A Renal Variant of Fabry Disease Diagnosed by the Presence of Urinary Mulberry Cells.

Fabry disease is a lysosomal storage disorder caused by a deficiency of α-galactosidase A. This disease is classified into two types, namely a classical and variant type. We herein present the case of a 36-year-old man who showed a renal variant of Fabry disease and was diagnosed at an early stage by the presence of mulberry cells.

Hyperthyroidism associated with obesity-related glomerulopathy-like pathologic features.

Thyroid dysfunction is related to many kidney diseases. We herein present the case of a 39-year-old woman who exhibited obesity-related glomerulopathy-like pathologic features in combination with hyperthyroidism. She displayed hyperthyroidism in spite of receiving anti-thyroid drug treatment, with massive proteinuria (4.

Human parvovirus B19-induced acute glomerulonephritis: a case report.

Human parvovirus B19 (HPV B19) infection is well known as a cause of erythema infectiosum in children. Acute glomerulonephritis due to HPVB19 infection is rarely observed in adults. Here, we present the case of a 45-year-old female who showed acute glomerulonephritis induced by HPVB19 infection with various autoantibodies.

A case of focal segmental glomerulosclerosis with myeloid bodies.

The presence of myeloid bodies in electron microscopy is a characteristic finding of Fabry's disease. Here, we present a male patient, whose renal biopsy findings suggested the coexistence of focal segmental glomerulosclerosis and Fabry's disease, because of the presence of segmental hyalinosis and/or sclerosis in glomeruli and myeloid bodies in electron microscopy. But finally, Fabry's disease was excluded as a diagnosis because the α-galactosidase A activity in leukocyte and plasma in this patient was within normal limits.

Serum TNF-related and weak inducer of apoptosis levels in septic shock patients.

Capillary permeability is a tightly regulated feature of microcirculation in all organ beds. In sepsis, this feature is fundamentally altered. We have previously reported elevated levels of angiopoietin-2 in patients with septic shock, and have investigated tumor necrosis factor (TNF)-related and weak inducer of apoptosis (TWEAK), which mediates both angiogenesis and inflammation, in those patients.

Angiopoietin balance in septic shock patients treated by direct hemoperfusion with polymyxin b-immobilized fiber.

Capillary permeability is a tightly regulated feature of microcirculation in all organ beds; however, in sepsis this feature is fundamentally altered. We previously reported elevated levels of vascular endothelial growth factor and its receptor (fms-like tyrosine kinase-1) in patients with septic shock, then investigated two kinds of angiopoietins in those patients. An enzyme-linked immunoassay was used to measure serum angiopoietin-1 and -2 levels in 12 patients with septic shock who were treated by direct hemoperfusion with a polymyxin B-immobilized fiber column (DHP-PMX).

Analysis of T-cell receptor usage in myeloperoxidase--antineutrophil cytoplasmic antibody-associated renal vasculitis.

Background: Bacterial superantigens produced by Staphylococcus aureus may be associated with the onset of proteinase-3 antineutrophil cytoplasmic antibody (PR3-ANCA)-associated vasculitis, including Wegener's granulomatosis. We investigated T-cell subsets to assess the superantigens present in patients with myeloperoxidase-antineutrophil cytoplasmic antibody (MPO-ANCA)-associated vasculitis.

Methods: Peripheral-blood mononuclear cells (PBMC) obtained from 40 normal controls and ten patients with MPO-ANCA-associated vasculitis were stained with fluorescence-labeled monoclonal antibodies against T-cell markers, including 17 variable regions of T-cell receptor beta-chains (TCR-Vbeta) and were then analyzed using flow cytometry.

Discovery of dense aggregations of stalked crinoids in Izu-Ogasawara trench, Japan.

Stalked crinoids are recognized as living fossils that typically inhabit modern deep-water environments exceeding 100 m. Previous records of stalked crinoids from hadal depths (exceeding 6000 m) are extremely rare, and no in-situ information has been available. We show here that stalked crinoids live densely on rocky substrates at depths over 9000 m in the Izu-Ogasawara Trench off the eastern coast of Japan, evidenced by underwater photos and videos taken by a remotely operated vehicle.

Vascular endothelial growth factor and soluble fms-like tyrosine kinase-1 in septic shock patients treated with direct hemoperfusion with a polymyxin B-immobilized fiber column.

Sepsis is characterized by a systemic inflammatory response to a microbial pathogen. In sepsis, capillary permeability is a tightly regulated feature of microcirculation in all organ beds and is fundamentally altered. We investigated the vascular endothelial growth factor (VEGF) level as a vascular permeability factor and the soluble fms-like tyrosine kinase-1 (Flt-1) level as an antagonist of the VEGF receptors.

Gac two-component system in Pseudomonas syringae pv. tabaci is required for virulence but not for hypersensitive reaction.

Pseudomonas syringae pv. tabaci 6605 causes wildfire disease on host tobacco plants. To investigate the regulatory mechanism of the expression of virulence, Gac two-component system-defective mutants, DeltagacA and DeltagacS, and a double mutant, DeltagacADeltagacS, were generated.

Effects of glycosylation on swimming ability and flagellar polymorphic transformation in Pseudomonas syringae pv. tabaci 6605.

The role of flagellin glycosylation on motility was investigated in Pseudomonas syringae pv. tabaci. The swimming activity of glycosylation-defective mutants was prominently decreased in a highly viscous medium.

A homologue of the 3-oxoacyl-(acyl carrier protein) synthase III gene located in the glycosylation island of Pseudomonas syringae pv. tabaci regulates virulence factors via N-acyl homoserine lactone and fatty acid synthesis.

Pseudomonas syringae pv. tabaci 6605 possesses a genetic region involved in flagellin glycosylation. This region is composed of three open reading frames: orf1, orf2, and orf3.

Volcanism in response to plate flexure.

Volcanism on Earth is known to occur in three tectonic settings: divergent plate boundaries (such as mid-ocean ridges), convergent plate boundaries (such as island arcs), and hot spots. We report volcanism on the 135 million-year-old Pacific Plate not belonging to any of these categories. Small alkalic volcanoes form from small percent melts and originate in the asthenosphere, as implied by their trace element geochemistry and noble gas isotopic compositions.