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Biomedical subjects

M Matsui

Publications and source records attributed to M Matsui.

At least 163 records · Page 9Linked to original sources

[A case of familial myoclonus showing extremely benign clinical course].

We report a patient with familial myoclonus showing an extremely benign clinical course. The patient was a 70-year-old woman, who first noticed shaking of hands at age of 25. The symptom did not worsen for more than 40 years. She visited our hospital at the age of 70 because of disturbance in chores because of worsening of her hand shaking in the past one year. A family history showed that 4 members had similar symptoms and that the two were afflicted with fits of loss of consciousness. On neurologic examination, rhythmic myoclonic jerks were noted in all the extremities, more in the upper limbs, both at rest and during action. Tandem gait was mildly disturbed. The remainings of neurologic examination were normal. SEP and jerk-locked back averaging provided evidence of cortical myoclonus. EEG showed multifocal polyspike discharges. Gene analysis for DRPLA, pyruvate and lactate levels in serum and the cerebrospinal fluid, serum amino acid levels, and CSF HVA and 5-HIAA levels were all normal. No brain atrophy was noted in cranial MRI. Myoclonus was markedly reduced after administration of clonazepam. The clinical features and electrophysiological data of our patient are consistent with the clinical diagnosis of familial essential myoclonus and epilepsy/benign adult familial myoclonic epilepsy.

Aged↗

[A sibling of delayed post-anoxic encephalopathy after strangulation].

A sibling of three year old girl and a year old boy, showed delayed post-anoxic encephlopathy after strangulation. After three days of the accident, the girl developed tetraplegia and choreo-athetosis. Her brother also developed choreo-athetosis two weeks after strangulation. T2 weighted MRI revealed a high signal intensity in the bilateral putamen and caudate nucleus. After hyperbaric oxygen therapy for two months, their symptoms diminished. We hypothesize that the functional damage of the neurons occurred in the bilateral basal ganglia as delayed neuronal death because of their vulnerability and peculiarity of the local circulation. Hyperbaric oxygen therapy may be effective in rescuing the neurons from hypoxia.

Asphyxia↗

[The Osaka concept. "Dome osteotomy" with of without labrum resection].

Acetabular dysplasia represents the most common aetiology for secondary osteoarthritis of the hip joint in Japan. On radiographs progress of the disease can be classified in four stages (I to IV). Labral lesions are common accompanying findings in acetabular dysplasia, representing an important prognostic factor. Therefore we routinely use conventional arthrography to classify labral lesions in three types (normal, torn and detached). From 1978 to 1983 a modified Chiari osteotomy of the pelvis (dome osteotomy) was performed in 64 hip joints without arthrotomy. The preoperative radiography showed labral lesions in 66%. After an average follow-up of 4 years, 43 of the 44 patients with a normal or torn labrum showed excellent or good results. On contrast, 50% of the 20 patients with a detached labrum showed fair and poor results only. From 1984 to 1989 an arthrotomy was combined with dome osteotomy in 29 hip joints and labral lesions had to be resected in 16 cases. The results were generally good in patients with arthrosis grade I and II, but in stage III they were only fair. Thus, the success of labral surgery can only be achieved in early stages of dysplasia. Based on our findings dome osteotomy combined with arthrotomy and labral surgery can be recommended in patients with labral lesions and hip dysplasia grade I and II.

Acetabulum↗

Mapping of eight testis-specific genes to mouse chromosomes.

We previously identified eight testis-specific genes using antibodies raised against testicular germ cells. They are expressed during spermatogenesis and are presumed to be involved in testicular germ cell differentiation and sperm formation. We have mapped the genomic loci for these testis-specific genes using restriction fragment length variants in interspecific backcross mice. The calmegin gene (Clgn) was mapped to Chr 8. The synaptonemal complex protein gene 1 (Sycp1) probe hybridized with two sequences on different chromosomes; Sycp1-rs2 was mapped to Chr 3, whereas Sycp1-rs3 was mapped to Chr 7. The relaxin-like factor gene (Rlnl) was mapped to Chr 8, and collapsin response mediator protein 1 (Crmp1) was mapped to Chr 5. Three novel genes encoding testis-specific proteins A2 (Tsga2), A8 (Tsga8), and A12 (Tsga12) were mapped to chromosomes 3, X, and 10, respectively.

