Apical hypertrophic cardiomyopathy with aneurysmal degeneration of the apex.
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Adult↗
Biomedical subjects
Akira Sato
Publications and source records attributed to Akira Sato.
Use of diamide-acridine orange fluorescence staining to detect aberrant protamination of human-ejaculated sperm nuclei.
OBJECTIVE: To investigate the influence of human sperm nuclear chromatin on fertilization. DESIGN: Prospective study. SETTING: Assisted reproductive technology unit at a university teaching hospital. PATIENT(S): Fifty men starting an IVF-ET program. INTERVENTION(S): Epifluorescent microscopic observation of human-ejaculated sperm nuclei stained with diamide-acridine orange. Sodium dodecyl sulfate-polyacrylamide gel electrophoresis (SDS-PAGE) analysis of extracted sperm nucleoproteins. MAIN OUTCOME MEASURE(S): Usefulness of diamide-acridine orange in analysis of human sperm nuclear chromatin and fertilization ability. RESULT(S): There was no correlation between the semen parameters and the diamide-acridine orange observation. A positive correlation was observed between the fertilization rate after conventional IVF and the green-type increase ratio (percentage of green-pattern sperm after diamide-acridine orange staining/percentage of green-pattern sperm after acridine orange staining). Furthermore, it was suggested by SDS-PAGE that structural differences were noticed between the fertile men and the men with sperm immaturity diagnosed after diamide-acridine orange staining. CONCLUSION(S): Diamide-acridine orange staining was a more precise method for detecting chromatin abnormalities in human-ejaculated sperm and evaluating fertilization ability than acridine orange staining alone. This method can be used as a diagnostic tool to assess the fertilization ability of human-ejaculated spermatozoa before IVF procedures.
Acridine Orange↗
Relationship between Fertilizing Ability of Ejaculated Human Spermatozoa and Its Chromatin Heterogeneity.
OBJECTIVE: The aim of this study was to examine the relationship between the fertilizing ability of ejaculated human sperm and its chromatin heterogeneity. METHOD: We used the D-AO staining method (acridine orange epifluorescence accompanied by diamide, a thiol oxidizing agent) to analyze the sperm chromatin structure of infertile patients with IVF-ET treatment. SDS-PAGE was performed to analyze the sperm nuclear proteins collected from patients with immature sperm (stained red with D-AO staining) and proven fertile men. RESULTS: 1. It was suggested that D-AO staining allowed the immature sperm to be divided into two groups. One was immature sperm, which had disturbance of S-S formation in the epididymides, and the other was sperm that which had an abnormal exchange process of nuclear proteins in the testes. 2. The stainability after D-AO staining showed no correlation with the findings of semen analysis. 3. The fertilization rate of IVF-ET was significantly correlated to the percentage of green sperm staining with D-AO staining. 4. In the pregnant group after IVF-ET, it was noticed that sperm of the green type with D-AO staining was increased in comparison with the non-pregnancy group. 5. The fertilization rate in the group of the sperm stained red with D-AO staining was increased to 73.5% by ICSI. 6. Definite differences were noticed between the protein components of the patients with immature sperm and those of the proven fertile men by analysing with SDS-PAGE. CONCLUSION: D-AO staining was an efficient method for evaluating the fertilizing ability of human ejaculated sperm, and to determine an appropriate ART tool such as ICSI.
Journal Article↗
Fetal hepatic hemangioma representing non-reassuring pattern in fetal heart rate monitoring.
To the best of our knowledge, this is the first report of a non-reassuring fetal pattern caused by a hepatic hemangioma that was found during fetal heart rate monitoring. A 37 weeks' gestation, a 32-year-old-woman was referred to our hospital for evaluation of a rapidly growing fetal abdominal tumor. Fetal heart rate monitoring revealed a non-reassuring pattern, and a cesarean section was performed as a result. Examination of the 2820 g female infant suggested a hepatic hemangioma accompanying an intratumor hemorrhage and coagulopathy. The infant died 2 days after birth. The autopsy confirmed that the cause of death was a result of a hepatic hemangioma. Frequent monitoring of fetuses and ultrasound examinations are necessary for determining the timing of delivery and for a favorable prognosis.
Adult↗
Prenatal diagnosis for placental steroid salfatase deficiency with fluorescence in situ hybridization: a case of X-linked ichthyosis.
