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Biomedical subjects

I Goto

Publications and source records attributed to I Goto.

At least 181 records · Page 10Linked to original sources
PubMed1988

Free sphingoid bases in normal murine tissues.

Free sphingoid bases, which have been considered not to occur naturally, were detected in murine tissues by derivatization with o-phthalaldehyde and the use of high-performance liquid chromatography. The concentrations were 10-30 pmol/mg tissue. The lung contained the largest amounts of sphingoid bases. In the molecular species of sphingoid bases, the most abundant was C18-sphingenine followed by C18-sphinganine, 4-hydroxysphinganine and C20-sphingenine, in that order. The central nervous tissues contained relatively high amounts of C20-sphingenine and there was a high concentration of 4-hydroxysphinganine in the kidney. In addition, galactosylsphingenine was detected simultaneously in the spinal cord and sciatic nerve. Sphingoid bases were purified from normal murine lungs using lipid-extraction, cation-exchange and silicic acid column chromatographies, alkaline saponification and preparative thin-layer chromatography. In the purified sphingoid bases, erythro-C18-sphingenine and erythro-C18-sphinganine were identified using thin-layer chromatography, high-performance liquid chromatography and fast-atom-bombardment mass spectrometry. Free sphingoid bases occurring in normal tissues may be metabolic intermediates required for the synthesis or be products of degradation of the sphingolipids and function to regulate cellular metabolism.

Animals↗
PubMed1988

Amyloid beta-protein gene duplication is not common in Alzheimer's disease: analysis by polymorphic restriction fragments.

The amyloid beta-protein(BP) is an important component of amyloid fibrils of both Alzheimer's disease(AD) and adult Down syndrome(DS). It has been hypothesized that sporadic AD may involve the duplication of a subregion of chromosome 21 containing the BP locus. However, an improved method for detection of the BP gene duplication using polymorphic Hind III fragments led us to a conclusion that BP gene duplication is rare, if any, in (Japanese) sporadic AD patients, indicating that the duplication of the BP gene itself is not the common underlying genetic defect in AD.

Adolescent↗
PubMed1988

Infantile and fetal globoid cell leukodystrophy: analysis of galactosylceramide and galactosylsphingosine.

Galactosylceramide and galactosylsphingosine (psychosine) were assayed in tissues from infants and fetuses with globoid cell leukodystrophy (GLD). Galactosylceramide concentrations were not increased in nervous tissues or other organs. Using a sensitive assay method, we found galactosylsphingosine accumulations in GLD tissues, both infantile and fetal, which suggests that GLD is a generalized galactosylsphingosine storage disease. High galactosylsphingosine levels were observed in the brain, spinal cord, and sciatic nerve of infants with GLD and in the spinal cord of a fetus with GLD, where lesions characteristic to GLD were noted. In tissues without morphological changes, such as somatic organs and the brain in fetal GLD, galactosylsphingosine concentrations were low. These results suggest that a close relationship exists between galactosylsphingosine accumulation and the pathogenesis of GLD. The finding that galactosylsphingosine, but not galactosylceramide, accumulates in the tissue of GLD can be explained by our previous observation that galactosylceramide, but not galactosylsphingosine, is readily hydrolyzed by an intact galactosylceramidase II, which is genetically distinct from galactosylceramidase I.

Brain↗
PubMed1988

Relationship between surface marker expression and encephalitogenic potency of BP-cultured lymphocytes.

The relationship between surface marker expression and encephalitogenicity of lymphocytes from various lymphoid organs of Lewis rats was studied. The encephalitogenicities after culture with BP were spleen cells greater than lymph node cells much greater than thymus cells, in this descending order. The cells from every lymphoid organ proliferated significantly in response to BP. In spleen and lymph node cells, the expression of W3/25 and OX-3 molecules on T cells increased markedly after culture with BP, but the expression of OX-19 or OX-8 molecules did not change significantly. The up-regulations of W3/25 and OX-3 molecules were more pronounced in spleen cells than in lymph node cells. Thymus cells also showed a significant increase in the W3/25 molecule after the culture with BP. Therefore, T cells from all the lymphoid organs showed a selective up-regulation of the W3/25 molecule after culture with BP, and the degree of the up-regulation seems to correspond to the encephalitogenic potency in vivo. Since the W3/25 molecule apparently plays a direct role in the effector phase of experimental allergic encephalomyelitis (EAE) by enhancing BP-reactive T cell/antigen-presenting cell interaction in the central nervous system, the up-regulation on BP-cultured T cells may strengthen interaction with the class II major histocompatibility complex molecule on antigen-presenting cells, and therefore, contribute to the efficient transfer of EAE.

Animals↗
PubMed1988

Early morphological changes in the striated muscles in normal and dystrophic chickens.

