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Biomedical subjects

S Fukuda

Publications and source records attributed to S Fukuda.

At least 289 records · Page 16Linked to original sources
PubMed1998

Amino acid and nucleotide sequences of human peroxisomal enoyl-CoA hydratase: 3-hydroxyacyl-CoA dehydrogenase cDNA.

Deficiency of enoyl-CoA hydratase: 3-hydroxyacyl-CoA dehydrogenase (peroxisomal bifunctional enzyme), one of the enzymes of the peroxisomal beta-oxidation system, leads to clinical manifestations resembling Zellweger syndrome with hypotonia, psychomotor delay, hepatomegaly, typical facial appearance and accumulation of very long-chain fatty acids. The nucleotide sequence of the human peroxisomal enoyl-CoA hydratase: 3-hydroxyacyl-CoA dehydrogenase cDNA has been reported by Hoefler and colleagues; however, we have found some amino acid differences from our originally isolated cDNA. Contrary to the findings described in a previous paper, we report here the cDNA sequence of human peroxisomal enoyl-CoA hydratase: 3-hydroxyacyl-CoA dehydrogenase in which there are 9 authenticated amino acid alterations.

3-Hydroxyacyl CoA Dehydrogenases↗
PubMed1998

Mutation analysis in the iduronate-2-sulphatase gene in 43 Japanese patients with mucopolysaccharidosis type II (Hunter disease).

Our series of studies on Hunter disease in Japanese patients showed allelic heterogeneity of IDS gene mutations, genotype/phenotype correlation and racial differences in distribution of mutations. Twenty-five different small mutations have been characterized. Small mutations in the Japanese population are widely distributed through the IDS gene, although some mutations were unevenly concentrated on exon 5 (28%) and on exon 9 (24%). Mutations were seen at the same codon 468 in exon 9 in 5 patients. These findings are in good agreement with data on other ethnic groups. Two unique mutations linked to a severe phenotype were apparently associated with aberrant splicings; one was a point mutation within exon 3 (P86L), partially activating a cryptic splice acceptor site at 28 bp downstream from the mutation site within exon 3 and producing a 44-base truncated mRNA, and the other was a point mutation at the consensus sequence of the splice donor site of intron 2, causing exon 2 skipping.

Base Sequence↗
PubMed1998

Lipid hydroperoxide stimulates retinal neovascularization in rabbit retina through expression of tumor necrosis factor-alpha, vascular endothelial growth factor and platelet-derived growth factor.

To test the hypothesis that oxidative damage associated with tissue hypoxia plays a role in neovascularization, a lipid hydroperoxide (LHP) was injected into the vitreous of rabbits. Single injections of LHP (50-600 microg) caused a sustained retinal neovascularization visualized clinically by ophthalmoscopy and confirmed by microscopy. Vasodilators, i.e. histamine and nitric oxide, peaked at 6h and 7 days, respectively. The levels of both tumor necrosis factor-alpha and interleukin-1alpha peaked at 12h and dropped to basal levels by 24h. Expression of vascular endothelial growth factor (VEGF) and transforming growth factor-beta peaked at 24h and were sustained throughout the following 3 weeks, and platelet-derived growth factor was also elevated throughout the same period. Upregulation of these five angiogenic cytokines, but not basic fibroblast growth factor, occurred prior to the appearance of neovascularization. Leakage of fluorescein at the tips of new vessels was demonstrated by fluorescein angiography. Linoleic hydroperoxide induced neovascularization, but saturated or unsaturated native C-18 fatty acids had no effect. The cascade of multiple, angiogenic cytokines induced by LHP may interact to promote sustained neovascularization.

Journal Article↗
PubMed1998

Treatment of MPS VII (Sly disease) by allogeneic BMT in a female with homozygous A619V mutation.