X-linked ichthyosis (XLI) is a relatively common genetic disorder that occurs in about one in every 2000-6000 male births. Clinically, XLI is characterized by a generalized scaling of the skin, with large, polygonal, dark brown scale, and more prominent on the extensor aspects of the limbs. It is known that an undetectable maternal serum, unconjugated estriol, associated with placental steroid sulfatase (STS) deficiency, may be the cause of cause of XLI. In most case, STS deficiency is caused by a complete or partial deletion of the STS gene mapped on chromosome Xp22.3. We describe here the prenatal detection of a male fetus affected with STS deficiency as a result of an undetectable unconjugated estriol in the second-trimester maternal serum screening. Microdeletion of the STS gene was confirmed by fluorescence in situ hybridization analysis of cultured amniotic fluid.
Adult↗
Zinc finger protein sall2 is not essential for embryonic and kidney development.
SALL/Sall is a mammalian homolog of the Drosophila region-specific homeotic gene spalt (sal), and heterozygous mutations in SALL1 in humans lead to Townes-Brocks syndrome. We earlier reported that mice deficient in Sall1 die in the perinatal period and that kidney agenesis or severe dysgenesis are present. We have now generated mice lacking Sall2, another Sall family gene. Although Sall2 is expressed mostly in an overlapping fashion versus that of Sall1, Sall2-deficient mice show no apparent abnormal phenotypes. Morphology and gene expression patterns of the mutant kidney were not affected. Mice lacking both Sall1 and Sall2 show kidney phenotypes comparable to those of Sall1 knockout, thereby demonstrating the dispensable roles of Sall2 in embryonic and kidney development.
Animals↗
Dual role of SRC homology domain 2-containing inositol phosphatase 2 in the regulation of platelet-derived growth factor and insulin-like growth factor I signaling in rat vascular smooth muscle cells.
Src homology domain 2 (SH2)-containing inositol phosphatase 2 (SHIP2) possesses 5-phosphatase activity and an SH2 domain. The role of SHIP2 in platelet-derived growth factor (PDGF) and IGF-I signaling was studied by expressing wild-type (WT-) and a catalytically defective (Delta IP-) SHIP2 into rat aortic smooth muscle cells by adenovirus-mediated gene transfer. PDGF- and IGF-I-induced tyrosine phosphorylation of their respective receptors and phosphatidylinositol 3-kinase (PI3-kinase) activity were not affected by the expression of either WT- or Delta IP-SHIP2. SHIP2 possessed 5'-phosphatase activity to hydrolyze the PI3-kinase product phosphatidylinositol 3,4,5-trisphosphate in vivo. Akt and glycogen synthase kinase 3beta are known to be downstream molecules of PI3-kinase, leading to the antiapoptotic effect. Overexpression of WT-SHIP2 inhibited PDGF- and IGF-I-induced phosphorylation of these molecules and the protective effect of poly(ADP-ribose) polymerase degradation, whereas these phosphorylations and the protective effect were enhanced by the expression of Delta IP-SHIP2, which functions in a dominant negative fashion. Regarding the Ras-MAPK pathway, PDGF- and IGF-I-induced tyrosine phosphorylation of Shc was not affected by the expression of either WT- or Delta IP-SHIP2, whereas both expressed SHIP2 associated with Shc. Importantly, PDGF and IGF-I stimulation of Shc/Grb2 binding, MAPK activation, and 5-bromo-2'-deoxyuridine incorporation were all decreased in both WT- and Delta IP-SHIP2 expression. These results indicate that SHIP2 plays a negative regulatory role in PDGF and IGF-I signaling in vascular smooth muscle cells. As the bifunctional role, our results suggest that SHIP2 regulates PDGF- and IGF-I-mediated signaling downstream of PI3-kinase, leading to the antiapoptotic effect via 5-phosphatase activity, and that SHIP2 regulates the growth factor-induced Ras-MAPK pathway mainly via the SH2 domain.
Adaptor Proteins, Signal Transducing↗
Cholesterol synthesis in mice is suppressed but lipofuscin formation is not affected by long-term feeding of n-3 fatty acid-enriched oils compared with lard and n-6 fatty acid-enriched oils.
Hypocholesterolemic activity of dietary polyunsaturated fatty acids is observed after relatively short-term but not long-term feedings, and their long-term feedings are suspected to accelerate aging through tissue accumulation of lipid peroxides and age pigments (lipofuscin). To define the long-term effects of fats and oils in more detail, female mice were fed a conventional basal diet supplemented with lard (Lar), high-linoleic (n-6) safflower oil (Saf), rapeseed oil (Rap), high-alpha-linolenic (n-3) perilla oil (Per), or a mixture of ethyl docosahexaenoate and soybean oil (DHA/Soy) from 17 weeks to 71 weeks of age. The DHA/Soy and Per groups had decreased serum cholesterol levels compared with the Lar and Saf groups, but the difference between the Lar and Saf groups was not significant. The 3-hydroxy-3-methyglutary-CoA (HMG-CoA) reductase activity in the liver was also significantly lower in the Per and DHA/Soy groups. However, no significant difference in lipofuscin contents in the brain and liver was observed among the 5 dietary groups, despite significant differences in peroxidizability indices of the dietary and/or tissue lipids. These results indicate that n-3 fatty acid-rich oils are hypocholesterolemic by suppressing hepatic HMG-CoA reductase activity compared with animal fats and high-linoleic (n-6) oil, but tissue lipofuscin contents are not affected by a long-term feeding of fats and oils with different degree of unsaturation in mice.
