Biomedical subjects

M Sugiyama

Publications and source records attributed to M Sugiyama.

At least 163 records · Page 9Linked to original sources

PubMed1999

[A case of Fabry's disease with chronic renal failure].

Fabry's disease is a genetic disorder caused by the absence of alpha-galactosidase (alpha-Gal), the gene of which is carried on the long arm of the X chromosome. This enzymatic defect leads to an accumulation of glycosphingolipids in the plasma and lysosomes of endothelial, perithelial, and smooth muscle cells, especially involving those of the cardiovascular, renal and cerebrovascular systems. We report one male case of Fabry's disease with renal deterioration. A 36-year-old man who was a classic case with acroparesthesia, angiokeratoma, and hypohidrosis from 10 years of age, was diagnosed to be a hemizygote of Fabry's disease at 27 years as a result of severe decreased alpha-Gal activity of his peripheral white blood cells. This patient was found to have a point mutation of a G to A transition in exon 1. In May, 1989, he was reported to have proteinuria with normal renal function and admitted to our hospital due to renal deterioration in September, 1993. Laboratory examinations revealed a serum urea nitrogen of 65 mg/dl and creatinine value of 6.9 mg/dl. Urinary protein excretion was 3.9 g/day and urinary sugar was negative. On the renal biopsy specimens, light microscopic examinations revealed multiple sclerosing and collaptic lesions in glomeruli without severe tubulo-interstitial damage, but with stenotic change of the small arteries and arterioles. Electron microscopic examinations revealed a large number of electron dense deposits in the tubules. We diagnosed this case as Fabry's disease with chronic renal failure, however the pathogenesis of this renal progressive deterioration remained obscure. In this case, degenerative changes in the renal vessels due to Fabry's disease may be associated with rapid deterioration in renal function.

Adult↗

PubMed1999

[Clinical study on intracranial air in the severe head injury].

Intracranial air tends to be neglected in severe head injury, because other lesions are more life threatening. We retrospectively studied patients of severe head injury with pneumocephalus. We studied 250 patients' (Glasgow Coma Scale score < or = 8 on admission) computed tomography (CT) scans and found 77 patients with pneumocephalus. These patients' medical records and neuroimagings were studied. The air was most frequently located in the subarachnoid spaces followed by subdural and epidural spaces. Intraparenchymal and intraventricular location were rare. Sphenoid or petrous bone fracture was common. In the cases with petrous or paranasal sinus bone fracture, opacification of these structures were statistically significant. In conclusion, pneumocephalus is common in the severe head injury and most frequently located in the subarachnoid spaces, however, the precise location of air is unclear in many cases. Bone CT scan is useful and opacification of air containing structures suggests the possibility of skull base fractures.

Adolescent↗

PubMed1999

Pancreatic disorders associated with anomalous pancreaticobiliary junction.

BACKGROUND: The aim of this study was to establish an optimal management strategy for pancreatic disorders associated with anomalous pancreaticobiliary junction (APBJ). METHODS: In 64 adult surgical cases of APBJ (common channel 15 mm or longer) (43 with and 21 without choledochal cyst), associated pancreatic disorders, pancreatographic results, and treatment outcomes were analyzed. RESULTS: Of 64 patients, 56 had pancreatobiliary symptoms. Twenty-four patients (38%) had pancreatic disorders: acute pancreatitis (n = 20), chronic calcifying pancreatitis (n = 2), and pancreatic carcinoma (n = 2). Twenty patients (31%) had abnormal pancreatograms. The incidence of acute pancreatitis was significantly higher in patients with an abnormal pancreatogram, particularly dilatation, protein plugs or stones of the common channel or main pancreatic duct, and coexisting pancreatic ductal anomaly. All patients with choledochal cyst underwent cyst excision and hepaticojejunostomy. Eleven patients without choledochal cyst or pancreatobiliary carcinoma underwent cholecystectomy alone. Protein plugs and pancreatic stones were extracted through the bile duct stump or by sphincterotomy. No patients experienced pancreatitis during a mean postoperative follow-up of 6.7 years. CONCLUSIONS: In managing APBJ, attention should be paid to the possibility of associated pancreatic disorders and an abnormal pancreatogram. APBJ with choledochal cyst requires cyst excision. Cholecystectomy alone may be adequate for APBJ without cyst.

