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Biomedical subjects

S Miyake

Publications and source records attributed to S Miyake.

At least 19 recordsLinked to original sources
PubMed1992

Demonstration of cathepsins B, H and L in xenografts of normal and Duchenne-muscular-dystrophy muscles transplanted into nude mice.

The activities and contents of the lysosomal cysteine proteinases cathepsins B, H and L were examined in xenografts of biopsied muscles transplanted from age-matched normal subjects and Duchenne-muscular-dystrophy (DMD) patients into nude mice. The activity of cathepsin B increased 9-fold and that of B-plus-L increased 24-fold in the first week after transplantation in normal muscle xenografts. By the third week, the activity of cathepsin B increased a total of 20-fold and B-plus-L increased to 36-fold the original level. The activity levels of cathepsin B, B-plus-L, H and D, and acid phosphatase in normal and DMD xenografts were not significantly different when compared 2 weeks after transplantation. However, the protein content of cathepsin B in DMD muscle xenografts was more than 3-fold that of normal xenografts at 2 weeks. The profile of cathepsin H activity in normal muscle xenografts was different than those of cathepsins B and B-plus-L. In the first week, the cathepsin H diminished sharply to about one-third of the biopsied muscle level and then, by 3 weeks after transplantation, it had increased slightly to about half the original level. The amount of endogenous cysteine-proteinase inhibitor changed in parallel with the activity of cathepsins B and B-plus-L. Cathepsins B and H, but not cathepsin L, were found immunohistochemically in regenerating muscle fibres of normal and DMD xenografts 2 weeks after transplantation. Staining of cathepsin B in DMD xenografts was slightly stronger than that in normal subjects. There was no immunostaining in degenerating or necrotic muscle fibres 2 weeks after transplantation. Western-blot analysis revealed that the cathepsin B band at 29 kDa was increased in normal xenografts 2 and 3 weeks after transplantation. Also, 2 weeks after transplantation the staining intensity of this band was slightly stronger in DMD xenografts than in normal xenografts. These results suggest that cathepsin B participates in the regeneration of transplanted muscle, both normal and DMD, and in the DMD muscle fibre-wasting processes, during regeneration.

Acid Phosphatase↗
PubMed1992

Pigmentary dysplasias and chromosomal mosaicism: report of 9 cases.

Chromosomes were studied in 9 individuals with pigmentary dysplasias of the skin and other abnormalities. Of the 9 individuals, 5 were chromosomal mosaics in both blood lymphocytes and skin fibroblasts (46,XY/47,XY, + 13;46,XX/47,XX, + 14;46,XY/47,XY, + 18;46,XX/47,XX, + 18;46, XX/47,XX, + mar), while the other 4 individuals were chromosomally normal in both tissues studied. The pigmentary dysplasias involved hypo- or hyperpigmented patches/flecks or lines/whorls. The latter ran along Blachko lines on the back, abdomen and the limbs. These patterns varied not only between individuals but also between different regions of an individual. The possibility of chimerism was studied but ruled out (1/32 to 1/256) in 7 individuals, using chromosomal heteromorphisms in the patients and their parents as markers.

Adolescent↗
PubMed1992

A newly identified null allelic mutation in the human lipoprotein lipase (LPL) gene of a compound heterozygote with familial LPL deficiency.

In a Japanese patient with familial LPL deficiency, a new null allelic mutation, one base pair deletion at nucleotide position 916 was identified in exon 5 of one allele. In exon 3 of the other allele, we found the same nonsense mutation as we described previously in other Japanese kindreds. For the deletional mutant allele, we developed a simple detection method and constructed the DNA haplotype.

Alleles↗
PubMed1992

Unique karyotypes in two patients with Prader-Willi syndrome.

A physical disruption of the Prader-Willi syndrome (PWS) chromosome region is thought to cause PWS. We describe 2 girls with PWS phenotype, who had unique chromosome 15 abnormalities. The first patient showed mosaicism: 45,XX,t(15;15)(qter----p11.1::q11.200----qter)/46,XX,t(15;15)(qter----p1 1.1::q 11.200----qter), +mar. The band 15q11.2 apparently remained intact in the t(15;15) chromosome, and the mar chromosome was considered as r(15) (p11.1q11.1). The second patient had a karyotype of 47,XX,del(15)(q11.200----q11.207), +idic (15)(pter----q11.1::q11.1----pter). The complex breakage and reunion involving the 15q11.2 regions of the father's homologous chromosomes 15 at meiosis appeared to have resulted in the idic(15) and the del(15) chromosomes. These cytogenetic findings suggest that the PWS chromosome region may be localized on the very proximal portion of band 15q11.2.

