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Biomedical subjects

E Satoyoshi

Publications and source records attributed to E Satoyoshi.

At least 55 records · Page 3Linked to original sources

Autosomal recessive distal muscular dystrophy: a comparative study with distal myopathy with rimmed vacuole formation.

To clarify the clinical and morphological characteristics of distal muscular dystrophy, clinical and pathological material from 4 affected persons was compared with similar studies in 4 patients with distal myopathy with rimmed vacuole formation. Although these two forms of autosomal recessive distal myopathy with onset in young adulthood were highly similar in their clinical symptoms, histochemical and electron microscopic findings of muscles subjected to biopsy were quite different. The muscle abnormalities in distal muscular dystrophy were almost the same as those in Duchenne muscular dystrophy, showing massive fiber necrosis followed by active fiber regeneration. In contrast, distal myopathy with rimmed vacuole formation showed a progressive muscle fiber atrophy and loss, rimmed vacuoles in the sarcoplasm, and no apparent fiber necrosis or regeneration.

Adolescent↗

Effects of thymic myoid cell culture supernatant on cells from lymphatic tissues.

Conditioned media (MCM) of cloned thymic myoid cells (IT45R92, R613Ad, and R615B2) were used to investigate their possible involvement in thymic biological events. Those myoid cells produced in a culture medium biological activities capable of stimulating the growth of thymocytes, spleen cells, and bone marrow cells of mice and rats. Surface markers detected on spleen cells proliferating in MCM were characteristic of monocyte-macrophage lineages (C3R, Fc gamma R, asialo GM1) and T-cell lineages (Thy 1) but not B cells (sIgG). Chromatographic studies also suggested that the biological activities of MCM could be separated into two different molecular entities, such as a colony-stimulating activity and an interleukin 1-like activity which supported the growth of monocyte-macrophage lineages and T-cell lineages, respectively. These results indicate that thymic myoid cells produce cytokines important for the regulation of intrathymic interleukin cascade by which clonally differentiated thymic lymphocytes may be expanded into a sizable pool.

Animals↗

Glabella tap sign. Is it due to a lack of R2-habituation?

In 30 patients with Parkinson's disease, 55 patients with other neurological disorders and 25 normal subjects, both upper eyelid movements and orbicularis oculi reflexes to repetitive glabella taps were simultaneously recorded using a newly devised apparatus for the measurement of eyelid movement. Upper lid movement during the blink reflex has been thought to correspond to the late component of the two components of the orbicularis oculi reflex, and failure of habituation of the late component to repetitive stimuli has been considered to be responsible for the glabella tap sign. However, the present study showed that the eyelid lowered after the early component (R1), and habituation of the late component (R2) was recognized in 31% of subjects with the glabella tap sign. This shows that there is no direct causal relationship between the glabella tap sign and lack of the habituation of the late component.

Adolescent↗

Reduction of acid sphingomyelinase activity in human fibroblasts induced by AY-9944 and other cationic amphiphilic drugs.

AY-9944 (trans-1,4-bis(2-chlorobenzylaminoethyl)cyclohexane dihydrochloride), a cationic amphiphilic drug, caused a rapid, irreversible and dose-dependent reduction of acid sphingomyelinase activity in normal human fibroblasts without changing the activities of other lysosomal hydrolases tested. Examinations of activities against synthetic substrates and of the pH-dependency of sphingomyelinase in the drug-treated cells also suggested that the reduction of activity was specific to acid sphingomyelinase. Such a specific reduction was also found with 12 other cationic amphiphilic drugs, most of which have been shown to be inducers of experimental phospholipidosis in animals and/or cultured cells. These results strongly suggest that acid sphingomyelinase is involved in the process of drug-induced lipidosis. The reduction of acid sphingomyelinase seemed not to be due to direct inhibition by these drugs, a specific loss of the enzyme into the culture medium, the presence of inhibitor in the drug-treated cells, or impaired synthesis of the enzyme. There was no indication that changes in the catalytic properties of the enzyme, or changes in the requirement of detergents for its activity occurred in the cell. These results suggest that AY-9944 and other cationic amphiphilic drugs may cause the reduction of acid sphingomyelinase activity by inducing an increased rate of degradation of the enzyme or by causing an irreversible inactivation via some undetected factor.