Adolescent↗

PubMed1998

New electrocardiographic criteria for predicting the site of coronary artery occlusion in inferior wall acute myocardial infarction.

In patients with inferior wall acute myocardial infarction (AMI), the site of the culprit lesion is an important determinant of outcome. Patients with right ventricular infarction have a poor prognosis, whereas those with occlusion of the left circumflex coronary artery (LCx) have a good prognosis. Therefore, we assessed whether standard 12-lead electrocardiograms obtained on admission could identify the site of coronary artery occlusion, (i.e., a site proximal to the origin of the right ventricular branch of the right coronary artery [RCA], a site distal to the origin of the right ventricular branch of the RCA, or a site in the LCx). The ratio of ST depression in lead V3 to ST elevation in lead III (V3/III ratio) was evaluated immediately before coronary angiography in 152 patients with a first inferior wall AMI confirmed by coronary angiography within 12 hours after the onset of symptoms. For occlusion of the proximal RCA, distal RCA, and LCx, V3/III ratio was 0.2+/-0.3, 0.8+/-0.5, and 2.5+/-2.5 (p = 0.0001), respectively. The V3/III ratio <0.5 identified proximal RCA occlusion, 0.5 <V3/III ratio < or = 1.2 identified distal RCA occlusion, and 1.2 <V3/III ratio identified LCx occlusion with sensitivities of 91%, 84%, and 84%, and specificities of 91%, 93%, and 95%, respectively. We conclude that the V3/III ratio is useful in predicting the site of coronary artery occlusion in patients with inferior wall AMI.

Adult↗

PubMed1998

Site-directed mutagenesis of the A-factor receptor protein: Val-41 important for DNA-binding and Trp-119 important for ligand-binding.

The A-factor receptor protein (ArpA) plays a key role in the regulation of secondary metabolism and cellular differentiation in Streptomyces griseus. ArpA binds the target DNA site forming a 22 bp palindrome in the absence of A-factor, and exogenous addition of A-factor to the ArpA-DNA complex immediately releases ArpA from the DNA. An amino acid (aa) replacement at Val-41 to Ala in an alpha-helix-turn-alpha-helix (HTH) motif at the N-terminal portion of ArpA abolished DNA-binding activity but not A-factor-binding activity, suggesting the involvement of this HTH in DNA-binding. On the other hand, an aa replacement at Trp-119 to Ala generated a mutant ArpA that was unable to bind A-factor, thus resulting in an A-factor-insensitive mutant that bound normally to its target DNA in both the presence and absence of A-factor. These data suggest that ArpA consisting of two functional domains, one for HTH-type DNA-binding at the N-terminal portion and one for A-factor-binding at the C-terminal portion, is a member of the LacI family. Consistent with this, two ArpA homologues, CprA and CprB, from Streptomyces coelicolor A3(2), each of which contains a very similar aa sequence of the HTH to that of ArpA, also recognized and bound the same DNA target. However, neither CprA nor CprB recognized A-factor, probably due to much less similarity in the C-terminal domains.

4-Butyrolactone↗

PubMed1998

Alliinase [S-alk(en)yl-L-cysteine sulfoxide lyase] from Allium tuberosum (Chinese chive)--purification, localization, cDNA cloning and heterologous functional expression.

