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Biomedical subjects

M Okabe

Publications and source records attributed to M Okabe.

At least 505 records · Page 28Linked to original sources
PubMedDate not supplied

Skeletal muscle involvement in mucopolysaccharidosis type IIA: severe type of Hunter syndrome.

A patient with mucopolysaccharidosis type IIA (MPS IIA) and progressive gait disturbance is described. The histopathology of biopsied muscle was studied; Dorling's method revealed muscle fibers and interstitial cells containing metachromatic granules which suggested the storage of sulfated acidic glycosaminoglycans. Electron microscopy demonstrated that the membrane-bound vacuoles were present in muscle fibers, subsarcolemmal area, vascular endothelial cells, satellite cells, and endomysial fibroblasts. Besides clinical features, this ultrastructural pathology in MPS IIA muscles of MPS IIA was more severe than MPS IIB muscles. The accumulation of glycosaminoglycans in muscle tissue may be an additional factor contributing to gradual motor impairment of patients with MPS IIA.

Biopsy↗
PubMedDate not supplied

Rett syndrome: findings suggesting axonopathy and mitochondrial abnormalities.

We report the histopathologic findings of 3 sural nerve biopsies and 1 muscle biopsy from 3 patients with Rett syndrome. The 3 sural nerve biopsies demonstrated a few ultrastructural abnormalities, including the presence of many Pi-granules and mitochondrial changes in the cytoplasm of Schwann cells, occasional bands of Büngner and onion-bulb formations, and mitochondrial alterations in myelinated axons. Morphometric analysis disclosed reduction in the number of large myelinated fibers with normal densities in comparison to those of an age-matched normal control. Light microscopic examination of the biopsied muscle from a 6-year-old patient with Rett syndrome revealed the existence of many small, dark, angulated fibers with NADH-TR staining. Ultrastructural investigation of the muscle confirmed the presence of the dumbbell-shaped mitochondria. Peripheral nerve involvement and the possibility of mitochondrial abnormalities in Rett syndrome were suggested by the results.

Axons↗
PubMedDate not supplied

Proximal and distal motor nerve conduction velocities in Werdnig-Hoffmann disease.

Using the latencies of M and F responses, we assessed motor nerve conduction velocity along the entire course of the median and ulnar nerves from the spinal cord to the muscle in 14 patients with the less severe forms of Werdnig-Hoffmann disease. In these forms, the motor nerve conduction velocities were decreased significantly over both proximal (cord-to-elbow) and distal (elbow-to-wrist) segments in both the nerves as compared with normal values; however, the mean motor nerve conduction velocities in the proximal segments were faster than those in the distal segments by about the same amount as the normal controls. These findings indicate that motor conduction abnormalities in Werdnig-Hoffmann disease are diffuse over the entire course of the nerve and appear to eliminate a dying-back process in which the affected axons are severely damaged, beginning with the more distal sites.

Adolescent↗
PubMedDate not supplied

Attenuated SEPs with no latency shifts in a family with hereditary spastic paraplegia.

Five siblings with hereditary spastic paraplegia of autosomal-dominant inheritance were studied with somatosensory evoked potentials. Somatosensory evoked potentials were recorded from Cz', T12, and the left popliteal fossa following left posterior tibial nerve stimulation. The latency and amplitude of the corresponding potentials (i.e., P37, N20, and N7) were compared with normal values obtained from age- and height-matched controls. There was no significant difference in the values of N7, suggesting an intact afferent peripheral pathway; in contrast, the amplitudes of P37 and N20 were decreased with normal latencies. The degree of amplitude decrease correlated with the severity of vibration sense impairment in the lower limbs. These results appear to support selective axonopathy of the centrally directed axons of the dorsal root ganglion cells. Furthermore, our results suggest that different degrees of dorsal column involvement in hereditary spastic paraplegia can cause different types of somatosensory evoked potential abnormalities, namely, attenuated amplitudes with no latency shifts, as recorded in this family, and the prolonged latencies, as reported previously.

Adolescent↗
PubMedDate not supplied

Backscattered electron imaging: A new method for the study of cardiomyocyte architecture using scanning electron microscopy.