Animals↗

Construction and expression of chimeric rat liver hydroxysteroid sulfotransferase isozymes.

The St-20 and ST-40 cDNAs encode rat liver hydroxysteroid sulfotransferases (HS-ST) that are 90% identical in amino acid sequence but exhibit different substrate preferences for dehydroepiandrosterone (DHEA), androsterone (AD), and cortisol (CS). ST-40 is active for all three substrates, whereas ST-20 is mainly active for cortisol. To determine the domain responsible for the substrate preferences of the HS-STs, 20 chimeric HS-STs were constructed by reciprocal exchanges of DNA fragments derived from the cDNAs and were expressed in Escherichia coli. Some chimeric enzymes were enzymatically active for all three substrates, and some displayed reduced or lost CS-ST activity, with retention of DHEA- and AD-ST activities. Others lost all HS-ST activity. Analysis revealed that a central region (region III spanning amino acids 102-164 with five amino acid differences between ST-20 and ST-40) is essential for HS-ST activity, whereas regions II (amino acids 65-101) and IV (amino acids 165-219) are unimportant with regard to substrate preference. It was also shown that the parental combination of regions I (amino acids 1-64) and V (amino acids 220-284) is essential for CS-ST activity. Photoaffinity labeling with [35S]3'-phosphoadenosine 5'-phosphosulfate (PAPS) revealed that some inactive chimeras lost affinity for PAPS. These results suggested that an ordered structure formed by regions I, III, and V is required for HS-ST activity, especially for substrate preference and PAPS binding.

Androsterone↗

High sulfotransferase activity for phenolic aromatic odorants present in the mouse olfactory organ.

Mouse nasal cytosols show high sulfotransferase (ST) activities toward phenolic aromatic odorants, but have little activities for most alcoholic aromatic odorants. Most ST activities toward the phenolic odorants preferred slightly acidic pH (6.4) and were sensitive to 2,6-dichloro-4-nitrophenol, a specific inhibitor for phenol ST (P-ST) but were not inhibited by triethylamine and tetra-n-butylammonium chloride, which are specific inhibitors for hydroxysteroid ST (HS-ST). These results suggested that P-ST activities are responsible for sulfation of the phenolic odorants. The spectra of the ST activities for these odorants were similar in mouse nasal and liver cytosols, however, nasal cytosols showed much higher ST activity toward cinnamyl alcohol than liver cytosols. This activity preferred higher pH (7.4) compared to the phenolic odorant-ST activities and was inhibited by both types of inhibitors, specific for P-ST and HS-ST. These results appear to indicate the participation of multiple ST isoforms for the sulfation of odorants in mouse nasal cytosols. The existence of P-ST(s) active for the phenolic odorants in olfactory cytosols suggests a role in odorant perception, in particular, in the signal termination process.

Alcohols↗

AP-1 transcriptional activity is regulated by a direct association between thioredoxin and Ref-1.

Thioredoxin (TRX) is a pleiotropic cellular factor that has thiol-mediated redox activity and is important in regulation of cellular processes, including proliferation, apoptosis, and gene expression. The activity of several transcription factors is posttranslationally altered by redox modification(s) of specific cysteine residue(s). One such factor is nuclear factor (NF)-kappa B, whose DNA-binding activity is markedly augmented by TRX treatment in vitro. Similarly, the DNA-binding activity of activator protein 1 (AP-1) is modified by a DNA repair enzyme, redox factor 1 (Ref-1), which is identical to a DNA repair enzyme, AP endonuclease. Ref-1 activity is in turn modulated by various redox-active compounds, including TRX. We here report the molecular cascade of redox regulation of AP-1 mediated by TRX and Ref-1. Phorbol 12-myristate 13 acetate efficiently translocated TRX into the HeLa cell nucleus where Ref-1 preexists. This process seems to be essential for AP-1 activation by redox modification because co-overexpression of TRX and Ref-1 in COS-7 cells potentiated AP-1 activity only after TRX was transported into the nucleus by phorbol 12-myristate 13 acetate treatment. To prove the direct active site-mediated association between TRX and Ref-1, we generated a series of substitution-mutant cysteine residues of TRX. In both an in vitro diamide-induced cross-linking study and an in vivo mammalian two-hybrid assay we proved that TRX can associate directly with Ref-1 in the nucleus; also, we demonstrated the requirement of cysteine residues in the TRX catalytic center for the potentiation of AP-1 activity. This report presents an example of a cascade in cellular redox regulation.