Alliinase [S-alk(en)yl-L-cysteine sulfoxide lyase], a pyridoxal-phosphate-(Pxy-P)-dependent enzyme, is responsible for the degradative conversion of S-alk(en)yl-L-cysteine sulfoxide to volatile odorous sulfur-containing metabolites in Allium plants. We have purified alliinase from shoots of Allium tuberosum (Chinese chive) to apparent homogeneity by SDS/polyacrylamide gel electrophoresis. A cDNA clone encoding alliinase was isolated from a cDNA library constructed from whole plants of A. tuberosum by hybridization screening with a synthetic 50-residue oligonucleotide encoding a conserved region of the alliinases from onion and garlic. The isolated cDNA encoded a protein of 476 amino acid residues with a molecular mass of 54083 Da. The deduced amino acid sequence exhibited 66-69% identities with those of reported alliinases from onion, garlic and shallot. The partial amino acid sequence, which was determined for a V8 protease-digested peptide fragment of the purified alliinase, was perfectly matched with the sequence deduced from the cDNA. An expression vector of recombinant alliinase cDNA was constructed in yeast. The catalytically active protein was in the soluble fraction of transformed yeast. Site-directed mutagenesis experiments indicated that Lys280 was essential for the catalytic activity and, thus, a possible Pxy-P-binding residue. The mRNA expression of the alliinase gene comprising a multigene family in the shoots of green plants was twofold higher than that in the roots of green plants; however, the expression in the shoots of etiolated plants was only 13% that in green shoots, although the expression in the roots was not remarkably different between in green and etiolated plants. Immunohistochemical investigation indicated that the alliinase protein is predominantly accumulated in the bundle sheath cells of shoots of A. tuberosum.

Allium↗

PubMed1998

BEN1 and ZEN1 cDNAs encoding S1-type DNases that are associated with programmed cell death in plants.

We describe the isolation and characterization of cDNAs encoding two DNases that may be involved in the programmed death of plant cells: a 35-kDa nuclease of barley, which had been reported to be secreted from the aleurone layer into the endosperm during germination and may be responsible for the digestion of nuclear DNA in the course of degradation (cell death) of endosperm, and a 43-kDa nuclease of zinnia, which appears transiently in association with differentiation to tracheary elements and is likely to participate in the autolysis at the final step of the differentiation. Genes for these nucleases of barley and zinnia were designated BEN1 and ZEN1, respectively. The amino acid sequence of BEN1 protein deduced from the nucleotide sequence of BEN1 cDNA consisted of 288 residues with a putative signal sequence of 23 residues. RNA gel blot analysis revealed that BEN1 mRNA increased in the embryo-less half seeds of barley in response to the application of gibberellic acid. The deduced amino acid sequence of ZEN1 protein consisted of 303 residues with a putative signal sequence of 25 residues. Temporal accumulation of ZEN1 mRNA was detected during transdifferentiation of zinnia mesophyll cells into tracheary elements. Significant similarities were found among the amino acid sequences of BEN1, ZEN1, nuclease S1 from Aspergillus oryzae, and two other S1-type nucleases.

Amino Acid Sequence↗

PubMed1998

An ultrahigh-vacuum goniometer for in situ soft X-ray standing-wave analysis of semiconductor surfaces.

An ultrahigh-vacuum goniometer was developed for in situ X-ray standing-wave (XSW) analysis of semiconductor surfaces prepared by molecular-beam epitaxy (MBE). Although two ultrahigh-vacuum motors for chi and phi rotating axes are inside the analysis chamber, low-energy photoelectrons can still be collected as the magnetic field is sufficiently suppressed by using metal shields. Furthermore, the sample can be annealed at temperatures higher than 870 K on the goniometer in the analysis chamber. This goniometer is used at beamline 1A (BL-1A) at the Photon Factory, where both monochromated soft X-rays and UV radiation are available. This analysis system was shown to be suitable not only for in situ soft-XSW and X-ray absorption near-edge structure (XANES) studies but also for synchrotron radiation photoelectron spectroscopy (SRPES) studies. The annealing effects on an S-adsorbed GaAs(001) surface could be studied by SRPES, XANES and XSW using this new goniometer.