A 12-year-old girl with Sly disease (mucopolysaccharidosis VII; beta-glucuronidase deficiency), who is homozygous for the A619V mutation, had a successful allogeneic BMT, donored by an HLA-identical unrelated female to replace the deficient enzyme. Within 5 months after BMT, the enzyme activity of the recipient's lymphocytes increased to normal range. No signs of acute or chronic GVHD were observed. For the successive 31 months post-BMT, beta-glucuronidase activity in her lymphocytes was maintained at almost normal levels and excretion of glycosaminoglycans in the urine was greatly diminished. Ultrastructural findings demonstrated no abnormal vacuoles and inclusion bodies in the cytoplasm of her rectal mucosal cells. Coincident with the restoration of the enzyme activity, clinical improvement was dramatic. Especially notable were improvements in motor function. The patient was able to walk alone for a long time without aid, and she even became able to ride a bicycle and take a bath. In addition, recurrent infections of the upper respiratory tract and the middle ears decreased in frequency and severity, and dyspnea on exertion, severe snoring and vertigo have substantially improved. Thus, allogeneic BMT in this patient produced a better quality of life and provided a more promising outlook.

Bone Marrow Transplantation↗
PubMed1998

A splicing mutation of the RHAG gene associated with the Rhnull phenotype.

Rhnull is a syndrome serologically characterized by the deficiency of all Rh antigens on human red blood cells. Rhnull is divided into two types: regulator and amorph. Recently, Cherif-Zahar et al. proposed that the RHAG gene encoding the Rh50 glycoprotein is a candidate for inducing regulator type Rhnull. We investigated both the RH and RHAG genes in an Rhnull individual. The reticulocytes from the propositus had RHD, RHcE, and RHCe transcripts without any mutation. However, the sequence analysis of RHAG cDNA showed a deletion of 122 bp from nucleotide 946 to 1067. This deletion was revealed to be due to a homozygous splicing mutation, which is a single base substitution at the consensus sequence of the splicing acceptor site (AG-->AT). The mutation appeared to break the 'GT-AG' splicing rule and to cause 122 bp exon skipping accompanied by a frameshift. This study confirms that the RHAG gene is the most likely candidate for the 'regulator' gene of Rhnull cases.

Adolescent↗
PubMed1998

Postischemic reperfusion induces alpha-fodrin proteolysis by m-calpain in the synaptosome and nucleus in rat brain.

A membrane cytoskeletal protein, fodrin, is a substrate for a Ca2+-dependent protease, calpain. It remains unknown whether mu-calpain or m-calpain is involved in the proteolysis of either alpha- or beta-fodrin and in what subcellular localization during ischemia and reperfusion of the brain. To address these issues, we examined the distribution of fodrin and calpain and the activities of calpain and calpastatin (endogenous calpain inhibitor) in the same subcellular fractions. Rat forebrain was subjected to ischemia by a combination of occlusion of both carotid arteries and systemic hypotension, whereas reperfusion was induced by releasing the occlusion. Immunoblotting, activity measurement, and casein zymography did not detect the presence of mu-calpain or a significant change of m-calpain level after ischemia or reperfusion. However, casein zymography revealed a unique Ca2+-dependent protease that was eluted with both 0.18 and 0.40 M NaCl from a DEAE-cellulose column. Alpha- and beta-fodrins and m-calpain were found to be rich in the synaptosomal, nuclear, and cytosolic subfractions by immunoblotting analysis. Reperfusion (60 min) following ischemia (30 min) induced selective proteolysis of alpha-fodrin, which was inhibited by a calpain inhibitor, acetylleucylleucylnorleucinal (400 microM, 1 ml, i.v.). The mu-calpain-specific fragment of beta-fodrin was not generated during ischemia-reperfusion, supporting the possibility of the involvement of m-calpain rather than mu-calpain in the alpha-fodrin proteolysis.

Animals↗
PubMed1998

The incidence of colorectal cancer during pregnancy in Japan: report of two cases and review of Japanese cases.