Child↗
PubMed1992

Removable magnetic dental attachment that permits magnetic resonance imaging.

Magnetic dental attachments cause magnetic resonance image (MRI) degradation. A new magnetic dental attachment that allows removal of magnetic parts to exchange them for nonmagnetic parts was developed and tested. It allows high quality MRI with no change in occlusal vertical dimension. A volunteer test subject with combinations of attachments in place was examined by MRI. The images produced showed no degradation or distortion. Magnetic attachments for overdentures or maxillofacial prostheses should be removable to permit use of MRI.

Cobalt↗
PubMed1992

Power spectral analysis of heart rate variability in Type As during solo and competitive mental arithmetic task.

It has been reported that there are differences in autonomic balance between Type As and Type Bs. This study evaluated the sympathovagal interaction in Type A (N = 10) and Type B (N = 10) male students during mental arithmetic task in a solo and a competitive condition by the spectral component analysis of heart rate variability (HRV). The low-frequency (LF) component to high-frequency (HF) component ratio was significantly greater in Type As than in Type Bs, though no significant differences were found in task performance, heart rate change, and blood pressure between the two subject groups in both conditions. The present findings indicate that there was a significant difference in sympathovagal balance between Type As and Type Bs, and that Type As showed dominant sympathetic activity. The results suggest that the power spectral analysis of HRV, which is convenient and non-invasive, has enough sensitivity to discriminate differences in autonomic balance between Type A subjects and Type B subjects, not only during the solo and competitive task period but also during the resting period.

Heart Rate↗
PubMed1992

Congenital polyneuropathy in Walker-Warburg syndrome.

Polyneuropathy was found in a patient with the Walker-Warburg syndrome. The most dominant features were the presence of extremely and tortuously proliferated myelin sheaths, the most of which having no neurofilaments and neurotubules. The other peculiar findings were the presence of microfilaments in Schwann cell cytoplasms, which were very similar to neurofilaments, and the presence of partial and abrupt disappearance of myelin sheaths. The severity of neuropathy was variable among nerve bundles, and a few nerve bundles looked normal on light microscopy. The above-mentioned lesions did not suggest the degeneration and/or regeneration of normally developed nerve fibers. We could not conclude the pathogenesis of this neuropathy, however, it was logical to consider that they reflected dysplastic myelination due to Schwann cell dysmaturity as well as the cerebral dysplasia.

Axons↗
PubMed1992

Ranges of dynamic motion of the wrist in healthy young and middle-aged men.

The difference in the range of dynamic motion of the wrist between young and middle-aged men was studied. To analyse wrist function easily, a new quantitative evaluation technique for wrist circumduction using a biaxial flexible electrogoniometer was introduced. A phase plane was made from two-channel signals of the electrogoniometer, and the 'ROM (range of motion) Index' for wrist circumduction was defined. Twelve healthy young men (age 19-31 years) and twelve healthy middle-aged men (age 45-63 years) were studied. Under forearm fixed conditions, there was no difference of the ROM Index between the two age groups. A significant decrease of the ROM Index in the middle-aged group was found in the non-dominant wrists without forearm fixation. The four quadrantal analysis of wrist circumduction clarified that this difference appeared especially in the radiodorsal direction. These results suggest that in the middle-aged men, the motor function involving the radiocarpal and midcarpal joints does not decrease, but that for the proximal and distal radioulnar joints in the non-dominant side drops.

Adult↗
PubMed1992

[Detection of dystrophin with anti-peptide antibodies].

The analysis of dystrophin in skeletal muscles was performed to identify Duchenne and Becker muscular dystrophy (DMD and BMD) by means of immunohistochemical stain and Western blotting with antisera against synthetic dystrophin peptides. The control muscle specimens derived from normal healthy persons, and patients without DMD and BMD revealed clearly continuous stains of dystrophin at surface membrane. A band with 400 kDa of molecular size by Western blotting was positively stained by anti-dystrophin antibodies. The muscle specimens from eleven DMD patients showed no observation both in the band on Western blotting and in the immunohistochemical staining of dystrophin on frozen-thin sections. BMD muscle specimens showed patchy and faint stains, but no detection of any band on Western blotting except a 380 kDa minor band with anti-peptide IX antibody in one patient muscle. The immunohistochemical procedure was found to be more sensitive than Western blotting for the detection of dystrophin. These results indicate that the dystrophin analysis by both methods is an useful tool for the differential diagnosis of patients with DMD and BMD.