Animals↗

Expression analysis of mortalin, a unique member of the Hsp70 family of proteins, in rat tissues.

We have investigated the expression of mortalin in rat tissues by Northern analysis, RNA in situ hybridization, and immunohistochemical studies. By Northern assay, the highest level of expression was detected in brain, heart, and skeletal muscle followed by lung, liver, and kidney, and the least level of expression was detected in testis and spleen. RNA in situ and immunohistochemical studies showed that mortalin expression is significantly higher in upper nondividing layers than in the lower basal layers of skin, in neurons and nerve fibers than in surrounding glial cells in brain, in cardiomyocytes than in nonmyocytes in heart, and in interstitial secretory tissue than in germinating follicles in ovary. Such tissue- and cell-specific expression patterns of mortalin coordinates with its earlier reported antiproliferative function in normal cells. However, a deregulation of the expression is observed in rat brain tumor along with the detection of nonpancytosolic mortalin in rat glioma cell line C6. The study suggests that mortalin is involved in pathways that regulate division capacities of cells in vivo.

Animals↗

Heterogeneous zonal distribution of sulfotransferase isoenzymes in rat liver.

By employing dual-digitonin-pulse perfusion technique, the cytosolic fractions were prepared from periportal (PP) and perivenous (PV) hepatocytes of male and female rat livers. Sulfotransferase (ST) activities toward 2-naphthol (2NAP) (at pH 5.5 and 7.4), 4-nitrophenol, dehydroepiandrosterone (DHEA), and cortisol were measured in each fraction. DHEA-ST activity was mainly localized in PP fraction in males, while in females it was slightly higher in PP fraction than in PV samples. In contrast, phenol ST activities were higher in PV fraction in both sexes. With anti-HS-ST and anti-P-ST antisera, the levels of immunoreactive ST polypeptides were compatible with the levels of ST activities except that in female PP fraction the level of immunoreactive P-ST polypeptide was low in spite of comparatively high levels of P-ST activities. Chromatofocusing of PP and PV fractions separated P-ST activities into three major fractions (I-III), which have distinct catalytic and electrochemical properties. Fraction I was localized only in the PP samples in both sexes and revealed ST activities toward 2NAP at pH 5.5 and 7.4, while fraction II was localized in the PP and PV samples in both sexes with 2NAP-ST activity only at pH 7.4. Fraction III which had ST activities at pH 5.5 and 7.4 was present only in the PV samples in female rats, whereas in male rats it was present in both PP and PV samples. With anti-P-ST antiserum, the immunoreactive polypeptide was present in fraction III, but no immunoreactive band was detected in fractions I and II, suggesting the presence of immunochemically and electrochemically different P-ST(s) in these fractions.

Age Factors↗

Aberrant immunity in the central nervous system in relation to disease progression in HAM/TSP.

The immunological status of the central nervous systems of 19 patients with HTLV-I-associated myelopathy/tropical spastic paraparesis (HAM/TSP) was distinct from that of 6 asymptomatic HTLV-I carriers. Cross-sectional analysis of the time course of disease-related abnormalities in the cerebrospinal fluid (CSF) showed that activated B cell function was a feature in relatively early HAM/TSP patients in whom duration of the disease was less than 5 to 6 years. During this period many patients experienced notable neurological deterioration. By contrast, an increase in CD8+CD11a+ cytotoxic T lymphocytes along with elevated beta 2-microglobulin levels in the CSF was a consistent finding in early as well as late patients with more than a 10-year history of the illness. In light of the generally progressive course of this disorder, the mode of immunity related to the pathogenesis of HAM/TSP may be different according to the stages of the disease.

