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Biomedical subjects

K Ohno

Publications and source records attributed to K Ohno.

At least 379 records · Page 21Linked to original sources
PubMed1995

[Carbohydrate-deficient glycoprotein syndrome].

Carbohydrate-deficient glycoprotein syndrome is characterized by mental retardation, ataxia, hepatopathy during infancy, cerebellar hypoplasia, peripheral neuropathy, internal strabismus, growth retardation and stroke-like episodes. Since the description of female siblings with unique clinical and biochemical features by Jaeken (1980) and the discovery of unique isoforms of serum transferrin in the patients by Jaeken (1984), more than 120 patients have been diagnosed. The biochemical marker is asialo- and disialo-transferrin. We have found the first Japanese patients and, through analysing serum glycoproteins from these patients, we was noted that multiple serum glycoproteins contain abnormal fractions, on isoelectric focusing. By analysing the sugar chain of transferrin, we have found that the abnormality is caused by a defect in the transfer of asparagine-N-linked oligosaccharide. Recently, two clinical and biochemical variants have been reported. One, characterized by severe mental retardation, no cerebellar hypoplasia, no peripheral neuropathy, diasirotransferrin dominancy, has proven to have a deficiency of N-acetylglucosaminyltransferase II, by Jaeken (1993).

Congenital Disorders of Glycosylation↗
PubMed1995

[Lysosomal membrane transport disorders--cystinosis and sialic acid storage disorders (Salla disease, ISSD)].

Cystinosis and sialic acid storage diseases (Salla disease, ISSD; infantile sialic acid storage disease) are lysosomal membrane disorders resulting from defective carrier-mediated transport of cystine and sialic acid across the lysosomal membrane. Both are rare autosomal recessively inherited disorders. The major clinical manifestations of cystinosis are renal failure and ocular damages. Sialic acid storage diseases are characterized by various degrees of psychomotor retardation. Salla disease patients trace a mild clinical course, and the life span is relatively long. While, in patients with ISSD follow a very severe progressive clinical course and often die in the first year of life. The genes responsible for each disease have not been isolated, the etiologies are not well known, and there is no specific treatment.

Biological Transport↗
PubMed1994

[Clinical trials of TAE using micro-coil coated with n-butyl 2-cyanoacrylate and lipiodol].

Clinical trials of TAE using microcoils (Target, USA) coated with n-butyl 2-cyanoacrylate and lipiodol were performed. The procedures were as follows. First, a small amount of lipiodol was injected into a Tracker 18 catheter. Secondly, a microcoil coated with n-butyl 2-cyanoacrylate and lipiodol was inserted into the microcatheter. Thirdly, a small amount of lipiodol was again administered into the catheter just behind the coil. Finally, the coil was pushed safely out from the catheter with a special guidewire. With this method, excellent occlusion of small arteries was obtained. There were no complications.

Embolization, Therapeutic↗
PubMed1994

Hemostatic studies in patients with carbohydrate-deficient glycoprotein syndrome.

The carbohydrate deficient glycoprotein (CDG) syndrome is a newly described disorder characterized by impaired glycosylated molecules. It has been reported that transient stroke-like episodes appear in half of the patients. We performed hemostatic studies on three CDG syndrome patients belonging to two unrelated families. The most characteristic findings were decreases in antithrombin III (AT III), protein C and alpha 2 plasmin inhibitor to nearly half normal levels. Protein S was reduced in two (siblings) patients. Isoelectric focusing of AT III in native plasma revealed decreased intensity of the major band and increased intensity of a minor cathodal band. These minor AT III molecules were considered to lack an oligosaccharide sidechain. A 12-year-old girl defective not only for AT III but also protein C and protein S developed disseminated intravascular coagulation accompanied by arterial thrombosis in her left hand following dyspnea associated with bronchial asthma. These findings suggest that thrombotic predisposition in patients with CDG syndrome is due to decreased levels of major coagulation inhibitors, particularly as a result of impaired glycosylation of AT III.

Adolescent↗
PubMed1994

[Usefulness and safety of segmental-subsegmental TAE for hepatocellular carcinoma in cases of liver hypofunction].

We analyzed the safety of the segmental-subsegmental TAE for hepatocellular carcinoma in 18 cases of liver hypofunction by checking total bilirubin change after TAE. The TAE was performed safely in all of the cases. The highest value of total bilirubin before TAE was 5.1 mg/dl in the 1-2 subsegment TAE group, 3.8 mg/dl in the 3-4 subsegment TAE group and 2.0 mg/dl in the 6 subsegment TAE group. We conclude that segmental TAE is safe even in the patient of liver hypofunction.

Aged↗
PubMed1994

Expression of GABAA receptor gamma 1 and gamma 2 subunits in the peripheral vestibular system of the rat.

The distribution of GABAA receptor gamma 1 and gamma 2 subunits in the rat vestibular ganglion cells and end-organs was examined by using immunohistochemical techniques. Both gamma 1 and gamma 2 subunit-like immunoreactivities were observed in most vestibular ganglion cell bodies and peripheral terminal endings in the vestibular sensory epithelia. These results indicate that GABAA receptors are expressed in the vestibular afferent terminal endings and therefore suggest that GABA in addition to glutamate is a neurotransmitter which effects on vestibular afferents.