Colorectal cancer during pregnancy is very rare. We report two additional cases of rectal carcinoma. A 34-year-old woman with an obstructive adenocarcinoma of the rectal region was diagnosed at labor. A 35-year-old woman with an adenocarcinoma of the rectal region was diagnosed at 32 weeks of gestation and underwent a cesarean section and rectal resection at 35 weeks of gestation. A retrospective review of the Japanese literature was performed to identify patients who appeared to have primary colorectal cancer during pregnancy. Thirty-six patients with colon cancer (75.0%), 10 (20.8%) with rectal cancer, and two (4.2%) of unknown sites have been reported in Japanese series. The average age of the mother was 32.2 years. The calculated incidence of colorectal cancer among Japanese pregnant women was one case per 502,316 live births during the years between 1986 through 1995. Although the majority of colorectal cancers diagnosed during pregnancy are rectal carcinomas, the patients in Japan were predominantly complicated by colon cancer. The fetal risk seems small, because there were no cases of colorectal cancer metastatic to the products of conception.

Adenocarcinoma↗
PubMed1998

Use of intraperitoneal vessels in reconstructive microsurgery: an account of 117 cases.

The purpose of this study was to evaluate our experience of microvascular anastomosis of intraperitoneal vessels. Between 1985 and 1994, 117 microsurgical reconstructions were done using intraperitoneal vessels. These included oesophageal reconstruction (n = 106), reconstruction of the hepatic arterial, superior mesenteric arterial, or portal venous system (n = 8), and reconstruction of the chest or abdominal wall (n = 3). We used 129 intraperitoneal arteries and 117 intraperitoneal veins. Of a total of 246 intraperitoneal vessels, five hepatic arteries, seven splenic, 14 gastroepiploic, six superior mesenteric, 178 jejunal, 30 ileocolic, four middle colic arteries or veins, and two portal veins were used for microvascular anastomosis. The overall successful rate for these reconstructive cases was 97% (114/117). Microsurgical use of intraperitoneal vessels is a safe and developing procedure in plastic and reconstructive surgery.

Adult↗
PubMed1998

Topoisomerase I and II consensus sequences in a 17-kb deletion junction of the COL4A5 and COL4A6 genes and immunohistochemical analysis of esophageal leiomyomatosis associated with Alport syndrome.

Diffuse esophageal leiomyomatosis (DL), a benign smooth-muscle-cell tumor, is characterized by abnormal cell proliferation. DL is sometimes associated with X-linked Alport syndrome (AS), an inherited nephropathy caused by COL4A5 gene mutations. COL4A5 is tightly linked, in a head-to-head fashion, to the functionally related and coordinately regulated COL4A6 gene. No X-linked AS cases are due to COL4A6 mutations, but all DL/AS cases are always associated with deletions spanning the 5' regions of the COL4A5/COL4A6 cluster. Unlike the COL4A5 breakpoints, those of COL4A6 are clustered within intron 2 of the gene. We identified a DL/AS deletion and the first characterization of the breakpoint sequences. We show that a deletion eliminates the first coding exon of COL4A5 and the first two coding exons of COL4A6. The breakpoints share the same sequence, which, in turn, is closely homologous to the consensus sequences of topoisomerases I and II. Additional DNA evidence suggested that the male patient is a somatic mosaic for the mutation. Immunohistochemical analysis using alpha-chain-specific monoclonal antibodies supported this conclusion, since it revealed the absence of the alpha5(IV) and alpha6(IV) collagen chains in most but not all of the basement membranes of the smooth-muscle-cell tumor. We also documented a similar segmental staining pattern in the glomerular basement membranes of the patient's kidney. This study is particularly relevant to the understanding of DL pathogenesis and its etiology.

Adult↗
PubMed1998

Differential inhibitory effects of thiopental, thiamylal and phenobarbital on both voltage-gated calcium channels and NMDA receptors in rat hippocampal slices.

