High levels of LDL-cholesterol rescue the neonatal mortality associated with afibrinogenemia in mice.
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Afibrinogenemia↗
Biomedical subjects
T Iwaki
Publications and source records attributed to T Iwaki.
Auditory and tactile processing in a postmeningitic deaf-blind patient with a cochlear implant.
The authors examined the neural function of a postmeningitic deaf-blind patient who regained his hearing with a multichannel cochlear implant. Auditory stimuli activated the temporal cortices of both sides in a manner similar to that of controls, reflecting the successful recruitment of the auditory cortex after implantation. The patient's occipital lobes were deactivated during the tactile language task, the results of which were completely different from those before cochlear implantation.
Adult↗
Expression of foreign type I ribulose-1,5-bisphosphate carboxylase/ oxygenase (EC 4.1.1.39) stimulates photosynthesis in cyanobacterium Synechococcus PCC7942 cells.
A reporter gene assay revealed that promoters derived from Synechococcus PCC7942 (S.7942) psbAI and Synechocystis PCC6803 (S.6803) psbAII were suitable for the expression of foreign ribulose-bisphosphate carboxylase (RuBisCO; EC 4.1.1.39) in S.7942 cells. Transformational vectors with a promoter and a foreign RuBisCO gene, cvrbc originated from Allochromatium vinosum, were constructed on a binary vector, pUC303, and introduced to S.7942 cells. When the cvrbc was expressed with the S.7942 psbAI promoter, the total RuBisCO activity increased 2.5- to 4-fold than that of the wild type cell. The S.6803 psbAII promoter increased the activity of the transformant 1.5-2 times of that of wild type cell. There was a significant increase in the rate of photosynthesis depending on the increase of RuBisCO activity. The maximum rate of photosynthesis of the transformant cell was 1.63 times higher than that of the wild type under the illumination of 400 micromol m(-2) s(-1), at 20 mM bicarbonate and at 30 degrees C. Although the photosynthesis of the higher plant is limited by the ability of photosystems under high irradiance and the high CO(2 )concentration, that of the S.7942 cell is limited by the RuBisCO activity, even at high CO(2) concentrations and under high irradiance.
Gene Expression↗
Clusterin expression in follicular dendritic cells associated with prion protein accumulation.
Peripheral accumulation of abnormal prion protein (PrP) in variant Creutzfeldt-Jakob disease and some animal models of transmissible spongiform encephalopathies (TSEs) may occur in the lymphoreticular system. Within the lymphoid tissues, abnormal PrP accumulation occurs on follicular dendritic cells (FDCs). Clusterin (apolipoprotein J) has been recognized as one of the molecules associated with PrP in TSEs, and clusterin expression is increased in the central nervous system where abnormal PrP deposition has occurred. We therefore examined peripheral clusterin expression in the context of PrP accumulation on FDCs in a range of human and experimental TSEs. PrP was detected immunohistochemically on tissue sections using a novel highly sensitive method involving detergent autoclaving pretreatment. A dendritic network pattern of clusterin immunoreactivity in lymphoid follicles was observed in association with the abnormal PrP on FDCs. The increased clusterin immunoreactivity appeared to correlate with the extent of PrP deposition, irrespective of the pathogen strains, host mouse strains or various immune modifications. The observed co-localization and correlative expression of these proteins suggested that clusterin might be directly associated with abnormal PrP. Indeed, clusterin immunoreactivity in association with PrP was retained after FDC depletion. Together these data suggest that clusterin may act as a chaperone-like molecule for PrP and play an important role in TSE pathogenesis.
Adolescent↗
Delayed inflammatory responses to endotoxin in fibrinogen-deficient mice.
Severe inflammation leads to haemostatic abnormalities, such as the development of microvascular thrombi. As a result, ischaemia-related downstream organ damage can occur. The present study demonstrates that mice with a total deficiency of fibrinogen (Fg(-/-)) present with altered responses to challenge with Gram-negative lipopolysaccharide (LPS). Early survival in response to continuous LPS challenge was increased in Fg(-/-) mice and histological findings indicated that this improvement correlated with a lack of fibrin deposition in organs. Neutrophils appeared early in the lungs of challenged wild-type (WT) mice, but occurred in Fg(-/-) mice at later times. This delayed response in Fg(-/-) mice was confirmed by studies that showed a strong dependence on Fg of binding of neutrophils to endothelial cells in the presence of LPS. While cytokines were also elevated in both WT and Fg(-/-) mice, their levels were generally lower at early times in this latter group. The time course of MIP-2 expression correlated with the occurrence of pulmonary leakage after LPS challenge, which was delayed in Fg(-/-) mice. These results suggest that fibrin(ogen) plays a role as an early mediator in the cross-talk between coagulation and inflammation.