Journal Article↗

PubMed1998

Recent performance of the Photon Factory gratingcrystal monochromator station BL-1A in the soft X-ray region.

This paper describes the recent performance of the Photon Factory beamline 1A with InSb(111) crystals used as the diffracting elements of the grating/crystal monochromator for monochromatizing soft X-rays. Pt-coated collimating and focusing mirrors located upstream and downstream of the monochromator have recently been replaced with Ni-coated mirrors in order to remove absorption structures at the Pt M-edges from output spectra in the soft X-ray region. Output spectra without the absorption structures and with higher intensity in the range 2000-3400 eV were obtained by using the Ni-coated collimating and focusing mirrors.

Journal Article↗

PubMed1998

Synthesis and 5 alpha-reductase inhibitory activities of benzofuran derivatives with a carbamoyl group.

A series of 2-phenylbenzofuran derivatives with a diphenylmethylcarbamoyl group at the 5 or 6 position of the benzofuran ring were synthesized and evaluated for rat and human testosterone 5 alpha-reductase inhibitory activities in vitro. They had inhibitory activities against both enzymes and the 6-carbamoyl derivatives tended to be more potent than the 5-carbamoyl compounds.

5-alpha Reductase Inhibitors↗

PubMed1998

Cloning and characterization of a gene (UVR3) required for photorepair of 6-4 photoproducts in Arabidopsis thaliana.

UV radiation induces two major classes of pyrimidine dimers: the pyrimidine [6-4] pyrimidone photoproduct (6-4 product) and the cyclobutane pyrimidine dimer (CPD). Many organisms produce enzymes, termed photolyases, that specifically bind to these damage products and split them via a UV-A/blue light-dependent mechanism, thereby reversing the damage. These photolyases are specific for either CPDs or 6-4 products. A gene that expresses a protein with 6-4 photolyase activity in vitro was recently cloned from Drosophila melanogaster and Xenopus laevis. We report here the isolation of a homolog of this gene, cloned on the basis of sequence similarity, from the higher plant Arabidopsis thaliana. This cloned gene produces a protein with 6-4 photolyase activity when expressed in Escherichia coli. We also find that a previously described mutant of Arabidopsis (uvr3) that is defective in photoreactivation of 6-4 products carries a nonsense mutation in this 6-4 photolyase homolog. We have therefore termed this gene UVR3. Although homologs of this gene have previously been shown to produce a functional 6-4 photolyase when expressed in heterologous systems, this is the first demonstration of a requirement for this gene for photoreactivation of 6-4 products in vivo.

Amino Acid Sequence↗

PubMed1998

Crystallization and preliminary X-ray diffraction studies of bleomycin-binding protein from bleomycin-producing Streptomyces verticillus.

A bleomycin-binding protein (BLMA) produced by bleomycin-producing Streptomyces verticullus was crystallized in a form suitable for X-ray diffraction analysis using the vapor-diffusion method. Crystals were grown at pH 5.7, in 0.2 M NH4 actate and 0.1 M Na acetate, using 30% PEG 4000 as a precipitant. They belong to the orthorhombic system, with space group P21212, cell dimensions a = 54. 90, b = 67.94, c = 35.60 A, and one BLMA molecule in the asymmetric unit. The crystals diffract X-rays well and the diffraction intensity data was collected up to 1.5 A resolution with a merging R value of 0.054 at beamline 6B of the Photon Factory. The diffraction data set is 94% complete.

Bacterial Proteins↗

PubMed1998

99mTc-tetrofosmin uptake in bone metastases from breast cancer.

99mTc-tetrofosmin myocardial imaging was performed in a 62-year-old woman who had undergone standard radical mastectomy for right breast cancer 6 years ago. Although the result was negative for the ischemic heart disease, it showed abnormal accumulation corresponding to the bone metastases in the spine. We believe that 99mTc-tetrofosmin imaging is helpful in detecting bone metastases from breast cancer.

Bone Neoplasms↗

PubMed1998

Is percutaneous cholecystostomy the optimal treatment for acute cholecystitis in the very elderly?