Adult↗

Saccadic eye movements and regional cerebral blood flow in schizophrenic patients.

This study examined saccadic eye movements, using simple stationary targets, in schizophrenic patients. The targets were eight black points or eight arabic-numbered points placed in randomized order on the circumference of a circle. Self-paced eye movements during clockwise tracking of these points, by 23 patients and 23 controls, were recorded using an infrared eye-mark recorder. Then the relationship between the saccades and clinical symptoms was investigated. Finally, the relationship between the performance of the saccades and resting regional cerebral blood flow (rCBF) was examined using single photon emission computed tomography with 99mTc-hexamethyl propyleneamine oxime (HMPAO). The results indicate that patients track with significantly fewer correct scores and more deviant scores than controls, in agreement with our previous study. There were two groups of patients: an ordinary group who obtained a full-target-hitting score at a 200-ms setting and a fast group who obtained the full score at 100 ms but not at 200 ms. Some patients displayed significantly more hypermetria than controls. Significant correlations were found between hallucination and delusion symptoms and correct score. With respect to relative rCBF, fast-group patients showed significantly decreased rCBF in the left limbic and inferior parietal areas as compared with ordinary group patients. These findings suggest that some schizophrenic patients view the stationary targets too fast and this may be related to dysfunction in the limbic-parietal association area in the left hemisphere.

Adolescent↗

Abutiloside A, a 26-acylamino-3 beta, 16 alpha-dihydroxy-5 alpha-cholesta-22-one glycoside from Solanum abutiloides.

In addition to solamargine and proto-dioscin, three new steroid glycosides, abutilosides A-C, have been isolated from roots of the Solanaceae Solanum abutiloides. The structure of abutiloside A has been elucidated as 3 beta, 16 alpha-dihydroxy-26-isovalerylamino-5 alpha,25 xi H-cholestan-22-one 3-O-[O-beta-D-xylopyranosyl-(1-->2)-O-alpha-L- rhamnopyranosyl-(1-->4)-beta-D-glucopyranoside]. De-N-acylation of its aglycone yielded solafloridine by 22,N-cyclization. Therefore, 26-aminocholestan-22-one derivatives are considered to be crucial intermediates in steroid biosynthesis. The co-occurrence of the (25R)-steroids soladulcidine, solafloridine and diosgenin together with abutiloside A in roots of S. abutilosides suggests that the configuration of abutiloside A at C-25 will also be R.

Carbohydrate Conformation↗

Stimulation of the development of bovine embryos by insulin and insulin-like growth factor-I (IGF-I) is mediated through the IGF-I receptor.

To study the effects of insulin and insulin-like growth factor-I (IGF-I) on the development of bovine embryos, fertilized bovine embryos in vitro were cultured in a chemically defined, protein-free medium: modified synthetic oviduct fluid (mSOF) supplemented with 1 mg/ml polyvinyl alcohol. Dose-response studies showed that insulin (0.5 to 10 microg/ml) and IGF-I (2 to 200 ng/ml) stimulated the development of bovine embryos to the morula stage 5 d after in vitro fertilization. The addition of 0.5 microg/ml insulin or 2 ng/ml IGF-I to the mSOF had beneficial effects on embryonic development to the morula stage in the presence of amino acids, but insulin and IGF-I did not affect the development of bovine embryos to the morula stage in the absence of amino acids. The antiIGF-I receptor antibody (alphaIR-3) completely blocked the stimulation of development to the morula stage by insulin and IGF-I. These findings suggest that the stimulation of embryonic development by insulin and IGF-I is mediated through the IGF-I receptor.

Journal Article↗

Characterization of 26S proteasome alpha- and beta-type and ATPase subunits from spinach and their expression during early stages of seedling development.