Histological and histochemical analyses were performed on the anterior latissimus dorsi muscle (ALD, red muscle) and the posterior latissimus dorsi muscle (PLD, white muscle) in normal (line 412) and dystrophic chickens (line 413) from 19 day embryos to 6 weeks of age. PLD, the white muscle, in dystrophic chickens showed higher percentages of red and intermediate fibres than those of normal chickens during the early development of muscles. Increases of the oxidative enzyme activities and the numbers of NADH--TR formazan granules in the white fibres of PLD were already found at 1 week of age in dystrophic chicken. Fibre types, oxidative enzyme activities and NADH--TR formazan granules showed no differences in ALD between normal and dystrophic chickens. These results suggest that increases of oxidative enzyme activities and formazan granule numbers and incomplete fibre type differentiation in PLD of dystrophic chickens are early pathological processes in such birds.

Animals↗
PubMed1988

Systemic triglyceride storage disease with normal carnitine: a putative defect in long-chain fatty acid metabolism.

A 45-year-old Japanese man presented with lipid storage myopathy, fatty liver, cardiomyopathy, vacuolated leukocytes (Jordans' anomaly) and perceptive deafness. His parents were consanguineous and his younger sister was also affected. Histopathological and biochemical studies revealed an abnormal accumulation of triglyceride in muscle, liver, leukocytes, gastrointestinal endothelial cells and cultured skin fibroblasts. On electron microscopy, the vacuoles lacked limiting membranes and were adjacent to the mitochondria. Total and free carnitines in muscle were normal levels. Production rate of 14CO2 or acid-soluble [14C]metabolites from [1-14C]palmitate in the patient's cells was decreased to about 50% of that in control cells, whereas that from [1-14C]butyrate was normal. Long-chain fatty acyl esterase activities in the patient's leukocytes were normal at both pH 4.0 and pH 8.0. Despite the strong suggestion of an impaired metabolism of long-chain fatty acids, there were no evidences of abnormalities in carnitine metabolism or uptake of fatty acids into cells. The disorder is clinically different from defects in carnitine metabolism, defects in the carnitine-acylcarnitine translocase system or in mitochondrial beta-oxidation enzymes. Although the underlying metabolic defect has not been elucidated, this disease seems to be an autosomal-recessively inherited disorder of systemic triglyceride storage, probably due to an impaired regulation of lipolysis and triacylglycerol synthesis.

Carnitine↗
PubMed1988

Leukoencephalopathy in HTLV-I-associated myelopathy: MRI and EEG data.

Magnetic resonance imaging (MRI) of the brain and electroencephalography (EEG) were carried out for patients with human T-lymphotropic virus type I (HTLV-I)-associated myelopathy (HAM). Nineteen of 22 patients (86%) had abnormalities on brain MRI and/or EEG. Thirteen patients showed high signal intensity lesions scattered in the cerebral white matter, as seen on the T2-weighted MRI images. The lesions were mostly located in the subcortical and deep cerebral white matter and were not contiguous with the lateral ventricles. Patients with abnormalities on the brain MRI had a longer duration of disease and a greater disability than did those without MRI abnormalities. The EEG records showed definite abnormalities in 9, and minor abnormalities in 5. All patients with HAM for more than 5 years showed definite evidence of cerebral involvement. Our findings strongly suggest that a multifocal pathological process over the entire CNS is probably a common occurrence in HAM.

Adult↗
PubMed1988

Expression of HTLV-I antigen in cultured peripheral blood mononuclear cells from patients with HTLV-I associated myelopathy.

The HTLV-I antigen (Ag) was detected in short-term cultured peripheral blood mononuclear cells (PBMNC) from 44% of patients with HTLV-I-associated myelopathy (HAM), using the indirect immunofluorescence method. The HTLV-I Ag-positive cells accounted for less than 4% of cultured PBMNC in all but one case. The kinetics and mechanism of HTLV-I Ag expression in cultured cells were these studied in this individual with about 20% positive cells. HTLV-I Ag was 0.3% at 6 h after the culture and the number of positive cells increased to 9.3% at 48 h. The sera from HAM patients had a suppressive effect on the expression of HTLV-I Ag in the cultured cells. This suppression was more potent in sera from patients with a high than with low antibody titer. There were no correlations between the HTLV-I Ag expression in cultured cells and the various clinical and laboratory findings, in each case.

Adult↗
PubMed1988

Elevated serum antibody titers to Epstein-Barr virus in HTLV-I-associated myelopathy (HAM).

We studied the serum antibody titers to Epstein-Barr virus (EBV) in 11 patients with HTLV-I-associated myelopathy (HAM). HAM patients showed significant increases in IgG antibodies to EBV-capsid antigen (VCA) and -early antigen (EA) and in VCA-specific IgA compared with 25 seronegative subjects. However, there were no significant differences in antibodies to EBV-nuclear antigen (EBNA) or to other viruses between the two groups. In HAM patients, altered antibody responses occurred specifically to EBV-associated antigens, not to other viruses.