Acyl Coenzyme A↗
Effects of nitric oxide donors on non-pregnant and pregnant rat uterine and aortic smooth muscle.
OBJECTIVE: To compare the effects of nitric oxide (NO) donors, diethylamine/nitric oxide (DEA/NO) and nitroglycerin (NTG), on isolated uterine and aortic tissues from non-pregnant, mid and late pregnant rats. METHODS: The uterus and thoracic aorta were obtained from non-pregnant (estrous cycle) and pregnant Sprague-Dawley rats on day 14 and day 21. The uterine and aortic rings were incubated in organ chambers filled with Krebs-Henseleit solution bubbled with 5% CO2 in air for isometric tension recordings. Cumulative concentration-response relationships to DEA/NO and NTG were obtained in the aortic rings contracted with phenylephrine and in spontaneously contracting uterine rings. RESULTS: The sensitivity and the maximal inhibitory effects of DEA/NO did not differ in aortic tissues of any group. DEA/NO-induced Maximal inhibition of spontaneous contractions of uterine tissues from mid-pregnant rats was greater (although not significantly) than in the tissues from non-pregnant animals (with similar sensitivity), but it was significantly depressed in tissues from late pregnant rats. The sensitivity to and maximal inhibitory effects of NTG were less in aortic tissues from late pregnant versus mid-pregnant and non-pregnant rats. In uterine tissues from late pregnant rats the effect of NTG was negligible. The inhibitory action of both NO donors was much more pronounced in aortic versus uterine tissues. CONCLUSIONS: Uterine smooth muscle is less sensitive than vascular smooth muscle to NO. Uterine smooth muscle from late pregnant animals demonstrates refractoriness to both DEA/NO and NTG, while vascular smooth muscle from late pregnant animals demonstrates refractoriness to NTG, but not to DEA/NO.
Animals↗
[Very-long-chain acyl-CoA dehydrogenase deficiency presenting with recurrent rhabdomyolysis in an adult].
We report a 30-year-old man with very-long-chain acyl-coenzyme A deficiency presenting recurrent rhabdomyolysis. Since the age of 18-year-old, he had noticed recurrent episodes of exercise induced limb muscle pain, limb weakness and dark colored urine. At 29-year-old, he developed the same symptoms, and was referred to our hospital for further examinations under a diagnosis of recurrent rhabdomyolysis. He had no history of trauma, administration of drugs, infections and other factors causing rhabdomyolysis. There were no similar cases in his household. Neurological examinations on admission revealed no abnormal findings. Routine laboratory findings only showed mildly elevated levels of muscle-origin enzymes including CK and aldolase. Ischemic forearm exercise test showed normal levels of lactate and pyruvate in resting state, and normal response after exercise. Organic acids in urine at asymptomatic period were normal. Total carnitine and acyl-carnitine levels in serum were low. Electrospray tandem mass spectrometry in dried blood spots and serum identified elevated level of tetradecenoic acid (C14:1), and palmitoyl-CoA dehydrogenase activity of lymphocytes was deficient. Based on these data, we made a diagnosis of very-long-chain acyl-coenzyme A (VLCAD) deficiency in this patient. Several reports showed that muscular form (adult onset form) of VLCAD deficiency demonstrated recurrent rhabdomyolysis, but true 'adult-onset' case with VLCAD deficiency have been rarely reported. We emphasize that muscular form of VLCAD deficiency should be regarded as one of the causes of recurrent rhabdomyolysis in adult.
Acyl-CoA Dehydrogenase, Long-Chain↗
Screening for basidiomycetous fungi capable of degrading 2,7-dichlorodibenzo-p-dioxin.