In elderly patients emergent cholecystectomy for acute cholecystitis is a high risk procedure. We prospectively assessed the value of percutaneous cholecystostomy for acute cholecystitis in 38 consecutive elderly (> or = 80 years) patients. All 38 underwent percutaneous transhepatic cholecystostomy under ultrasonographic and fluoroscopic guidance for acute cholecystitis (25 calculous, 13 acalculous). Eight (21%) patients had acute severe medical problems, such as shock and respiratory distress. Thirty-one (82%) patients had chronic severe underlying diseases, including cardiovascular and neurologic diseases. Cholecystostomy was successful in all 38 patients. Prompt clinical improvement was obtained in 36 (95%) patients. Morbidity and mortality rates were 3% and 3%, respectively. After cholecystostomy, 10 patients with cholelithiasis underwent elective cholecystectomy without serious complications. Two patients underwent percutaneous cholecystolithotomy, which produced complete resolution of symptoms. Four of 12 patients with and none of 12 without cholelithiasis had recurrent cholecystitis after catheter removal during a mean follow-up of 1.8 years. A second cholecystostomy was successful in these four patients. Elderly patients are often poor surgical candidates because of severe cholecystitis or concomitant medical problems. Percutaneous cholecystostomy is a safe, effective treatment for acute cholecystitis even in elderly patients. For calculous cholecystitis, cholecystostomy can be followed by elective surgery, if possible, or by nonsurgical treatment or expectant conservative management in high-risk patients. Cholecystostomy may be a definitive treatment for acalculous cholecystitis.

Acute Disease↗

PubMed1998

Expression of activin A is increased in cirrhotic and fibrotic rat livers.

BACKGROUND & AIMS: Activin A, a member of the transforming growth factor (TGF)-beta superfamily, recently has been reported to suppress DNA synthesis and to induce apoptosis of hepatocytes. These biological functions are similar to those of TGF-beta1, which is overexpressed in liver cirrhosis. The aim of this study was to examine whether activin A is involved in liver cirrhosis and fibrosis. METHODS: Liver cirrhosis or fibrosis was induced by intraperitoneal injections of dimethylnitrosamine or porcine serum into rats. The kinetics of activin A messenger RNA (mRNA) expression in cirrhotic and fibrotic livers and primary cultured rat hepatocytes were assessed by Northern blotting, and the localization of activin A was determined immunohistochemically. Modulation of type 1 collagen mRNA expression by activin A in rat cultured Ito/fat-storing cells and fibroblasts was also examined. RESULTS: Northern blotting showed that activin A mRNA expression was enhanced in fibrotic livers. The numbers of hepatocytes expressing immunoreactive activin A were significantly greater, especially around the fibrotic areas. Activin A mRNA expression in cultured hepatocytes was increased significantly by TGF-beta1 and by activin A itself. Furthermore, type 1 collagen mRNA expression in cultured cells was enhanced by activin A and TGF-beta1 in a synergistic manner. CONCLUSIONS: Activin A is overexpressed in rat cirrhotic and fibrotic livers and may contribute to hepatic fibrogenesis.

Activins↗

PubMed1998

Intraductal papillary tumors of the pancreas: evaluation with endoscopic ultrasonography.