Cations↗

Nerve growth factor secreted by mouse heart cells in culture.

Mouse heart cells in culture synthesized and secreted a nerve growth factor (NGF) which cross-reacted with mouse submaxillary gland beta NGF. The synthesis of NGF by heart cells was greatly reduced by addition of actinomycin D or cycloheximide but not by cytosine arabinoside, suggesting that the synthesis of NGF by heart cells required DNA transcription but not DNA replication. Heart NGF and beta NGF were virtually equivalent with respect to the neurite outgrowth stimulating activity and immunologically indistinguishable. The molecular weight of heart NGF estimated by the gel filtration method was identical with that of beta NGF. Isoelectric point of heart NGF was about 9.5, like beta NGF. These results suggest that NGF synthesized and secreted by mouse heart cells is an identical molecule to beta NGF of the mouse submaxillary gland.

Animals↗

Neoplastic angioendotheliosis of the central nervous system.

A case of neoplastic angioendotheliosis demonstrating unusual manifestations is reported. A 56-year-old male showed recurrent attacks of neurological symptoms including paraplegia, brain-stem symptoms, tonic seizures, aphasia, apraxia and cortical blindness over 2 years. The EEG disclosed transient, periodic, lateralized, epileptiform discharges. Brain CT scan revealed low-density areas mainly in the white matter. Other laboratory examinations were negative except for CSF protein fractions. Post-mortem examination disclosed remarkable intravascular proliferation of atypical cells in the CNS with prominent proliferation of blood vessels and softening. Other organs were not affected, which suggested that the atypical cells had a high affinity to CNS blood vessels.

Brain↗

Clonal heterogeneity of thymic muscle-cell precursors.

Three myoid-cell clones were established from the thymuses of two Wistar rats; one thymus yielded two clones, R615A and R615B2, and the other yielded one clone, R613Ad. The three clones were divided into two subtypes. Both subtypes were able to form myofibrils, expressed AChR on their cell-surface membrane, and contained myofibrillar ATPase characteristic of undifferentiated type-2C fibers, but they differed from each other in morphology, expression of Thy 1 antigen, spontaneous contractility, and qualitative accumulation of AChR. Immunolocalization studies using antisera against the respective cell subtypes also indicated regional differences in their cellular origin. These results show that the thymus contains heterogenous myoid-cell precursors.

Animals↗

Low energy levels in thiamine-deficient encephalopathy.

Pyrithiamine-induced acute thiamine-deficient encephalopathy was produced in adult male Wistar rats. Twenty-four hours before the onset of neurological signs the brain showed no morphological abnormalities. Encephalopathic rats had symmetrical lesions of edematous necrosis localized in the thalamus, mammillary body, and pontine tegmentum. Biochemically, encephalopathic rats had brain thiamine levels less than 20% of controls. For the assay of the concentrations of adenosine triphosphate (ATP) and phosphocreatine, the brains were fixed using 5 KW microwave irradiation and were divided into four parts: cerebral cortex, diencephalon, lower brainstem, and cerebellum. In the lower brainstem of the encephalopathic rats ATP concentrations were 89.5% of normal controls. Phosphocreatine levels were lowered to 70% of controls in the diencephalon and to 75% in the lower brainstem. Total high energy phosphate levels were decreased to 89% of controls in the diencephalon and 91% in the lower brainstem before the onset of neurological signs and to 76% and 79%, respectively, after the onset. In the cerebral cortex and cerebellum high energy phosphates were not significantly reduced. Lower high energy phosphate levels and the distribution of edematous lesions were coincident in the brain. These findings suggest that a low energy state is closely related to the formation of edematous lesions in thiamine-deficient encephalopathy.

Animals↗

Triiodothyronine (T3) toxicosis with hypokalemic periodic paralysis.