We devised a screening method to obtain basidiomycetous fungi capable of degrading dioxins. About 200 fungal strains were selected from more than 1500 strains by their ability to decolorize Remazol brilliant blue R dye as an indicator. To attempt to eliminate the factor of dioxin sorption by mycelia, we prepared two series of living cultures exposed either long term or short term to 2,7-dichlorodibenzo-p-dioxin (2,7-DCDD), and compared the decreases in the levels of this chemical. In only 11 strains was there a significant difference between the two treatments. We chose Panellus stypticus strain 99-334 as a new, effective dioxin degrader, because it gave a close to 100% decrease in 2,7-DCDD levels (from an initial concentration of 10 microM) after 40 days of exposure. The detection of a metabolic intermediate (1-chloro-3,4-dihydroxybenzene) by gas chromatography-mass spectrometry analysis supported the ability of this strain to degrade 2,7-DCDD.
Agaricales↗
Lack of acrosome formation in mice lacking a Golgi protein, GOPC.
The acrosome is a unique organelle that plays an important role at the site of sperm-zona pellucida binding during the fertilization process, and is lost in globozoospermia, an inherited infertility syndrome in humans. Although the acrosome is known to be derived from the Golgi apparatus, molecular mechanisms underlying acrosome formation are largely unknown. Here we show that Golgi-associated PDZ- and coiled-coil motif-containing protein (GOPC), a recently identified Golgi-associated protein, is predominantly localized at the trans-Golgi region in round spermatids, and male mice in which GOPC has been disrupted are infertile with globozoospermia. The primary defect was the fragmentation of acrosomes in early round spermatids, and abnormal vesicles that failed to fuse to developing acrosomes were apparent. In later stages, nuclear malformation and an abnormal arrangement of mitochondria, which are also characteristic features of human globozoospermia, were observed. Interestingly, intracytoplasmic sperm injection (ICSI) of such malformed sperm into oocytes resulted in cleavage into blastocysts only when injected oocytes were activated. Thus, GOPC provides important clues to understanding the mechanisms underlying spermatogenesis, and the GOPC-deficient mouse may be a unique and valuable model for human globozoospermia.
Acrosome↗
Stationary and time-resolved resonance Raman spectra of His77 and Met95 mutants of the isolated heme domain of a direct oxygen sensor from Escherichia coli.
The heme environments of Met(95) and His(77) mutants of the isolated heme-bound PAS domain (Escherichia coli DOS PAS) of a direct oxygen sensing protein from E. coli (E. coli DOS) were investigated with resonance Raman (RR) spectroscopy and compared with the wild type (WT) enzyme. The RR spectra of both the reduced and oxidized WT enzyme were characteristic of six-coordinate low spin heme complexes from pH 4 to 10. The time-resolved RR spectra of the photodissociated CO-WT complex had an iron-His stretching band (nu(Fe-His)) at 214 cm(-1), and the nu(Fe-CO) versus nu(CO) plot of CO-WT E. coli DOS PAS fell on the line of His-coordinated heme proteins. The photodissociated CO-H77A mutant complex did not yield the nu(Fe-His) band but gave a nu(Fe-Im) band in the presence of imidazole. The RR spectrum of the oxidized M95A mutant was that of a six-coordinate low spin complex (i.e. the same as that of the WT enzyme), whereas the reduced mutant appeared to contain a five-coordinate heme complex. Taken together, we suggest that the heme of the reduced WT enzyme is coordinated by His(77) and Met(95), and that Met(95) is displaced by CO and O(2). Presumably, the protein conformational change that occurs upon exchange of an unknown ligand for Met(95) following heme reduction may lead to activation of the phosphodiesterase domain of E. coli DOS.
Carbon Monoxide↗
Puromycin insensitive leucyl-specific aminopeptidase (PILSAP) is involved in the activation of endothelial integrins.
We previously reported that mouse orthologue of puromycin insensitive leucyl-specific aminopeptidase (mPILSAP) played an important role in angiogenesis by regulating the proliferation and migration of endothelial cells (ECs) (Miyashita et al., 2002. Blood 99:3241-3249). Here, we examined the mechanism as to how mPILSAP regulates the migration of ECs. Cell adhesion through integrins plays a crucial role in cell migration, and ECs use at least type-1 collagen receptor integrin alpha2beta1, fibronectin receptor alpha5beta1, and vitronectin receptors alphavbeta3 and alphavbeta5. mPILSAP antisense oligodeoxynucleotide (AS-ODN) or leucinethiol (LT), a leucyl-aminopeptidase inhibitor, did not affect the attachment but did significantly inhibit the spreading of cells of the murine endothelial cell line MSS31 when they were plated on vitronectin-, fibronectin-, or type-1 collagen, although they did not affect the expression of integrin alpha2, alpha5, alphav, beta1, beta3, and beta5 subunits on the cell surface. AS-ODN and LT also inhibited the tyrosine phosphorylation of FAK when cells were plated on vitronectin, fibronectin, or type-1 collagen. This inhibition of cell spreading and of tyrosine phosphorylation of FAK could be negated by Mg(2+). These results suggest that mPILSAP is involved in the activation of endothelial integrins.