Although it is known that there are some pharmacological differences between the structurally similar barbiturates, the underlying mechanism of action remains unclear. We have compared the effects of thiopental, thiamylal and phenobarbital on both voltage-gated calcium channels (VGCC) and N-methyl-D-aspartate (NMDA) receptors in rat hippocampal slices by determining changes in intracellular calcium ([Ca2+]i). Experiments were performed in adult rat hippocampal slices perfused with Krebs solution (37 degrees C). Concentrations of [Ca2+]i in the pyramidal cell layer of the CA1 region were measured using a calcium indicator dye, fura-2. To activate VGCC and NMDA receptors, slices were exposed to K+ 60 mmol litre-1 (< or = 60 s) and NMDA 100 mumol litre-1 (30 s), respectively. Thiopental, thiamylal and phenobarbital were present 5 min before, during and 1 min after high K+ or NMDA application. Both thiamylal and thiopental (50-600 mumol litre-1) attenuated the increases in [Ca2+]i produced by high K+ or NMDA in a concentration-dependent manner, while phenobarbital 50-1000 mumol litre-1 only slightly attenuated the [Ca2+]i increase produced by high K+ at concentrations of more than 200 mumol litre-1 and was ineffective on the [Ca2+]i response produced by NMDA. Although the increases in [Ca2+]i caused by membrane depolarization with high K+ were reduced equally with thiamylal and thiopental, thiamylal was more effective in attenuating the increase in [Ca2+]i produced by NMDA receptor activation than thiopental. We conclude that the depressant effects of barbiturates on both VGCC and NMDA receptors varied between agents. Differential inhibition of both VGCC and NMDA receptors may determine the pharmacological properties of barbiturates and their ability to protect neurones against ischaemia.

Anesthetics, Intravenous↗
PubMed1998

Thiopental inhibits increases in [Ca2+]i induced by membrane depolarization, NMDA receptor activation, and ischemia in rat hippocampal and cortical slices.

BACKGROUND: This study examined the effects of thiopental on intracellular calcium ([Ca2+]i) changes induced by membrane depolarization, N-methyl-D-aspartate (NMDA) receptor activation, and ischemia. METHODS: Experiments were performed in brain slices prepared from Wistar rats. [Ca2+]i measurements were taken on the CA1 pyramidal cell layer of the hippocampus or layers II to III of the somatosensory cortex using the fura-2 fluorescence technique. Membrane depolarization and NMDA receptor activation were induced by exposing slices to 60 mM K+ and 100 microM NMDA, respectively. In vitro ischemia was induced by superfusing slices with glucose-free Krebs solution equilibrated with 95% nitrogen and 5% carbon dioxide. Thiopental was applied 5 min before application of high K+ and NMDA, or before in vitro ischemia. RESULTS: Ischemia for 15 min produced a characteristic [Ca2+]i increase in both hippocampal and cortical slices. Thiopental prolonged the latency to the appearance of the [Ca2+]i plateau and reduced the magnitudes of increase in [Ca2+]i 8, 10, and 15 min after the onset of ischemia. Thiopental also suppressed the high K+- and NMDA-induced [Ca2+]i increases. The NMDA-induced [Ca2+]i increases were attenuated to a greater extent in cortical slices than were those in hippocampal slices. The inhibition of thiopental on the 200-microM NMDA-mediated [Ca2+]i response was confirmed in cultured cortical neurons. CONCLUSIONS: The results indicate that thiopental attenuates ischemia-induced [Ca2+]i increases in the hippocampus and cortex in vitro, probably because of its inhibition of both voltage-gated calcium channels and NMDA receptors. The regionally different inhibition of thiopental on NMDA receptors may relate to its region-specific action against ischemia.

Anesthetics, Intravenous↗
PubMed1998

Healing of cimetidine-resistant Ménétrier's disease by eradication of Helicobacter pylori infection.