A case of triiodothyronine (T3) toxicosis associated with hypokalemic periodic paralysis is reported. Thyrotoxic manifestation was minimal except for mild struma. Serum T3 levels were moderately elevated, though other thyroid function tests were within the normal range. The patient was treated with methimazole, and paralytic attacks ceased. This is the first report of this combination and the necessity of careful thyroid function tests in sporadic periodic paralysis is emphasized.

Adult↗

Action-induced rhythmic dystonia: an autopsy case.

We studied a patient with action-induced rhythmic dystonia that followed a stroke. Postmortem studies showed an infarct in the right posterolateral ventral part of the thalamus. Electrophysiologic analysis indicated that the eliciting factor of the involuntary movement was an impulse, promoting voluntary contraction of muscle. CSF 5-HIAA content was low, and HVA was high. Administration of 5-HTP and clonazepam abolished the involuntary movements.

5-Hydroxytryptophan↗

Idiopathic hyperCKemia.

In three adult men, serum creatine kinase activity was constantly raised for at least 4 years. They had been normal in other neuromuscular functions and did not have any established disease. Quantitative morphologic and pharmacologic studies were performed on biopsied muscle. The biceps brachii of patient 1 contained 0.3% necrotic fibers. In patient 2, only slight variation of muscle fiber diameter was noted. Muscle of patient 3 contained a few small angular fibers, and 11% of fibers exhibited internal nuclei. Sensitivity to caffeine in vitro was increased in patients 2 and 3, as seen in survivors of malignant hyperthermia; patients in hyperCKemia may be susceptible to malignant hyperthermia.

Adult↗

The Crow-Fukase syndrome: a study of 102 cases in Japan.

Clinical manifestations of 102 cases with the Crow- Fukase syndrome (the syndrome of polyneuropathy, anasarca, skin changes, endocrinopathy, dysglobulinemia, and organomegaly), with or without myeloma, were reviewed. Fifty-six cases with myeloma consisted of 31 with osteosclerotic, 17 with mixed osteosclerotic and osteolytic, and 8 with osteolytic. Forty-six cases without myeloma consisted of 2 with extramedullary plasmacytoma, 33 with M protein alone, and 11 with polyclonal protein alone. There was no significant difference in incidence of the major clinical manifestations between the two groups with and without myeloma. They had a common characteristic histologic finding of the lymph node resembling that of Castleman's disease.

Adult↗

Abnormalities of erythrocytes in Duchenne muscular dystrophy.

Erythrocytes from patients with Duchenne muscular dystrophy (DMD) were studied in relation to the extracellular calcium ion concentration. The shape of fresh erythrocytes in DMD was normal and not affected by Ca++. When DMD erythrocytes were stored in buffer solution with Ca++, however, the rate of the shape change over time was enhanced compared with that of cells from normal controls. The calcium content of DMD erythrocytes stored in buffer solution with Ca++ and dextrose was also two and one-half times that of control cells. In contrast, when DMD erythrocytes were stored in buffer solution with dextrose but without Ca++, no differences from controls were observed. The adenosine triphosphate content of DMD erythrocytes stored in the buffer solution was not affected by Ca++ concentration.

Adenosine Triphosphate↗

A highly sensitive enzyme immunoassay for mouse beta nerve growth factor.

A sensitive two-site enzyme immunoassay system for mouse beta nerve growth factor (NGF) was developed, based on the sandwiching of the antigen between anti-mouse beta NGF antibody IgG coated to a polystyrene tube and anti-mouse beta NGF antibody Fab'-linked beta-D-galactosidase (beta-D-galactoside hydrolase, EC 3.2.1.23). This method has the following advantages: (a) the procedures are simple and rapid compared to bioassay or two-site radioimmunoassay; (b) antibody Fab'-beta-D-galactosidase complex is more stable than 125I-labeled antibody; (c) purified beta NGF is detectable at a concentration as low as 10 pg/ml. Our enzyme immunoassay was used to examine the levels of NGF in some tissues of mice. The submaxillary gland contained a high concentration of NGF. However, other tissues, such as the heart, brain, and skeletal muscle, and serum did not contain detectable NGF. These results support recent findings by other investigators that NGF was not found in the organs/tissues other than the submaxillary gland of mice.

Animals↗