Androstadienes↗
Identification of a dysfibrinogenemia of gammaR275C (Fibrinogen Fukushima).
BACKGROUND: Various dysfibrinogenemias have been identified worldwide. This paper describes a case of dysfibrinogenemia recently identified in our laboratory. PATIENT: A 34-year-old pregnant woman without any clinical complaints was admitted to our hospital for delivery. She had an extremely low fibrinogen concentration as determined by the thrombin time method though immunoassay showed a titer within the reference range. Dysfibrinogenemia was suspected and further analyses were performed including on her family. Thrombin time was measured using human and bovine thrombin with and without calcium ion. Reptilase time was also measured. To identify the genetic mutation responsible for this dysfibrinogen, genomic DNA extracted from the blood was analyzed for mutation-rich regions in the fibrinogen gene. RESULTS: The subject, her mother and her two infants showed the same pattern of results while her father showed a regular pattern. Thrombin time calculated using both human and bovine thrombin and reptilase time was elongated in the propositus. The extent of the elongation was decreased in the presence of calcium ion. DNA sequencing showed heterogeneous fibrinogen gammaR275C mutations in the propositus, mother and two children. The father showed no mutation. CONCLUSIONS: A case of dysfibrinogenemia gammaR275C without any clinical symptoms was found by routine coagulation testing and was genetically identified.
Adult↗
Case of congenital infection with Candida glabrata in one infant in a set of twins.
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Candidiasis↗
TT virus infection during childhood.
BACKGROUND: TT virus (TTV) is widespread in the general population, however, the mode of its transmission and the mechanism of maintaining it in the general population are unclear. STUDY DESIGN AND METHODS: To determine the possible mother-to-infant route of transmission, 54 infants bom to 50 anti-HCV-positive mothers were assessed longitudinally. Nucleotide sequences amplified by seminested PCR with primers targeting the N22 variable coding region of genotypes 1 through 6 were compared in mothers and their infants. RESULTS: The prevalence of TTV DNA was 30 percent (15/50; 95% CI, 18-45) in mothers and 44 percent (24/54; 95% CI, 31-59) in their infants. TTV DNA was detected during a follow-up period in 13 (87%; 95% CI, 60-98) of 15 infants born to infected mothers and in 11 (28%; 95% CI, 15-45) of 39 infants bom to DNA-negative mothers. None of 38 cord blood samples, but one of 14 blood samples, obtained at 1 month of age had detectable TTV DNA. The lowest infection rate at the earliest ages and the subsequent increasing prevalence of infection (22% at 6 months and 33% [43% cumulative rate] at 2 years) is consistent with an age-dependent acquisition of TTV by nonparenteral routes. In 13-mother-infant pairs positive for TTV DNA, six showed a high degree of nucleotide sequence similarity (99.1-100%), whereas the remaining seven pairs differed more than 10 percent from each other (46.8-89.2%). The viral load of matemal blood was not a plausible risk factor for transmission. Genotype 1, of which pathogenicity failed to be shown by measurement of hepatic enzymes, was more rapidly cleared (88 vs. 8% other genotypes, p < 0.001) among infants. CONCLUSIONS: These observations strongly suggest that the main factor for TTV acquisition in children involves their age-associated increase in environmental interactions with infectious materials. Genotype 1 might be involved in a weak or a limited pathologic role, which can possibly be diluted by other harmless genotypes.
Adult↗
Diagnosis and management of primary aldosteronism in pregnancy: case report and review of the literature.
Primary aldosteronism rarely complicates pregnancy. We present a woman with primary aldosteronism in pregnancy associated with severe preeclampsia. A 33-year-old Japanese woman with hypertension was referred to our hospital at 25 weeks of gestation. Her blood pressure was 180/100 mmHg, and laboratory tests identified a low serum potassium level and moderate proteinuria on urinalysis. The fetus was diagnosed with growth restriction. Plasma renin activity (PRA) value was 2.2 ng/mL/h and plasma aldosterone concentration (PAC) was elevated (260 pg/mL). The patient was treated medically. At 27 weeks of gestation, we noted persistent late fetal heart rate decelerations associated with uterine contractions. Therefore, elective caesarean section was performed and she was delivered of a 698-g female. After delivery, PRA declined and PAC remained elevated. Abdominal computerized tomography scan and I131-iodochoresterol scan revealed a tumor in the left adrenal gland. Laparoscopic adrenalectomy was performed and confirmed the clinical diagnosis.
Adenoma↗