Scanning electron microscopy (SEM) with secondary electron emissions is useful for the study of cardiomyocyte architecture, however, the information is limited from the cell surface. Whereas backscattered electron (BSE) emission can give a high-resolution image of the specimen's intracellular structure after heavy metal staining. In this study, we applied BSE imaging analysis to the study of the arrangement of cardiomyocytes in the myocardium. The tissue specimens from a normal fresh monkey heart, normal human heart obtained at autopsy, and surgically resected tissue from a patient with old myocardial infarction in the left ventricular aneurysmectomy were used. The tissue specimens were fixed in neutral formalin, treated with NaOH and then stained with Gomori's silver methenamine reagent followed by tannic acid and osmium tetroxide. After dehydration and drying, the specimens were coated with carbon and examined by SEM with a BSE detector. In the tissue preparations, the A bands of sarcomeres were selectively stained with silver so that the arrangements of subsarcolemmal myofibrils and the intercalated discs were clearly seen in the BSE images. In the left ventricular aneurysmal walls of old myocardial infarction, atrophied cardiomyocytes with disarray of subsarcolemmal myofibrils were observed. The results strongly suggest that BSE images are further applicable to the study of the architecture of cardiac myocytes and their branches, and the arrangement of intracellular myofibrils in various diseased myocardium.

Animals↗
PubMedDate not supplied

Dependence of apparent viscosity on mycelial morphology of Streptomyces fradiae culture in various nitrogen sources.

To examine what causes increased viscosity in culture broth in Streptomyces fradiae culture, various natural nitrogen sources were investigated. Extracellular protease activity increased with culture time and decomposed the natural nitrogen source into amino acids. In the case of gluten meal, after a culture time of 5 d, concentrations of glutamic acid and aspartic acid had increased to 600 and 200 mg/L, respectively, which were about 3- and 2-fold as high as levels in cultures under similar conditions using Pharmamedia. For various amino acids tested, the addition of glutamic acid or aspartic acid mixture to the culture medium raised the apparent viscosity to its highest demonstrated value, 260 mPa.s after 5 d of culture, which was 3-fold higher than without amino acids. Consumption of the decomposed glutamic acid and aspartic acid was dependent on the activities of glutamate dehydrogenase and aspartate aminotransferase, respectively. When ammonium ion was used as the nitrogen source, cell concentration reached 1.75 g/L measured as an intracellular nucleic acid concentration, which was about 2.3-fold higher than that with any other natural nitrogen source. However, apparent viscosity was only 75 mPa.s, a value one-third that of the amino acid mixture, and 70% of the pellets were bigger than 1.2 x 10(4) microm(2). In the case of gluten meal or the amino acid mixture, pellets bigger than 1.2 x 10(4) microm(2) comprised only 8%. This demonstrates that consumption of some amino acids affected the formation of filamentous morphology, which caused an increase in the apparent viscosity of the culture broth, and the apparent viscosity was not caused by the mycelial concentration but the mycelial morphology.

Amino Acid Oxidoreductases↗
PubMedDate not supplied

Efficient production of L-(+)-lactic acid using mycelial cotton-like flocs of Rhizopus oryzae in an air-lift bioreactor.

L-(+)-Lactic acid production was enhanced in a culture of Rhizopus oryzae by induction of a mycelial flocs morphology. By conventional culture the morphology of R. oryzae is that of a pellet-like cake; however, when mineral support and poly(ethylene oxide) are added to the culture, the morphology of R. oryzae takes on a cotton-like appearance. The formation of these cotton-like mycelial flocs was induced by the addition of 5 ppm poly(ethylene oxide) into a 12-14 h culture containing 3 g/L of the mineral support before the formation of the conventional pellet morphology. The cotton-like flocs were also formed in cultures grown in an air-lift bioreactor. This morphology allowed effective mass transfer inside the flocs and effective fluidity of culture broth in an air-lift bioreactor. L-(+)-Lactic acid concentration produced by mycelial flocs in an air-lift bioreactor, with the support and poly(ethylene oxide), was 104.6 g/L with a yield of 0.87 using 120 g/L of glucose as the substrate; for this culture without both, the concentration was 43.2 g/L. These results demonstrate that cotton-like mycelial flocs are the optimal morphology for use in the air-lift bioreactor culture of R. oryzae.

Bioreactors↗
PubMedDate not supplied

A rhodopsin is the functional photoreceptor for phototaxis in the unicellular eukaryote Chlamydomonas.

Rhodopsin is a visual pigment ubiquitous in multicellular animals. If visual pigments have a common ancient origin, as is believed, then some unicellular organisms might also use a rhodopsin photoreceptor. We show here that the unicellular alga Chlamydomonas does indeed use a rhodopsin photoreceptor. We incorporated analogues of its retinal chromophore into a blind mutant; normal photobehaviour was restored and the colour of maximum sensitivity was shifted in a manner consistent with the nature of the retinal analogue added. The data suggest that 11-cis-retinal is the natural chromophore and that the protein environment of this retinal is similar to that found in bovine rhodopsin, suggesting homology with the rhodopsins of higher organisms. This is the first demonstration of a rhodopsin photoreceptor in an alga or eukaryotic protist and also the first report of behavioural spectral shifts caused by exogenous synthetic retinals in a eukaryote. A survey of the morphology and action spectra of other protists suggests that rhodopsins may be common photoreceptors of chlorophycean, prasinophycean and dinophycean algae. Thus, Chlamydomonas represents a useful new model for studying photoreceptor cells.