We report a case of Ménétrier's disease associated with hypoproteinemia who was successfully treated by eradication of Helicobacter pylori infection. A 26-year-old woman had diffuse giant gastric folds with proven protein leakage from the stomach. She received cimetidine, famotidine, and omeprazole for more than 3 months with little benefit. H. pylori infection was detected, and she was treated with clarithromycin (200 mg three times a day), lansoprazole (30 mg/day), and ecabet sodium (1.5 g twice a day) for 2 weeks. After starting the treatment, rapid increase of serum albumin and IgG levels were observed. The treatment resulted in eradication of H. pylori infection, and serum albumin and IgG levels returned to normal. Histologic specimens also showed the improvement of the irregularly elongated gastric pits with inflammatory infiltrates after the treatment. This case report suggests that eradication of H. pylori is recommended therapy for the immediate improvement of Ménétrier's disease associated with hypoproteinemia.

2-Pyridinylmethylsulfinylbenzimidazoles↗
PubMed1998

Improvement with disodium cromoglycate of neutrophil phagocytosis and respiratory burst activity in a patient with hyperimmunoglobulin E syndrome.

We report a case of hyperimmunoglobulin E syndrome (HIE) complicated by neutrophil deficiency which was successfully treated with oral administration of disodium cromoglycate. A 48-year-old Japanese man with HIE developed Streptococcus pneumoniae meningitis. Laboratory tests after the meningitis revealed persistent neutropenia (300-800/mm3) and defects of phagocytosis and bacterial killing by neutrophils. Administration of disodium cromoglycate was started, and neutrophil counts gradually increased to 1200-1600/mm3. The impaired neutrophil activities returned to normal. The patient improved clinically; during the 2-year treatment, he had only two brief episodes of the common cold. Disodium cromoglycate may have potential clinical use in the treatment of cases of HIE even with neutrophil deficiency.

Cromolyn Sodium↗
PubMed1998

Brother/sister siblings affected with Hunter disease: evidence for skewed X chromosome inactivation.

Hunter disease is an X-linked recessive disorder caused by a deficiency of iduronate-2-sulfatase activity. We describe a pair of brother/sister siblings with a typical feature of Hunter disease (mucopolysaccharidosis type II). They had normal karyotypes but a marked deficiency of iduronate-2-sulfatase activity in both lymphocytes and fibroblasts. The molecular analysis of the iduronate-2-sulfatase gene revealed the R468L(G1403-->T) substitution in their genes. Although the sister's genomic DNA was heterozygous for the mutant allele, the sister's cDNA was found to be homogeneous for this mutation. The mother was found to be a heterozygote. The analysis of X chromosome inactivation by comparison of the methylation patterns of the androgen-receptor (AR) gene which was isolated from the sister's fibroblasts and leucocytes revealed a skewed X chromosome inactivation of the paternal allele. These findings indicate that a skewed X chromosome inactivation of the paternal gene and a point mutation in the maternal gene were responsible for the lack of iduronate-2-sulfatase activity in the sister.

Binding Sites↗
PubMed1998

Comparison of health behaviour and oral/medical conditions in non-insulin-dependent (type II) diabetics and non-diabetics.

One hundred and two dentate patients with type II diabetes mellitus and 98 non-diabetic subjects were examined for oral conditions and metabolic state. Self-reported health behaviour was analysed. From factor analysis four factors emerged: general health behaviour (GHB), perceived fatigue (PF), diet control (DC) and regular diet (RD). In diabetics PF, DC and RD were significantly higher than that in non-diabetics. Patients with diabetes were more likely to control their disease through a programme of decreased kilojoule intake leading to weight management. However, they tended to tire. The mean gingivitis index was significantly higher (p < 0.01) among diabetics (2.39) than among non-diabetics (1.99). The number of missing teeth was significantly higher (p < 0.01) for diabetics (6.7) when compared with non-diabetics (4.3). On the other hand, aetiological factors (plaque, calculus) and the level of dental health behaviour as expressed in the HU-DBI scores were similar. Probing pocket depth did not differ statistically between groups. The increasing number of missing teeth in diabetics may primarily result from severe periodontitis with tooth mobility or deep pockets. Findings in this study suggest that the difference in the severity of periodontitis between diabetics and non-diabetics was significant although aetiological factors and the level of dental health behaviour were similar.

Adult↗