Afibrinogenemia↗
Static and dynamic structures of spherical nonionic surfactant micelles during the disorder-order transition.
We have investigated the static and dynamic structures of nonionic surfactant micelles, a C(12)E(8)/water binary system, during the disorder-order transition using small angle x-ray scattering, static light scattering, and dynamic light scattering techniques. In the disordered phase, the micelles have spherical shape and intermicellar interactions are governed by the hard core and weak long ranged attractive potentials. With increase of the micellar concentration, the disordered micelles transform to the three characteristic ordered micellar phases, a hexagonally close packed lattice, a body centered cubic lattice, and an A15 lattice having area-minimizing structure. The stability of these phases is well explained by balance of a close packing rule and a minimal-area rule proposed by Ziherl and Kamien [Phys. Rev. Lett. 85, 3528 (2000)]. The role of hydrodynamic interactions in surfactant micellar solutions was compared with that in hard sphere colloidal particle suspensions.
Journal Article↗
Fatal familial insomnia with an unusual prion protein deposition pattern: an autopsy report with an experimental transmission study.
We recently performed a post-mortem examination on a Japanese patient who had a prion protein gene mutation responsible for fatal familial insomnia (FFI). The patient initially developed cerebellar ataxia, but finally demonstrated insomnia, hyperkinetic delirium, autonomic signs and myoclonus in the late stage of the illness. Histological examination revealed marked neuronal loss in the thalamus and inferior olivary nucleus; however, prion protein (PrP) deposition was not proved in these lesions by immunohistochemistry. Instead, PrP deposition and spongiform change were both conspicuous within the cerebral cortex, whereas particular PrP deposition was also observed within the cerebellar cortex. The abnormal protease-resistant PrP (PrP(res)) molecules in the cerebral cortex of this case revealed PrP(res) type 2 pattern and were compatible with those of FFI cases, but the transmission study demonstrated that a pathogen in this case was different from that in a case with classical FFI. By inoculation with homogenate made from the cerebral cortex, the disease was transmitted to mice, and neuropathological features that were distinguishable from those previously reported were noted. These findings indicate the possibility that a discrete pathogen was involved in the disease in this case. We suggest that not only the genotype of the PrP gene and some other as yet unknown genetic factors, but also the variation in pathogen strains might be responsible for the varying clinical and pathological features of this disease.
Amyloid beta-Protein Precursor↗
Characterization of Schizosaccharomyces pombe mutants defective in vacuolar acidification and protein sorting.
The vacuolar H+-ATPases (V-ATPases) are ATP-dependent proton pumps responsible for acidification of intracellular compartments in eukaryotic cells. To investigate the functional roles of the V-ATPase in Schizosaccharomyces pombe, the gene vma1 encoding subunit A or vma3 encoding subunit c was disrupted. Both deletion mutants lost the capacity for vacuolar acidification in vivo, and showed sensitivity to neutral pH or high concentrations of divalent cations including Ca2+. The delivery of FM4-64 to the vacuolar membrane and accumulation of Lucifer Yellow CH were strongly inhibited in the vma1 and vma3 mutants. Moreover, deletion of the S. pombe vma1+ or vma3+ gene resulted in pleiotropic phenotypes consistent with lack of vacuolar acidification, including the missorting of vacuolar carboxypeptidase Y, abnormal vacuole morphology, and mating defects. These findings suggest that V-ATPase is essential for endocytosis, ion and pH homeostasis, and for intracellular targeting of vacuolar proteins and vacuolar biogenesis in S. pombe.
Cathepsin A↗
Competition between interchain and intrachain phase segregation.
Single-molecule observations of giant DNA have clarified that individual molecules undergo a marked discrete transition between an elongated coil state and a compact globule state. There is a relatively wide region of coexistence between the coil and the globule states, i.e., interchain phase segregation, with a change in intensive variables such as the concentration of the condensing agent, salt concentration, temperature. Very recently, the coexistence of coil and globule conformations within a single long DNA chain, i.e., intrachain phase segregation, has been reported under certain experimental conditions. In this study, we investigated general conditions for intrachain phase segregation in a single polyelectrolyte molecule, based on a simple statistical model. We consider the contribution of condensed counterions and the interaction energy of a charged coiled region. Intrachain phase segregation is stable with regard to free energy within a suitable parameter region. Our results suggest that intrachain phase segregation occurs when the electrostatic screening effect by the salt solution is negligible or when the screening effect is large and there is attractive interaction between polyelectrolyte segments.
