Preventive effect of ACE inhibitor on interstitial myofibroblast formation and matrix deposition in a nephrotic model.

The nephrotic mouse (ICGN strain) is a useful model for progressive nephrotic syndrome (NS). In the present study, we demonstrated the preventive effects of enalapril, an angiotensin converting enzyme (ACE) inhibitor, on the progression of renal dysfunction and tubulo-interstitial fibrosis in the NS mice. Administration of enalapril (5 mg/dL in drinking water) to the 4-week-old NS mice for a 4-week-period did not improve their nephrotic symptoms such as albuminuria and hypoalbuminemia, but significantly suppressed the increases in blood urea nitrogen and serum creatinine levels.

Suprachiasmatic nucleus in a patient with multiple system atrophy with abnormal circadian rhythm of arginine-vasopressin secretion into plasma.

We performed a quantitative investigation of arginine-vasopressin (AVP) immunopositive neurons in the suprachiasmatic nucleus (SCN), which is the endogenous clock of the brain of a patient with multiple system atrophy (MSA) who exhibited nocturnal polyuria associated with decreased urinary specific gravity and depression of nocturnal AVP secretion. Eleven age- and sex-matched subjects were used as controls. Although, the number of AVP-positive neurons was decreased in neither the supraoptic nucleus nor the paraventricular nucleus, the number of AVP-positive neurons in the SCN was decreased and gliosis was present in the SCN.

Sporadic motor neuron disease with severe sensory neuronopathy.

We report a 62-year-old man with sporadic motor neuron disease (MND) of 52 months' duration with progressive sensory disturbance and high cerebrospinal fluid protein content. Neuropathologically, both the upper and lower motor neuron systems were severely affected, and light and electron microscopy revealed Bunina bodies and skein-like inclusions, which are characteristic of amyotrophic lateral sclerosis, in the remaining anterior horn cells. Moreover, there was severe degeneration without inflammatory infiltrates in the spinal posterior columns, spinal ganglia, and peripheral sensory nerves.

Mitochondrial calcium in relaxed and tetanized myocardium.

The elemental composition of rat cardiac muscle was determined with electron probe x-ray microanalysis (EPMA) of rapidly frozen papillary muscles and trabeculae incubated with ryanodine (1 microM) in either 1.2 or 10 mM [Ca2+]o-containing solutions, paced at 0.6 Hz or tetanized at 10 Hz.

Mutation/polymorphism scanning of glucose-6-phosphatase gene promoter in noninsulin-dependent diabetes mellitus patients.

Glucose-6-phosphatase (G6Pase) catalyzes the rate-limiting step of gluconeogenesis, and hepatic G6Pase activity is increased in diabetes. We have cloned and analyzed the human G6Pase gene promoter region and identified putative regulatory sequences for insulin, cAMP, glucocorticoid, and hepatocyte nuclear factors. The promoter region of the G6Pase gene was analyzed in 154 noninsulin-dependent diabetes mellitus patients and 90 control subjects by PCR-single strand conformation polymorphism and direct sequencing methods.

Diffuse glomerulosclerosis without tubular injury does not directly manifest renal dysfunction in nephrotic mice (ICGN strain).

Chronic renal disease is characterized by pathological conditions, caused by a progressive and irreversible loss of renal function. The nephrotic mouse (ICGN strain) is a useful model for demonstrating progressive nephrotic syndrome (NS). In the present study, we examined the natural course of kidney disease shown by the NS mice, aging 6-26 weeks, in order to characterize their pathological conditions.

Atrophy of the cerebellum and brainstem in dentatorubral pallidoluysian atrophy. Influence of CAG repeat size on MRI findings.