BACKGROUND: We retrospectively analyzed the endoscopic ultrasonographic characteristics of intraductal papillary tumors of the pancreas and identified signs indicative of malignant tumors. METHODS: Twenty-eight patients with intraductal tumors and 38 with other pancreatic cystic lesions underwent endoscopic ultrasonography, conventional ultrasonography, and endoscopic retrograde cholangiopancreatography (ERCP). RESULTS: Intraductal tumors were classified into three subtypes, and endoscopic ultrasonographic characteristics were assessed. In main duct type tumors, the main pancreatic duct showed a segmental or diffuse moderate-to-marked dilatation, often associated with intraductal nodules. Pancreatic parenchymal atrophy was frequently recognized. Branch duct type tumors consisted of multiple 5 to 20 mm cysts with the appearance of a cluster of grapes. The main duct was mildly dilated or nondilated. Combined type tumors had features of both main duct and branch duct types. Endoscopic ultrasonography (92%) differentiated pancreatic cystic lesions more precisely than ultrasonography (82%) and ERCP (89%). Main duct or combined type tumors, large tumors (> or = 30 mm for branch duct type), marked dilatation of the main pancreatic duct (> or = 10 mm for other types), and mural nodules suggested malignancy. Endoscopic ultrasonography demonstrated these signs more accurately than ultrasonography or ERCP. CONCLUSION: Endoscopic ultrasonography is useful for diagnosing intraductal papillary tumors, particularly for predicting malignancy.

Aged↗

PubMed1998

The combined method: a novel access technique for fetal endoscopic surgery.

BACKGROUND/PURPOSE: To develop practical and less invasive techniques for fetal endoscopic surgery, new methods of lifting the uterine wall to allow fetal surgery without maternal laparotomy were developed and assessed. METHODS: Fetal endoscopic surgical procedures, including tracheostomy and umbilical vascular cannulation, were performed using one of the three methods to enter the uterus without maternal laparotomy in pregnant goats (n = 6; 105 to 115 days' gestation): (1) direct uterine lifting with an air-cushion device; (2) indirect uterine lifting, in which the uterine wall was fixed to the maternal abdominal wall using balloon tip ports inserted percutaneously by Seldinger's method, then the maternal abdomen was lifted mechanically; and (3) combined method, in which low pressure CO2 (5 mm Hg for initial inflation and 2 mm Hg for maintenance) was insufflated into the uterus in addition to the indirect uterine lifting cited above. RESULTS: The direct uterine lifting caused massive injury of myometrium and uterine membranes. The creation of intrauterine space and the protection of the membranes were not accomplished effectively by the indirect uterine lifting only. The combined method provided the adequate intrauterine space and excellent endoscopic visibility for completion of the endoscopic procedures with minimal uterine injury. CONCLUSION: The fetal endoscopic surgery may be accomplished simply and safely by the combined method, a novel technique of uterine lifting to allow fetal surgery without maternal laparotomy.

Animals↗

PubMed1998

Anomalous pancreaticobiliary junction without congenital choledochal cyst.

BACKGROUND: Anomalous pancreaticobiliary junction (APBJ) without congenital choledochal cyst (CCC) carries a high risk of gallbladder carcinoma development. The aim of this study was to obtain information allowing early diagnosis and appropriate management. METHODS: The clinical features, imaging findings and surgical outcome of 18 patients with APBJ without CCC were analysed retrospectively. RESULTS: Fourteen patients had symptoms, including those of acute pancreatitis (five patients). In 16 patients the gallbladder showed abnormalities, including carcinoma (eight) and mucosal hyperplasia (11). Ultrasonography detected gallbladder carcinoma with 100 per cent sensitivity and mucosal hyperplasia with 91 per cent sensitivity. A long common channel was demonstrated by endoscopic retrograde cholangiopancreatography (ERCP) in all patients, endoscopic ultrasonography in nine of ten, and magnetic resonance cholangiopancreatography (MRCP) in five of five. Five of eight patients with gallbladder carcinoma underwent extended cholecystectomy with bile duct excision. Three patients with cancer and eight with no cancer had cholecystectomy alone. None developed bile duct carcinoma or acute pancreatitis after operation. All patients without malignancy remained asymptomatic for a mean follow-up period of 4.7 years. CONCLUSION: Prophylactic cholecystectomy is recommended for patients with APBJ without CCC. For early diagnosis of APBJ, gallbladder abnormalities on ultrasonography or acute pancreatitis of unknown aetiology should prompt further investigation with ERCP or less invasive imaging modalities such as endoscopic ultrasonography and MRCP.

Adult↗