Biophysical Phenomena↗
Rapid extraction of emotional expression: evidence from evoked potential fields during brief presentation of face stimuli.
Although the emotional expression of faces is believed to be accessed rapidly, previous ERP studies hardly found correlates of these processes. Here, we report findings from a study that investigated dichoptic binocular interaction using emotional face stimuli. Thirty-one subjects were briefly presented with schematic normal and scrambled faces (of neutral, positive, or negative expression) that occurred simultaneously in the left and right visual fields. Stimuli for both eyes could be congruent (control) or incongruent (dichoptic). Subjects decided which of the superimposed images in both hemi-fields appeared more "face-like" and during this task, the EEG was recorded from 30 channels. VEPs were analysed topographically according to the influence of the different experimental conditions (defined by presentation form, emotional expression, and location). Behavioural responses to the ambiguous dichoptic stimuli demonstrated a functional eye dominance not related to visual acuity and conventional eye preference. Electrophysiological data revealed three components with mean latencies of 85, 160, and 310 ms. Topography of the second component (equivalent to the face-related N170) differed in left-right and anterior-posterior direction compared with simple checkerboard stimuli. Dichoptic presentation caused reduced field strength of all three, and increased latency of the first component. Faces with negative expression yielded largest field strength of the second and third components. Besides that, emotional expression affected topography not only of late, but also the first component. This provides new evidence about the timing of perceptual processes related to facial expression, indicating that already VEP components occurring at 80-90 ms are sensitive to emotional content.
Adult↗
Astrocytoma of the pituitary gland (pituicytoma): case report.
A 34-year-old man presented with a 4-month history of visual obscuration. Magnetic resonance imaging showed a solid, discrete, contrast-enhancing pituitary mass with suprasellar extension. Surgery, which was performed via a transsphenoidal approach, disclosed the pituitary tumor to be a fibrillary astrocytoma (pituicytoma). This case report contains the clinical and neuroimaging features of this rare tumor of the neurohypophysis, which masqueraded as a pituitary adenoma.
Adult↗
Potential remedy against Echinococcus multilocularis in wild red foxes using baits with anthelmintic distributed around fox breeding dens in Hokkaido, Japan.
The effect of bait-delivered anthelmintic to reduce the prevalence of Echinococcus multilocularis in wild red foxes was evaluated in Koshimizu, in the eastern part of Hokkaido, Japan. The study area (200 km2) was divided into baited and non-baited sections. The anthelmintic baits were distributed around fox den sites in the baited section every month for 13 months. After 1 year of the anthelmintic bait distribution, the prevalence of E. multilocularis in foxes, evaluated either by the parasite egg examination (from 27.1 to 5.6%) or coproantigen ELISA (from 59.6 to 29.7%), decreased in the baited section contrasting to that in the non-baited section (parasite egg: from 18.8 to 24.2%; ELISA: from 41.9 to 45.8%). The prevalence of E. multilocularis in grey red-backed vole Clethrionomys rufocanus, caught around fox dens, born after bait distribution also decreased and was significantly lower than that in non-baited section. However, within the study periods, the coproantigen-positive rate in fox faeces sporadically increased, while egg-positive rate constantly decreased. Since coproantigen ELISA can detect pre-patent infection, this observation indicates that reinfection pressure in the baited section was still high even after the 13 months of anthelmintic bait distribution. Therefore, the bait distribution longer than our study period is required for the efficient control of E. multilocularis in wild red fox population.
Animals↗
Images in cardiology. A case of spontaneous closure of coronary artery fistula with familial hypercholesterolaemia.
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Adult↗
Altered expressions of glutamate transporter subtypes in rat model of neonatal cerebral hypoxia-ischemia.
Glutamate transporters are essential for maintaining the extracellular levels of glutamate at synaptic clefts and are regulated developmentally in a subtype-specific manner. We investigated chronological changes of immunoreactivities for glial glutamate transporters GLAST and GLT-1 and a neuronal glutamate transporter, EAAC1, in postnatal 7-day-old rat neocortices and hippocampi at 12, 24, 48 and 72 h after hypoxia-ischemia. Glutamate transporter subtypes are differentially expressed in the ischemic core and the boundary area of the neonatal rat brain with hypoxia-ischemia. Expressions of these glutamate transporters decreased in the ischemic core at 12 h, then immunoreactivities for GLAST and GLT-1 were recovered at the hippocampus. This was accompanied by a GFAP-positive gliosis at 72 h, whereas these immunoreactivities were reduced at the neocortex in the ischemic core. Glial glutamate transporters, especially GLAST, were noted in some astrocytes appearing as apoptosis as well as shrunken pyramidal neurons mainly in the boundary area of the neocortex. Increased perikaryal expression of EAAC1 was associated with that of MAP2 at the border of the boundary area. These temporal and regional expressions of glutamate transporters may contribute towards understanding the excitotoxic cell death mechanism in hypoxic-ischemic encephalopathy during the perinatal period.