To elucidate how the size of the expanded CAG repeat of the gene for dentatorubral pallidoluysian atrophy (DRPLA) and other factors affect the atrophy of the brainstem and cerebellum, and the appearance of high-intensity signals on T2-weighted MRI of the cerebral white matter of patients with DRPLA, we quantitatively analyzed the MRI findings of 26 patients with DRPLA, the diagnosis of which was confirmed by molecular analysis of the DRPLA gene. When we classified the patients into two groups based on the size of the expanded CAG repeat of the DRPLA gene (group 1, number of CAG repeat units > or = 66; group 2, number of CAG repeat units < or = 65), we found strong inverse correlations between the age at MRI and the areas of midsagittal structures of the cerebellum and brainstem in group 1 but not in group 2. Multiple regression analysis, however, revealed that both the patient's age at MRI and the size of the expanded CAG repeat correlated with the areas of midsagittal structures.

Spinocerebellar ataxia type 6: CAG repeat expansion in alpha1A voltage-dependent calcium channel gene and clinical variations in Japanese population.

Autosomal dominant spinocerebellar ataxias (SCAs) are clinically and genetically a heterogeneous group of neurodegenerative disorders. Recently, mild CAG repeat expansion in the alpha1A voltage-dependent calcium channel gene has been found to be associated with a type of autosomal dominant SCA (SCA6). We analyzed 98 Japanese families with autosomal dominant SCAs, for whom CAG repeat expansions of the SCA1, SCA2, Machado-Joseph disease/SCA3, and dentatorubral-pallidoluysian atrophy genes were excluded, and 5 apparently sporadic cases of cortical cerebellar atrophy.

Markedly reduced expression of platelet c-mpl receptor in essential thrombocythemia.

Thrombopoietin (TPO) is implicated as a primary regulator of megakaryopoiesis and thrombopoiesis through binding to the cytokine receptor c-Mpl (the product of the c-mpl proto-oncogene). In an effort to determine the pathophysiological role of TPO-c-Mpl system in essential thrombocythemia (ET), we have examined the levels of serum TPO and the expression and function of platelet c-Mpl in 17 patients with ET. In spite of extreme thrombocytosis, serum TPO levels were slightly elevated or within normal range in most, if not all, patients with ET (mean +/- SD, 1.

Organization and partial sequence of the hepatocyte nuclear factor-4 alpha/MODY1 gene and identification of a missense mutation, R127W, in a Japanese family with MODY.

Hepatocyte nuclear factor-4 alpha (HNF-4 alpha) is a member of the nuclear receptor superfamily, a class of ligand-activated transcription factors. A nonsense mutation in the gene encoding this transcription factor was recently found in a white family with one form of maturity-onset diabetes of the young, MODY1. Here, we report the exon-intron organization and partial sequence of the human HNF-4 alpha gene.

[Influence of preanesthetic medication on the effect of a local anesthetic tape].

We investigated the influence of preanesthetic medication on the pain relieving effect of the lidocaine tape during needle insertion for venous cannulation. Ninety patients scheduled for elective surgery were randomly divided into three groups of 30 each; patients without preanesthetic medication (group N), patients who received 0.1 mg.

Presence and comparison of Ca2+ transport activity of annexins I, II, V, and VI in large unilamellar vesicles.

This study evaluated whether annexins I, II and VI possess Ca2+ transport activity in phospholipid membranes by the burst method, and the activity of each was compared with that of annexin V. Briefly, in the presence of 400 microM Ca2+, each annexin at 50 nM was added to large unilamellar vesicles (LUV) which were then burst in fura-2 solution with 0.2% Triton X-100, followed by examination of Ca2+ signals.

Preoperative detection of the composition of atherosclerotic plaque in the carotid artery using ultrasonography and magnetic resonance imaging.

Preoperative identification of the components of atherosclerotic plaque was attempted using ultrasonography in five patients and magnetic resonance (MR) imaging in three patients before carotid endarterectomy. The correlation between surgical and histological findings, and preoperative ultrasonography and MR imaging was evaluated. Plaque consisting predominantly of calcification appeared as bright echo on ultrasonography and was tough.

Hemorrhagic and nonhemorrhagic stroke: diagnosis with diffusion-weighted and T2-weighted echo-planar MR imaging.

Purpose: To determine if diffusion- and T2-weighted echo-planar magnetic resonance (MR) imaging can be used to detect acute hemorrhagic stroke and to differentiate hemorrhagic from nonhemorrhagic stroke.