Cell Movement↗
PubMedDate not supplied

Myocardial inflammatory cell infiltrates in cases of dilated cardiomyopathy: light microscopic, immunohistochemical, and virological analyses of myocardium specimens obtained by partial left ventriculectomy.

OBJECTIVE: Partial left ventriculectomy was introduced for the treatment of refractory dilated cardiomyopathy (DCM). To determine the presence and degree of inflammatory cell infiltrates in DCM and the correlation between the underlying myocardial injury and early clinical outcomes after the operation, we performed histopathological, immunohistochemical, and virological studies of the resected myocardium. METHODS: Posterolateral walls of the left ventricle from 13 idiopathic DCM patients (9 males and 4 females; mean age = 53+/-14 years) were examined. Qualitative and quantitative analyses of the interstitial fibrosis and of the infiltrating inflammatory cells were conducted. For the immunohistochemistry, leukocyte surface markers and antibodies to adhesion molecules and cytokines were used. The histopathological findings were compared with the clinical results, including outcome within 1 year, and pre- and postoperative hemodynamic data. Genomic analysis of the myocardium with polymerase chain reaction was performed for enterovirus, mumps, influenza A, cytomegalovirus, and hepatitis C virus. RESULTS: (1) The three patients who died of cardiac insufficiency after surgery had a higher count of infiltrating inflammatory cells than the eight survivors (32.1+/-10.4 vs 16.3+/-11.9 cells/mm2, p = 0.07). The severity of interstitial fibrosis (percent fibrosis) did not differ significantly between these two groups (28.3+/-15.0 vs 24.0+/-11.7%). (2) In patients who died of myocardial dysfunction, focal accumulations of lymphocytes were common, in which cytotoxic/suppressor T cells and helper/inducer T cells were observed. (3) Enterovirus genome was detected in the myocardium of two patients, both of them died after surgery. CONCLUSIONS: Inflammatory cell infiltrates or active myocarditis appear in some cases to play an important role in the etiology and pathophysiology of clinically diagnosed DCM. There is a possibility that those patients with a more severe or ongoing inflammatory process might have poor outcomes after partial left ventriculectomy.

Adult↗
PubMedDate not supplied

Glowing podocytes in living mouse: transgenic mouse carrying a podocyte-specific promoter.

Green fluorescence protein (GFP) has been utilized as a marker of gene expression due to the great advantage in its simple and quick detectability. We generated transgenic mice carrying a GFP cDNA under the control of a beta-actin/beta-globin promoter (CX promoter) and cytomegalovirus enhancer. The green luminescence derived from GFP was apparent in skeletal muscle, pancreas, heart and kidney, but not in other tissues. The GFP expression in the kidney was localized in podocytes. Moreover, in situ hybridization of GFP showed that the transcriptional level was highly active in the podocytes. These results suggested that the glowing green fluorescence would be a useful in vivo marker of podocyte in these transgenic lines in the physiological and pathophysiological state, and that the CX promoter could allow a podocyte-specific expression of a molecule of interest in kidney.

Animals↗
PubMedDate not supplied

Subclavian vein puncture under ultrasonic guidance.

Ultrasonically guided subclavian venipuncture is described. Since this method permits direct tapping of the subclavian vein and control of the insertion to the innominate vein under ultrasonic guidance, complications such as pneumothorax, accidental subclavian artery puncture, and malposition of the catheter, which often accompany the conventional method, can be avoided. As a result, this technique produces no radiation damage.

Aged↗
PubMedDate not supplied

Preoperative evaluation and surgical treatment for tricuspid regurgitation associated with acquired valvular heart disease. The Kay-Boyd method vs the Carpentier-Edwards ring method.

This study compared the results of annuloplastic repair of tricuspid regurgitation (TR) using Doppler echocardiography. Sixty-three patients who underwent tricuspid annuloplasty were studied. Thirty-four patients received Kay-Boyd annuloplasty and 29 Carpentier-Edwards ring annuloplasty. A new classification of TR based on the direction and area of regurgitation flow on Doppler echocardiogram was applied preoperatively. In the Kay-Boyd group, 10 cases showed massive TR and 24 cases showed localized TR preoperatively. Localized TR was well controlled in all cases, but 8 of 9 cases of massive TR showed grade III residual TR. In the C-E group, 21 cases showed massive TR and 8 cases showed localized TR. All cases were well controlled postoperatively. We conclude that (1) although the Kay-Boyd method is acceptable for localized TR, the C-E method should be employed for massive TR; (2) analyzing the regurgitant pattern of TR by Doppler echocardiogram is useful in selecting an appropriate surgical technique.

Adult↗