Afferent Pathways↗
PubMed1994

Occurrence of galanin-like immunoreactivity in vestibular and cochlear efferent neurons after labyrinthectomy in the rat.

The origins of the vestibular and cochlear efferent systems lie in the lower brainstem and innervate the labyrinth. In the present study we investigated the changes in galanin-like immunoreactivity (GAL-IR) in the vestibular and cochlear efferent neurons in control and labyrinthectomized rats. In control animals, no GAL-IR was noticed in these neurons. However, in response to unilateral labyrinthectomy, similar GAL-IR was bilaterally expressed in the two systems. GAL-immunostained cells appeared on postoperative day 3 and reached a peak of intensity and number at postoperative week 2. Retrograde tracing by fluorogold combined with GAL immunohistochemistry demonstrated that, except for the cells of the contralateral lateral superior olivary nucleus (LSO), GAL-IR neurons project into the lesioned labyrinth.

Animals↗
PubMed1994

Phenytoin, an antiepileptic drug, competitively blocked non-NMDA receptors produced by Xenopus oocytes.

The blocking effect of phenytoin (PHT) and other antiepileptic agents on the glutamate receptors was investigated under voltage-clamp conditions using Xenopus oocytes that translated ddY-stock mouse brain mRNA. PHT shifted the concentration-response curve for kainate to right without significant block on the maximum response obtained, indicating that PHT competitively blocks the non-NMDA receptors. An apparent dissociation constant of PHT was 1.9 x 10(-4) M. The block was voltage-independent. Other antiepileptic agents examined hardly blocked the kainate responses except phenobarbital that blocked the responses. The possibility that the competitive block constitutes a part of the antiepileptic action of PHT was discussed.

Animals↗
PubMed1994

Cultured rat hepatocytes adapt their cellular glycolytic activity and adenylate energy status to tissue oxygen tension: influences of extracellular matrix components, insulin and glucagon.

The influence of extracellular matrix components, insulin, and glucagon on the cellular response to periportal- or pericentral-equivalent tissue oxygen tension was investigated in freshly isolated rat hepatocytes cultured at 13% O2 or 4% O2 in Teflon membrane dishes. With extended culture time, significant increases in lactate release and cellular lactate content were observed in cultures at 4% O2 compared with 13% O2. This shift toward glycolysis was detectable when hepatocytes were cultured on dishes coated with rat liver crude membrane fraction (CMF/COL) but not in collagen type I-coated dishes. This indicates that extracellular matrix components are involved in the process of adaptation. ATP and total adenylate content in cells cultured at 4% O2 were up to 40% lower than in cells cultured at 13% O2. However, the adenylate energy charge was not affected, suggesting that an adequate energy supply was maintained also in hepatocytes cultured at pericentral-equivalent oxygen tension. This adaptation was reversible. When hepatocytes were transferred either from 4% to 13% O2 or from 13% to 4% O2, they adapted the corresponding metabolic profile to the new oxygen tension within 2 days. This demonstrates that hepatocytes are not fully unidirectionally programmed. The modulation of the glycolytic activity by insulin and glucagon was effective in cultures at pericentral-equivalent oxygen tension (4% O2) only. Insulin (0.1-100 nM) shifted cellular metabolism toward the glycolytic pathway and glucagon (1-100 nM) counteracted the effect of insulin in a dose-dependent manner. Clearly, oxygen tension is the principal regulator in the hepatic glycolytic activity, whereas the hormones (insulin and glucagon) act as secondary modulators.

Adenosine Triphosphate↗
PubMed1994

Tensile and viscoelastic properties of human patellar tendon.

The tensile and viscoelastic properties of fresh-frozen, nonirradiated human patellar tendon were investigated in two groups of 15 specimens: one group was from individuals 29-50 years old and the other group was from individuals 64-93 years old. The central portion of each patella-patellar tendon-tibia complex was subjected to cyclic preconditioning, stress-relaxation, cyclic stress-relaxation, and load to failure tests. For each age group, stress-relaxation and stress-strain curves were obtained, from which percentage relaxation, ultimate tensile strength, strain at failure, modulus, and strain energy density were determined. Viscoelastic behavior was described with use of quasilinear viscoelasticity. The younger group showed a 46 +/- 9% (mean +/- SD) decrease in stress after 15 minutes, whereas the older group exhibited a 50 +/- 6% decrease. The values for ultimate tensile strength and strain at failure, respectively, were 64.7 +/- 15.0 MPa and 14 +/- 6% for the younger group and 53.6 +/- 10.0 MPa and 15 +/- 5% for the older group. Modulus values were 660 +/- 266 MPa for the younger group and 504 +/- 222 MPa for the older group. Except for ultimate tensile strength, which was 17% less for the older group than for the younger one, no statistically significant differences were found in tensile or viscoelastic properties. This study indicated that there were minimal differences in biomechanical properties of the substance of the patellar tendon between younger and older age groups.