Animals↗
PubMed1992

[A case of pulmonary alveolar proteinosis and disseminated atypical mycobacteriosis complicating chronic myelogenous leukemia].

A 47-year-old woman with chronic myelogenous leukemia was treated with daily busulfan (total dose approximately 500 mg) from December 1988 to January 1990. The disease thereafter remained stable with no evidence of blastic transformation. In February 1990 she developed productive cough and abnormal acinar lung shadows appeared transiently on her chest X-ray. In October 1990, productive cough and linear and abnormal acinar lung shadows reappeared. Expectorated sputa contained acid-fast bacilli (Gaffky 6, 10). Antituberculous therapy was started, which caused severe liver dysfunction. She was admitted to our hospital for evaluation of abnormal lung shadows. Transbronchial lung biopsy revealed pulmonary alveolar proteinosis with thickening of alveolar septa. The alveolar septal thickening was suspected to be a pathological change following pulmonary alveolar proteinosis. Cultures from sputum, cerebrospinal fluid, and bone marrow aspiration specimens revealed atypical mycobacterium (M. avium complex), and the diagnosis of disseminated atypical mycobacteriosis was established. The pathogenesis of the disseminated atypical mycobacteriosis was considered to be superinfection by mycobacteria.

Busulfan↗
PubMed1992

[An autopsy case of Fahr disease (infantile form)].

A 13-year-old girl with Fahr disease (infantile form) was reported. Her parents were consanguineous. Her elder sister had mental retardation and spasticity of the lower limbs, and died at 23 years of age. The patient suffered from infantile spasms at 3 month. She was bed-ridden, nonverbal, microcephalic and blind. Cranial CT revealed massive calcifications in the basal ganglia, periventricular white matter, dentate nucleus and cerebellar white matter. EEG showed a suppression-burst pattern. At 13 years, she died of pneumonia and hyperammonemia. Microscopic examination of brain showed perivascular non-arteriosclerotic ferro-calcinosis. The periventricular granules are 1-4 mu or 12 mu in diameter. This pathological change was observed only in the central nervous system above midbrain. No calcifications were found in the pituitary and the vessels of pia mater. Also a reduced ornithine transcarbamylase activity was found in the liver, which was probably not related with cerebral calcifications. Infantile form of Fahr disease is rare and may be heterogeneous in etiology. However, clinical manifestations and pathological findings were similar to those in previous reports of Fahr disease in childhood. It is one of the disorders causing infantile spasms.

Adolescent↗
PubMed1992

Pharmacological profile of a novel 1,5-benzothiazepine calcium antagonist.

The in vitro cardiovascular effects of a new 1,5-benzothiazepine derivative, RS-5773 ((2S,3S)-3-acetoxy-8-benzyl-2,3-dihydro-5-[2-(dimethylamino)ethyl]-2-(4- methoxy-phenyl)-1,5-benzothiazepine-4-(5H)-one hydrochloride, CAS 129173-57-5), were examined in isolated rat aorta and isolated guinea pig heart preparations. Like diltiazem or nifedipine, RS-5773 preferentially relaxed K(+)-contracted aorta rather than phenylephrine-contracted aorta, suggesting that the agent interfered with calcium channels. Vasorelaxant effects of RS-5773 on K(+)-contracted aorta was about 5 times more potent than those of diltiazem. The vasorelaxation with RS-5773 developed very slowly and was resistant to washout. The negative effects of the agent on contractile force of guinea pig papillary muscles and beating rate of guinea pig atria were not much different from those of diltiazem, although the actions of RS-5773 developed more slowly than those of diltiazem. These results indicate that RS-5773 is a diltiazem congener with vascular preference and long-lasting actions. In this respect, RS-5773 resembled clentiazem. But vascular and cardiac effects of RS-5773 developed more slowly than those of clentiazem and they were more resistant to washout.

Animals↗
PubMed1992

[Prevalence rate of severely mentally and physically handicapped children in school age in Yokohama].

Prevalence rate and pattern of disabilities were studied in severely mentally and physically handicapped children in school age in Yokohama. We visited institutions and schools for retarded children in Yokohama and its neighborhood or made contact with them by telephone. The study disclosed a total of 192 children on May 1, 1988. Prevalence rate was 0.51 per 1,000. Patterns of disabilities were: 1) most of the children (94.8%) lived with their families. 2) eighty-eight percent of the children attended school for retarded children. 3) about twenty percent of the children who attended school needed tube feeding.

Child↗