Adult↗
PubMed1988

Surface phenotypes of lymphoid cells altered in the human myasthenic thymus.

We investigated surface phenotypes of peripheral blood lymphocytes and thymic lymphoid cells from patients with myasthenia gravis (MG) by fluorocytometry, using monoclonal antibodies to human lymphoid cells. There were no significant differences in peripheral blood lymphocyte subsets in myasthenic patients, with or without a thymus. In the MG hyperplastic thymuses, the percentage of OKIa1+ cells or CCB1+ cells was significantly increased compared with controls. Although there were no significant differences in the percentage of T-cell lineage (CD3+, CD4+, CD8+, or CD1+ cells) between MG hyperplastic thymuses and the controls, the surface densities of T-cell lineage antigens (CD3, CD4, and CD8) were significantly increased on lymphoid cells in MG hyperplastic thymuses, compared with those in control thymuses. There were no significant differences in the percentage or fluorocytograph of lymphoid cells between the MG thymomas and the controls.

Adolescent↗
PubMed1988

Altered subsets of peripheral blood lymphocytes in patients with HTLV-I associated myelopathy (HAM).

We studied subsets of peripheral blood lymphocytes (PBL) from 12 patients with human T-lymphotropic virus type I (HTLV-I) associated myelopathy (HAM). The percent of OKT8+ cells was significantly decreased, and the percentages of OKDR+ cells and IL-2R+ cells and the ratio of OKT4+ cells to OKT8+ cells were significantly increased compared with either HTLV-I seronegative healthy adults (controls) or HTLV-I seropositive non-HAM subjects (carriers). The subsets of PBL from eight carriers showed no differences from those in controls. The patients with HAM showed significantly higher antibody titers to HTLV-I compared with carriers. These altered PBL subsets and high HTLV-I antibody titers in the patients suggest that immune functions are in an activated state in HAM.

Adult↗
PubMed1988

Spontaneous proliferation of peripheral blood lymphocytes increased in patients with HTLV-I-associated myelopathy.

We found unstimulated (spontaneous) peripheral blood lymphocyte (PBL) proliferation significantly increased in 14 patients with human T-lymphotropic virus (HTLV)-I-associated myelopathy (HAM) compared with findings in HTLV-I seropositive non-HAM carriers (N = 8) or HTLV-I seronegative controls (N = 16). The proliferative response to phytohemagglutinin, concanavalin A, or pokeweed mitogen was decreased in the HAM patients. Cell clusters were frequent in cultures of unstimulated PBL from the HAM patients, but much less common in the controls or carriers. This spontaneous PBL proliferation was depressed when adherent-cell populations were depleted from the cultures. IL-2 activity increased in the supernatant of 3-day cultured cells from HAM patients, but not in cultured cells from the controls. Since IL-2 receptor positive cells increased in HAM, this spontaneous PBL proliferation is probably a response to IL-2 through the expression of IL-2 receptors.

Adult↗
PubMed1988

[Painful ophthalmoplegia: the Tolosa-Hunt syndrome and orbital pseudotumor syndrome].

We experienced 9 patients with "painful ophthalmoplegia", which included 7 cases of the Tolosa-Hunt syndrome (2 males and 5 females, with ages ranging from 36 to 65 years) and 2 cases of the orbital pseudotumor syndrome (2 females aged 42 and 68). The diagnosis of these syndromes was based upon Hunt's criteria and the presence of the intraorbital mass on the brain CT scan. Main manifestations of both syndromes were periorbital pain and ipsilateral oculomotor nerve palsies. Out of 9 cases, 1 patient with Tolosa-Hunt syndrome and 1 patient with the orbital pseudotumor syndrome had bilateral retro-orbital pain and ophthalmoplegia. Pain preceded the ophthalmoplegia except in one patient with Tolosa-Hunt syndrome. Total paralysis of the extraocular muscles supplied by the oculomotor nerve was noted in all the nine patients, and mydriasis was observed on the affected side in 4 of 7 patients with Tolosa-Hunt syndrome and 2 patients with the orbital pseudotumor syndrome. Neurological involvement was not only the oculomotor nerve but also the other cranial nerves; the optic nerve (in 4 cases with Tolosa-Hunt syndrome and 2 cases with the orbital pseudotumor syndrome), the abducens nerve (in 3 cases with Tolosa-Hunt syndrome and 1 case with the orbital pseudotumor syndrome), and the first division of the trigeminal nerve (in 2 cases with Tolosa-Hunt syndrome). Six patients with Tolosa-Hunt syndrome and 2 patients with the orbital pseudotumor syndrome had palpebral edema. Visual disturbance and palpebral edema were severer in the patients with the orbital pseudotumor syndrome. After corticosteroid hormone was administered, there was diminution of the pain within 2 weeks.(ABSTRACT TRUNCATED AT 250 WORDS)

Adrenal Cortex Hormones↗