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Biomedical subjects

Y Eto

Publications and source records attributed to Y Eto.

At least 235 records · Page 13Linked to original sources
PubMed1986

Regulation of galactocerebroside and sulfatide synthesis by hormones in chick neural cultures.

Chick neural cultures were used to study effects of insulin, thyrotropin releasing hormone, growth hormone and glucagon on myelin lipid synthesis in vitro. The incorporation of [3H]galactose into myelin associated lipids such as cerebroside and sulfatide was used as an index for various hormonal effects on myelination. The data suggest that these hormones were effective on myelin lipid synthesis only in the central nervous cells, not in the peripheral nerve cells.

Animals↗
PubMed1986

4-Methylumbelliferyl lipase in human and mouse brain: a possible localization in myelin.

4-Methylumbelliferyl (4-MU) lipase activity in human and mouse brain, measured with 4-MU palmitate, was characterized with respect to effects of pH and detergents, and subcellular and myelin localization. Purified myelin isolated by Norton's procedure [J. Neurochem. 21, 749-757 (1983)] contained higher specific activity of 4-MU lipase, particularly in alkaline pH. Myelin lipase activity was markedly affected by the addition of different types of detergents, the amount of detergents added, and substrate. The optimal pH in myelin was bimodal--pH 4.5 and up to 8.0, respectively. These data indicate that myelin possesses 4-MU lipase activity at alkaline pH, with lower levels at acidic pH.

Animals↗
PubMed1986

Infective endocarditis in children: a current diagnostic trend and the embolic complications.

Twenty-three cases of IE, of which 91% had underlying congenital heart diseases were diagnosed by positive blood culture or vegetative lesion detected by 2-D ECHO when a patient revealed clinically suspicious episodes. VSD and TOF were the most common underlying heart diseases. The vegetation was detected successfully by 2-D ECHO in 91% of the cases. The causative organisms were identified in 83% of the cases. Streptococcus viridans and Staphylococcus aureus were the two major organisms detected. Candida albicans was found in 3 postoperative and fatal cases. Embolic complications occurred in 7 cases (30.5%): five cases of pulmonary infarction and two of cerebral infarction. The overall mortality was 22%. We recommend early surgical intervention whenever a patient reveals a very large vegetation with animated movability, and emphasize the importance of a proper prophylaxic regimen.

Adolescent↗
PubMed1986

Abnormal excretion of autofluorescent lipids in urine from patients with neuronal ceroid lipofuscinosis.

Two of our 5 patients with neuronal ceroid lipofuscinosis excreted a large amount of autofluorescent lipids in their urine. These autofluorescent lipids consisted of more than 5 bands in the thin-layer-chromatography. Excitation and emission spectra of these lipids showed that they had heterogeneous components. This finding was specific to neuronal ceroid lipofuscinosis, and never found in other neurological diseases. We also measured unesterified dolichol and dolichol fatty acid ester in urine, and 4 of our patients did not excrete a large amount of such compounds compared with control groups. This finding suggest that increased excretion of dolichol in urine of patient with neuronal ceroid lipofuscinosis may be the secondary effect, but there are some possibilities that certain abnormalities in glycoprotein metabolism exist in neuronal ceroid lipofuscinosis.

Chromatography, Thin Layer↗
PubMed1986

[Carnitine deficiency: a treatable cardiomyopathy].

This is a report of two brothers, six and five years of age, with systemic carnitine deficiency and cardiomyopathy, whose symptoms were improved after oral administrations of DL-carnitine. They had had progressive muscle weakness since three years of age. The elder brother's radiograph on admission showed cardiomegaly with a cardiothoracic ratio of 60%, and his electrocardiogram showed left ventricular hypertrophy and tall, peaked T waves in the precordial leads. The echocardiogram showed slight thickening of the cardiac muscle and decreased ejection fraction. Skeletal muscle biopsy specimens and sera were assessed for carnitine content. The skeletal muscle specimens revealed lipid storage myopathy, and the carnitine contents of the skeletal muscle and sera were both decreased. Myocardial biopsy for the elder brother revealed mitochondrial accumulation. Cardiomyopathy caused by carnitine deficiency is often fatal, but may be cured. Carnitine deficiency should be considered whenever a patient with cardiomegaly and progressive skeletal muscle weakness is encountered.

Cardiomyopathies↗
PubMed1985

Enzyme replacement with liposomes containing beta-galactosidase from Charonia lumpas in murine globoid cell leukodystrophy (twitcher).

Enzyme replacement with liposomes containing beta-galactosidase obtained from charonia lumpas was carried out in murine globoid cell leukodystrophy (GLD). Charonia lumpas beta-galactosidase was able to hydrolyze galactocerebroside trapped into liposomes prepared from lecithin, cholesterol and sulfatide (molar ratio; 7:2:1). Liposomes containing charonia lumpas beta-galactosidase were successfully incorporated into the mouse tissues. 3H-galactocerebroside labeled liposomes were also incorporated into mouse liver, spleen and other tissues. The accumulation rate of 3H-galactocerebroside into twithcer mice liver and spleen was almost 40 to 100 times higher than those of controls and degraded to 70 to 80% of accumulated radioactivity of 3H-galactocerebroside by single injection of liposomes containing charonia lumpas beta-galactosidase. Results suggest that exogeneous enzyme trapped in liposomes can be useful for the correction of accumulated compound.