Materials And Methods: A total of 118 examinations (diffusion- and T2-weighted MR imaging) in 19 patients with 27 nonhemorrhagic strokes and in six patients with seven hemorrhagic strokes were performed. The ratios of apparent diffusion coefficient and of signal intensity on T2-weighted MR images in lesions to those in contralateral control areas were calculated.

Neuropathology of sporadic amyotrophic lateral sclerosis of long duration.

We performed post-mortem examinations of three patients with progressive neurogenic amyotrophy of long duration. One patient had been clinically diagnosed as having sporadic amyotrophic lateral sclerosis (ALS) and two had been diagnosed with progressive spinal muscular atrophy (PSMA). The disease durations were 10, 17 and 20 years, respectively, and all of the patients died of respiratory failure with no artificial respiratory support.

MR angiography of intracranial aneurysms: a comparison of 0.5 T and 1.5 T.

The objective of this paper is to compare the diagnostic efficacy of 3 DFT time-of-flight MR angiography (MRA) at middle-field-strength and high-field-strength in diagnosis of intracranial aneurysms. Thirty-one patients, including 26 patients with angiographically confirmed intracranial aneurysms (n = 28), underwent MRA at 0.5 and 1.

Intracranial vascular stenosis and occlusion: MR angiographic findings.

Purpose: To investigate whether obtaining axial source images from three-dimensional Fourier transform (3DFT) time-of-flight MR angiography improves the detection of intracranial vascular stenosis and occlusion if added to maximum-intensity projection (MIP) images.

Methods: The angiograms of 103 patients who had MR angiography for evaluation of possible intracranial vascular disease were reviewed retrospectively in a quantitative and nonquantitative fashion. Diameters of vessels on MR angiograms were measured quantitatively by two reviewers using a magnifying loupe and compared with the results from conventional angiograms.

Hepatocyte nuclear factor-4 alpha gene mutations in Japanese non-insulin dependent diabetes mellitus (NIDDM) patients.

A mutation in the hepatocyte nuclear factor-4 alpha (HNF-4 alpha) gene has been recently reported to cause maturity-onset diabetes of the young (MODY) (Yamagata, Furuta, et al., 1996). The mutation can also be a good candidate for the responsible gene of non-insulin dependent diabetes mellitus (NIDDM).

Functional role of cation-independent mannose 6-phosphate/insulin-like growth factor II receptor in cell adhesion and proliferation of a human myeloma cell line OPM-2.

The molecular mechanism underlying the interaction between myeloma cells and stromal cells was investigated by using a human myeloma cell line (OPM-2) and human umbilical vein endothelial cells (HUVECs). Adhesion of OPM-2 cells to HUVECs was found to be significantly augmented with treatment of OPM-2 cells with an alpha-glycosidase inhibitor, castanospermine (CSP). The treatment of OPM-2 cells with CSP resulted in alteration of oligosaccharide structures of cell surface glycoproteins particularly at molecular weight of 220 kD (GP220).

The biologic properties of recombinant human thrombopoietin in the proliferation and megakaryocytic differentiation of acute myeloblastic leukemia cells.

Thrombopoietin (TPO) is implicated as a primary regulator of megakaryopoiesis and thrombopoiesis. However, the biologic effects of TPO on human acute myeloblastic leukemia (AML) cells are largely unknown. To determine if recombinant human (rh) TPO has proliferation-supporting and differentiation-inducing activities in AML cells, 15 cases of AML cells that were exclusively composed of undifferentiated leukemia cells and showed growth response to rhTPO in a short-term culture (72 hours) were subjected to long-term suspension culture with or without rhTPO.

Expression and distribution of glucose-6-phosphatase catalytic subunit messenger RNA and its changes in the diabetic state.

Glucose-6-phosphatase (G6Pase) plays a major role in gluconeogenic pathway. To analyze its regulation, we have cloned a full-length cDNA for G6Pase catalytic subunit from the rat liver. In the cloned cDNA, a 492 base insertion, respective to the previously reported sequence, was found in the 3'-noncoding region.