Three kinds of cDNAs encoding 26S proteasome subunits have been cloned from spinach (Spinacia oleracea L.). These genes, designated as SOPSC8, SOPSC1 and SOPRS7, encode an alpha-type and a beta-type subunit of the 20S catalytic core, and an ATPase subunit of the 19/22S regulatory complex, respectively. The deduced protein sequences showed high sequence similarities to other proteasome alpha- and beta-type and ATPase subunit proteins. Southern blot analysis indicates that there are additional members of these dispersed proteasome families in the spinach genome. These three subunit genes are expressed simultaneously during germination and reach a maximum one day after sowing followed by a decline. The expression of these genes also increases during cotyledon senescence.

Adenosine Triphosphatases↗

Aesthetic fingertip reconstruction with a free vascularized nail graft: a review of 60 flaps involving partial toe transfers.

Microsurgical toe transfer is an established procedure for functional reconstruction of fingers. However, even if the functional loss is minimal, the fingertip defect is often a large problem for patients for not only functional reasons but also aesthetic reasons. In these patients, although the normal appearance of the fingertip is very important, total toe transfer is not acceptable because of resection of an entire toe. With this background, partial toe transfer techniques have greatly progressed. On the other hand, various types of innervated finger flaps also have been developed in hand surgery. Based on this progress, we developed the combined technique of innervated finger flaps in the hand and osteo-onychocutaneous flaps from the toe. This technique provides better aesthetic results in fingertip reconstruction, thus broadening the indications for vascularized nail grafts. We have now experienced 60 flaps in partial toe transfer. In this report, a review of 60 consecutive flaps is presented, and the indications, technique, and postoperative treatment are discussed.

Adult↗

Smoking and neovascular form of age related macular degeneration in late middle aged males: findings from a case-control study in Japan. Research Committee on Chorioretinal Degenerations.

AIMS: The risk of smoking habits for developing the neovascular form of age related macular degeneration (neovascular form of AMD) were studied by a case-control study in Japan. METHODS: 56 male patients with the neovascular form of AMD and 82 healthy male controls, aged 50 to 69 years, were enrolled. A self administered questionnaire provided necessary information for the study subjects. Questions on smoking included whether the study subjects have ever smoked or not, and if smoked, depth of smoke inhalation, use of extra filter, age at starting smoking, average number of cigarettes smoked per day, and duration of smoking. When a smoker had stopped smoking, age at cessation was also recorded. Unconditional logistic analysis was adapted to calculate age adjusted odds ratios and their 95% confidence intervals (CIs) for smoking related factors. RESULTS: Age adjusted odds ratio of developing the neovascular form of AMD was 2.97 (95% CI 1.00-8.84) for current smokers and 2.09 (0.71-6.13) for ex smokers, compared with non-smokers. All smoking habit/smoking history related variables such as use of extra filter, smoke inhalation level, age at starting smoking, duration of smoking, and Brinkman index were found to be significantly related to an increased risk of the neovascular form of AMD. CONCLUSIONS: Suggested is the strong possibility that cigarette smoking enhances the neovascular form of AMD risk in late middle aged males, though the magnitude of risk by smoking variables might be overestimated, in part, because of health oriented controls.

Aged↗

Homozygosity for an allele carrying intermediate CAG repeats in the dentatorubral-pallidoluysian atrophy (DRPLA) gene results in spastic paraplegia.

We report a family with autosomal recessive spastic paraplegia. Patient 1 was a 37-year-old woman and patient 2 was her 35-year-old sister. They showed spastic paraplegia with mild truncal ataxia and dysarthria but no dementia, epilepsy, myoclonus, or other involuntary movements. They were the products of a consanguineous marriage but the parents were neurologically normal. We analyzed the CAG repeats of the dentatorubral-pallidoluysian atrophy (DRPLA) gene in the family members. The patients were homozygous for an allele carrying an intermediate size of CAG repeats (41 or 40 repeats) in the DRPLA gene; the parents were heterozygous for an intermediate allele and a normal allele in this gene. Homozygosity for an intermediate allele in the DRPLA gene appears to have resulted in spastic paraplegia different from any DRPLA phenotype.

Adult↗