Animals↗
PubMed1985

Increased urinary excretion of acid mucopolysaccharides and glycopeptides in hypothyroidism following thyroid hormone therapy.

Urinary excretion of acid mucopolysaccharides and glycopeptides in the urine of an untreated patient with cretinism were measured before and after thyroid hormone replacement. Urinary uronic acid and hexose excretion in the CPC-precipitated fraction increased four to ten times after thyroid hormone administration. The maximum excretion was observed after 2 months of thyroid replacement. The excreted acid mucopolysaccharides consisted of chondroitin sulphate A/C and minor quantities of heparan sulphate. Urinary excretion of glycopeptides, particularly small molecular glycopeptides rose also four to five-fold with thyroid hormone administration. These data suggest that thyroid hormone markedly influences the metabolism of acid mucopolysaccharides and glycoproteins. Possibly, the lack of thyroid hormone caused a decreased activity of various lysosomal glycosidases and sulphatases.

Child↗
PubMed1985

Neuropathologic study in the heterozygotes of X-linked brindled mutant mouse.

The clinical and morphological features were studied in female heterozygotes of the sex-linked brindled mutant mice, which are an appropriate animal model for human Menkes' kinky hair disease (MKHD). Clinically, female heterozygotes presented phenotypical variety. In these heterozygotes, we distinguished the unique group of mice, which showed mottled white and dark brown fur and curly whiskers. We designated this unique group "heterozygote, variant type", in contrast to the remaining group--"heterozygote, usual type"--, of which the fur was mottled dark and light brown, and the whiskers were straight. Ultrastructurally, various degrees of mitochondrial changes, from an almost normal appearance of the mitochondria to similar to those of the hemizygotes, were observed. Furthermore we noticed that, in the heterozygotes, there were positive correlations between this morphological spectrum and those phenotypical varieties. These findings were interpreted as a possible subclinical copper deficiency in the heterozygotes, and the morphological alterations in heterozygotes were probably due to X-chromosome inactivation according to Lyon's hypothesis. The presence, however, of clinical and morphological varieties in the heterozygotes leads us to the hypothesis that the inactivation rate is not necessarily the same for all carriers. Moreover, it can be speculated that pathologic changes similar to those in heterozygotes may be present in the female carriers of human MKHD.

Animals↗
PubMed1985

Pathochemical study of a case of hereditary cerebral degenerative disease--lipopigment accumulation and peroxidase deficiency in brain.

Ultrastructural study of autoptic cerebral tissue from a patient with hereditary degenerative disease of the nervous system revealed lipofuscin-like bodies and variable sized osmiophilic granular masses in neurons. Autofluorescent granules were also found in neurons. Biochemical study demonstrated a deficiency of diaminobenzidine peroxidase and the accumulation of lipopigment in cerebral tissue.

Brain↗
PubMed1985

[Intracoronary thrombolytic therapy in Kawasaki disease and the usefulness of two-dimensional echocardiography in detecting intracoronary thrombi].

The main cause of death in patients with Kawasaki disease is myocardial infarction due to thrombotic occlusion of a coronary aneurysm. Intracoronary thrombolytic therapy was administered to dissolve the intracoronary thrombi of one infarcted patient and five non-infarcted patients who had massive intracoronary thrombus formations which were detected by two-dimensional echocardiography (2-D echo). Intracoronary injections of Urokinase ranged in dose from 2000 to 240000 IU. Systemic Urokinase infusions were performed for two patients in addition to intracoronary injections. Coronary angiography revealed complete obstruction of coronary aneurysms in two patients and partial obstruction in one patient. Although coronary angiography failed to visualize the intracoronary thrombi in three patients, 2-D echocardiography imaged massive thrombus formations in coronary aneurysms. Partial but significant coronary arterial recanalization was achieved after injecting Urokinase in a patient with myocardial infarction. Complete resolution of massive intracoronary thrombi was observed in three of five patients using 2-D echocardiography. A decrease in size of the intracoronary thrombus in one patient was observed after thrombolytic therapy. In another patient, the size of a thrombus did not change after thrombolytic therapy. Recurrence of thrombus formation in coronary aneurysms was observed in three patients using serial 2-D echocardiography. Urokinase was readministered to them and one showed significant reduction in the thrombus size. We conclude that 1) 2-D echocardiography is more sensitive and reproducible than coronary angiography. Therefore, serial 2-D echocardiography should be performed for patients with Kawasaki disease to detect intracoronary thrombus formation and to evaluate serial changes in thrombi. 2) Intracoronary thrombolytic therapy is useful for patients who have intracoronary thrombi to treat or prevent myocardial infarction.

Aneurysm↗
PubMed1984

Canine GM2-gangliosidosis: chemical and enzymatic features.

The chemical and enzymatic features in tissues of GM2-gangliosidosis are characterized by the analysis of glycolipids and FD-MS, and also by enzymatic analysis with DEAE-Sepharose column chromatography. The results suggest that canine GM2-gangliosidosis is equivalent to human juvenile GM2-gangliosidosis.

Animals↗