone marrow suppression. On his third admission, he suffered from cryptococcal meningitis and therapy-resistant fungusemia. Finally he died of recurrent pneumonia regardless of appropriate therapies. Autopsy proved extended cryptococcal infection in the brain, meninx, lungs, liver and kidney, and cytomegalo-infection in the lungs, liver and kidney. Furthermore, atypical mycobacteriosis was found in the lymph nodes. There was no active findings compatible with PCP. Case 2. A 44 year-old Japanese homosexual male was admitted with oral candidiasis and diagnosed as AIDS related complex. He suffered from pneumonia with marked improvement on sulfamethoxazole-Trimethoprim. On his second admission, he developed diarrhea and was found to be infected with Giardia lambia. In addition, cytomegalo-viral infection damaged his eye sight. He died of pneumonia and meningitis shortly there after. Autopsy proved a cytomegalo-viral infection in the lung and colon, old lesions possibly caused by PCP in the lungs, and suppurative meningitis in the meninx. These experiences confirm that AIDS patients can be exposed to several opportunistic infections at the same time in the multiple organs. Furthermore, it is suggested that homosexual patients with AIDS may have unique opportunistic infections such as amebic dysentery or Giardia lamblia unlike other AIDS patients related to hemophilia.

We identified a novel mutant line lacking an α-subunit. The line was obtained by subjecting to gamma-ray irradiation Kari-kei 434, a genetically fixed line with a low level of β-conglycinin which is characterized by the absence of the α '-subunit and low levels of both the α -and β-subunits of β -conglycinin, one of the major soybean storage pro-teins. The mutant line which lacks both the α - and α '-subunits went through two generations without displaying any physiological abnormalities. We concluded that the induced trait characterized by the lack of the α -subunit is truly inherited.

The mode of inheritance of lipoxygenase-2 (L-2) in soybean seeds was determined from progeny of a cross between PI 86023 (L-2-less) and Suzuyutaka (normal) soybeans. Immunological and electrophoretic analyses of F2 and F3 seeds from the cross indicated that the absence of L-2 isozyme was due to a single allele recessive to an allele controlling the presence of the isozyme. The gene symbols. Lx2 and lx2 are assigned to the alleles for the presence and absence of L-2 isozyme, respectively. Electrophoretic analysis of a total of 474 F2 seeds from the reclprocal crosses between PI 86023 (L-2-less) and Tohoku N0.74 (L-3-less) indicated that the Lx2 locus is independent of the Lx3 locus. Electrophoretic analysis of a total of 157 F2 seeds from the cross between PI 86023 and a L-1 ?? -3-less double mutant line indicated that the Lx3 locus is independent of the Lx1 and Lx2 loci, respectively, being consistent with the no linkage between the Lx2 and Lx3 loci indicated by the analysis of F2 seeds from the former reciprocal crosses. In this study, two types of double mutant seeds were identified that lacked both L-1 and L-3, and both L-2 and L-3 isozymes at the same time. However, no double mutant seeds were identified that lacked both L-1 and L-2 isozymes, so far. The L-1.L-3-less and L-2.L-3-less double mutant seeds germinated, grew and matured normally to produce F3 seeds that lacked both L-1 and L-3, and both L-2 and L-3 isozymes, respectively.

Comparaison ďechantillons apparies ďetudiants japonais et americains (niveau lycee et college universitaire) relativement aux six valeurs que mesure ľinventaire Survey of Personal Values. On a ďabord teste la comparabilite des “valeurs” dans la langue originale de ľinventaire et dans la traduction japonaise : ľanalyse factorielle des items traduits et les correlations calculees entre ľinventaire en japonais et un choix ďechelles japonaises de personnalite donnent des garan- ties a cet egard. Chez les etudiants japonais des deux sexes et des deux niveaux scolaires, on trouve des moyennes plus faibles pour ce qui est de ľorientation pratique de ľesprit, la reussite et la variete, et des moyennes plus elevees pour ľordre et ľorientadon vers un but. Ainsi, les Japonais accordent moins ďimpor- tance aux considerations materialistes (reussite individuelle, changement ou diversite) mais ils donnent plus de valeur a un mode de vie organise et routinier et au fait de chercher systematiquement a realiser d...