Adult↗
PubMed1994

Chronological changes in brain edema induced by experimental intracerebral hematoma in cats.

To investigate the temporary profile of changing perifocal brain edema around an intracerebral hematoma (ICH), we developed an experimental ICH model using cats. The developing perifocal edema in the white matter around the hematoma was measured by means of a gravimetric technique. Edema was more severe near the ICH, and declined with increasing distance. Edema was mild 2 hours after the onset of the ICH, and was most severe in all the regions examined 3 days later. Edema decreased but still existed in all regions 7 days after the onset of the ICH. The results suggest that the mechanism of the development of edema associated with ICH seems to differ from that associated with a cold injury. This experimental ICH model proved to be useful for the study of formation, expansion, and resolution of edema associated with ICH.

Animals↗
PubMed1994

Effect of acetazolamide on early ischemic cerebral edema in gerbils.

Acetazolamide was given in the early stage of ischemic cerebral edema produced by unilateral permanent carotid occlusion in gerbils. The animals were studied 1, 4, and 6 hours after ischemia. The tissues were examined for water and electrolyte concentrations and ischemic areas were visualized by 2,3,5-triphenyltetrazolium chloride (TTC) and H-E staining. Acetazolamide injected just after occlusion showed a positive effect in reducing edematous changes. Later administration of the drug had neither positive nor harmful effect on the ischemic brains. Thus, acetazolamide seems to be useful for cerebrovascular response studies in the early stage of a brain lesion.

Acetazolamide↗
PubMed1994

Hypoxanthine-guanine phosphoribosyltransferase (HPRT) deficiency: identification of point mutations in Japanese patients with Lesch-Nyhan syndrome and hereditary gout and their permanent expression in an HPRT-deficient mouse cell line.

Two different single nucleotide transitions of hypoxanthine-guanine phosphoribosyltransferase (HPRT) were identified in a Japanese patient with Lesch-Nyhan syndrome (LNS) and a patient with hereditary gout. HPRT enzyme activities in the two patients were severely deficient, but the size and amount of mRNA were normal according to Northern analysis. Entire coding regions of HPRT cDNAs were amplified by PCR and sequenced. A G-to-A substitution at base 208 in exon 3, which predicted glycine 70 to arginine, was detected in the LNS patient (identical mutation with HPRT Utrecht). A C-to-A substitution at base 73 in exon 2, which predicted proline 25 to threonine, was detected in the gout patient (designated HPRT Yonago). We transfected normal HPRT cDNA, mutant cDNA with HRPT Utrecht or mutant cDNA with HPRT Yonago, respectively, to HPRT-deficient mouse cells and isolated permanent expression cell lines. The HPRT-deficient mouse cells had no detectable HPRT activity and a very low amount of HPRT mRNA. When the HPRT-deficient mouse cells were transfected with normal human cDNA, HPRT enzyme activity increased to 21.8% that of normal mouse cells. The mouse cells transfected with HPRT Utrecht showed no increase in HPRT activity; however, when the mouse cells were transfected with HPRT Yonago, the activity increased to 2.4% that of normal activity. The proliferative phenotypes of these cells in HAT medium and in medium containing 6-thioguanine were similar to those of skin fibroblasts from the patients. This series of studies confirmed that each of the two point mutations was responsible for the decreases in HPRT enzyme activity, and the proliferative phenotypes in HAT medium and medium containing 6-thioguanine.

Adolescent↗
PubMed1994

Induction of apoptosis in a T lymphoblastoid cell line infected with feline immunodeficiency virus.

The mechanism of cell death induced by feline immunodeficiency virus (FIV) infection was investigated in an interleukin 2(IL-2)-dependent T-lymphoblastoid cell line (MYA-1). DNA extracted from FIV-infected MYA-1 cells showed a ladder of nucleosomal DNA, indicating that the cytopathic effect (CPE) observed in these cells was due to apoptosis. Infection of MYA-1 cells with FIV was associated with suppression of the proliferative response of the cells to exogenous IL-2 prior to DNA fragmentation. These findings suggest that FIV-induced CPE in these T-lymphoblastoid cells is associated with apoptosis possibly due to a defect in the IL-2 signal transduction pathway.

Animals↗
PubMed1994

Immediate insertion of two types of implants into vascularized bone grafts used for mandibular reconstruction in miniature pigs.

Reaction of bone tissue was investigated after placement of two different types of implants in vascularized iliac bone grafting. After the mandibles of six Göttingen minipigs were reconstructed with vascularized bone of the iliac crest, hydroxyapatite-coated implants and titanium screw implants were placed in the graft. The animals were killed at 14, 28, and 56 days after the implantation, and the healing processes were examined histologically. In the vascularized bone graft, new bone formed well around the implants, but some of the implant surfaces were covered with soft tissue by advancing bone resorption. The results suggest that a hydroxyapatite-coated implant as well as a titanium screw-type implant might be applied with a vascularized bone graft for jaw reconstruction. However, implants placed simultaneously with the vascularized bone graft had less osteointegration than implants placed in nongrafted control sites.

Animals↗