Amino Acid Transport System X-AG↗
Association of HSPB2, a member of the small heat shock protein family, with mitochondria.
We previously identified HSPB2, a new member of the small heat shock protein family, expressed in heart and skeletal muscles. In this study, we used a polyclonal anti-HSPB2 antibody and examined the subcellular localization of HSPB2 in differentiated C2C12 cells, KNS-81 cells, and NIH3T3 transfectants expressing human HSPB2. Double staining with anti-HSPB2 and various markers for cytoplasmic structures showed that HSPB2 was present in the cytosol as granules, some of which colocalized with mitochondria. This colocalization was not altered by a colchicine treatment, indicating that it is independent of microtubules. The subcellular fractionation of differentiated C2C12 cells revealed that HSPB2 was mainly detected in the postmitochondrial supernatant, but mild heat treatment enriched the amount of HSPB2 in the mitochondrial fraction. The expression of HSPB2 protected the cells from heat-induced cell death. In addition, Northern blot analysis revealed that expression of HSPB2 mRNA is higher in slow-twitch muscle than in fast-twitch muscle, which correlates with the amounts of mitochondria present in these two types of tissue. Taken together, these results suggest that HSPB2 may not localize in the matrix, but rather associates with the outer membrane components of the mitochondria and thus plays a role in the stress response.
Animals↗
Expression of hMTH1 in the hippocampi of control and Alzheimer's disease.
The oxidized purine nucleoside triphosphatase, hMTH1, has a critical role towards preventing errors caused by oxidized purine nucleoside triphosphates such as 8-oxo-dGTP and 2-hydroxy-dATP. We investigated the immunohistochemical expression of hMTH1 in human hippocampal postmortem tissues representing non-neurological disease and Alzheimer's disease (AD). In the non-neurological subjects the hMTH1 protein was enriched in the stratum lucidum at CA3 corresponding to mossy fiber synapses. In AD subjects, the synaptic immunoreactivities at CA3 were significantly decreased, whereas they tended to be increased at the entorhinal cortex. We suggest that the expression of hMTH1 indicates indirect evidence of oxidative stress and its regulation is regionally differentiated in AD.
Aged↗
Limbic encephalitis associated with recurrent thymoma: a postmortem study.
The authors report an autopsied patient with limbic encephalitis and recurrent thymoma. The immunohistochemical study showed selective depositions of immunoglobulin G on the neurons in the limbic system and the tumor cells of the recurrent thymoma. The immunoblotting study detected two types of antibodies that react with the human brain, rat brain, and rat thymus.
Brain↗
Dorsal horn lesion resulting from spinal root avulsion leads to the accumulation of stress-responsive proteins.
The aim of this study was to demonstrate acute to subacute molecular episodes in the dorsal horn following root avulsion using immunohistochemical methods with the markers for synapses, astrocytes and such stress-responsive molecules as heat shock proteins (Hsps) and p38 MAP kinase (p38). Among them, Hsp27 was accumulated selectively in the injured substantia gelatinosa 24 h after avulsion injury. The localization of Hsp27 in astrocytes within the substantia gelatinosa was confirmed by the double immunofluorescence method using anti-Hsp27 antibody and either anti-synaptophysin antibody or anti-glutamine synthetase antibody and by immunoelectron microscopy for Hsp27. The pattern of Hsp27 expression subsequently changed from glial pattern to punctate pattern by 7 days. Immunoelectron microscopy revealed that the punctate pattern in the subacute stage corresponded to distal parts of the astrocytic processes. Hsp27 immunoreaction was decreased 21 days after root avulsion. In the distal axotomy model, Hsp27 was accumulated later in the ipsilateral dorsal horn in a punctate pattern from 7 days after the axotomy. Phosphorylation of p38 was detected in microglia in the dorsal horn following both avulsion and axotomy. Substance P was slightly decreased in the injured substantia gelatinosa in both the avulsion and axotomy models around 14-21 days. We conclude that Hsp27 is a useful marker for demonstrating dorsal horn lesions following avulsion injury and that avulsion injury may induce Hsp27 in the dorsal horn more rapidly than distal axotomy.
Animals↗