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Biomedical subjects

T Mutoh

Publications and source records attributed to T Mutoh.

At least 109 records · Page 6Linked to original sources
PubMed1991

[An autopsy case of adult T-cell leukemia complicated with metastatic calcification of the lung].

46-year-old male patient was born in Niigata Prefecture and thereafter lived in Tokyo. In late January 1985, he noticed swelling of the bilateral inguinal lymph-nodes followed by fever and lumbago. In February, he consulted a local doctor and hepatosplenomegaly, marked leukocytosis and renal dysfunction were pointed out and he was referred to our hospital on February 22nd. The clinical laboratory data on admission were as follows; WBC 23,200/microliter, serum-Ca 18.4 mg/dl, BUN 85.3 mg/dl, creatinine 5.4 mg/dl, antibody to ATLV x160. ATL was diagnosed by biopsy of lymph nodes and examinations of peripheral blood and bone marrow hemogram. Remission was achieved in March by the treatment with adriacin. Renal failure and hypercalcemia also improved. However his respiratory dysfunction gradually worsened. The chest radiographies++ showed pulmonary edema, although there was no clinical evidence of heart failure. When his condition became stable, TBLB was performed and revealed extensive deposition of calcium along alveolar septae, suggesting that pulmonary edema was induced by the metastatic calcification of the lung. After the second treatment for ATL, he died of pneumonia. The autopsy showed calcium deposition not only in the lung but in pyramids of the kidney and in sub-serous layer of the small intestine. There was no tumor cell invasion into the bone or parathyroid gland. High urinary c-AMP together with normal levels of PTH suggested that the hypercalcemia in this case was induced by PTH-related protein. It was concluded that careful treatment for hypercalcemia is important as regards the occurrence of pulmonary edema.

Calcinosis↗
PubMed1989

The role of phosphorylation in the action of nerve growth factor.

Although there is a great deal of information available about the biological effects of nerve growth factor, the detailed mechanism(s) by which it works is still not known. Some general outlines, however, are becoming clear. It is known that a number of second messenger systems are recruited and there is evidence that this recruitment has functional consequences. There is also increasingly strong evidence that nerve growth factor causes the activation of parallel pathways of phosphorylative cascades leading to changes in the phosphorylation and, consequently, in the function of key proteins throughout the cell. It can be suggested that changes in the phosphorylation of nuclear proteins initiate the alterations in gene expression known to occur after nerve growth factor treatment, which underlie the dramatic changes in the overall biology of nerve growth factor-responsive cells.

Animals↗
PubMed1988

Nerve growth factor, a differentiating agent, and epidermal growth factor, a mitogen, increase the activities of different S6 kinases in PC12 cells.

Treatment of PC12 cells with either nerve growth factor, a differentiating agent, or epidermal growth factor, a mitogen, leads to an increase in the phosphorylation of the ribosomal protein S6. The soluble fraction of PC12 cells contains two S6 kinases, separable on heparin-Sepharose. Treatment of the cells with nerve growth factor results in an increase in the activity of one of these kinases; treatment of the cells with epidermal growth factor results in an increase in the activity of the other one. The data suggest that the patterns of phosphorylation and, in turn, the functional properties of S6 are different in cells instructed to differentiate from those in cells instructed to divide.

Adrenal Gland Neoplasms↗
PubMed1988

Microassay for GM1 ganglioside beta-galactosidase activity using high-performance liquid chromatography.

A simple and sensitive assay for GM1 ganglioside (GM1) beta-galactosidase activity was devised by direct measurement of released D-galactose using high-performance liquid chromatography (HPLC). GM1 beta-galactosidase activity in crude samples such as brain homogenates could be measured by this method. After incubation of brain homogenate for 1 h with GM1 at 37 degrees C and pH 4.4 in the presence of sodium taurodeoxycholate, the reaction was terminated by heating at 100 degrees C for 2 min and the supernatant from the centrifuged sample was analysed directly by HPLC. D-Galactose isolated by HPLC was converted into a fluorescent compound by a post-column reaction with arginine at 150 degrees C and the fluorescence intensity at 430 nm was measured with excitation at 320 nm. By this method 10 pmol of D-galactose could be measured and the fluorescence intensity was linear up to 1 mmol of D-galactose. Using this method, the optimal conditions for the activity of this enzyme were re-examined. As an application, the enzyme activity in the brain of a patient with GM1 gangliosidosis was examined. This method can be applied to any natural substrates, glycolipids or glycoproteins, the terminal galactose of which is hydrolysed by this enzyme.

Brain↗
PubMed1988

Purification and characterization of human liver beta-galactosidase from a patient with the adult form of GM1 gangliosidosis and a normal control.

beta-Galactosidases were purified to homogeneity from livers of a normal control and a patient with the adult form of GM1 gangliosidosis. The purification was achieved by chromatography on DEAE-Sepharose fast flow, Con A-Sepharose, p-aminophenyl-1-thio-beta-D-galactopyranoside-Sepharose, and QAE-Mono Q. The normal and mutant enzymes were purified about 5000-fold with a yield of 10% and 1800-fold with a yield of 34%, respectively, and could hydrolyze 4-methylumbelliferyl-beta-D-galactoside, GM1 ganglioside, and asialofetuin. The purified normal enzyme was eluted from a TSK gel G-4000SW column as three symmetrical peaks of protein which were coincident with the three peaks of enzyme activity. The enzyme in these three peaks had apparent molecular weights of 800,000 (polymer), 140,000 (dimer), and 65,000 (monomer), whereas the mutant enzyme was eluted as two symmetrical peaks of protein and enzyme activity. The apparent molecular weight of a major monomeric form of the enzyme (beta-galactosidase A) was 60,000, and no dimeric form of the enzyme existed. Normal and mutant purified enzyme preparations migrated as a single major protein band with apparent molecular weights of 65,000 or 60,000, respectively, by SDS-polyacrylamide gel electrophoresis after treatment with mercaptoethanol. On isoelectric focussing, the mutant enzyme migrated more anodally than the normal enzyme. The mutant enzyme also had altered enzyme properties, such as pH optimum, Km values, substrate specificity and heat-stability. These data on the characteristics of the purified enzyme preparations provide the first direct evidence that patients with the adult form of GM1 gangliosidosis have a structurally altered beta-galactosidase.

Adult↗
PubMed1988

Severe orthostatic hypotension in a female carrier of Fabry's disease.

A 21-year-old woman in a family with a history of Fabry's disease showed orthostatic hypotension and whorl-like corneal opacity typical for Fabry's disease. Biochemical studies revealed that she was a heterozygote of the Fabry gene. A variety of autonomic function tests demonstrated both sympathetic and parasympathetic dysfunction. To our knowledge, the present case is the first report of a heterozygous female carrier of Fabry's disease presenting dysfunction of the autonomic nervous system.

Adult↗
PubMed1988

Airway hyperresponsiveness after endotoxin inhalation depends on leukocyte infiltration.

We have investigated the relationship between acute airway hyperresponsiveness and polymorphonuclear cell (PMN) migration in airways following saline or endotoxin (ET) aerosol inhalation in 63 guinea pigs. In 20 of these animals, granulocytopenia was induced by prior treatment with hydroxyurea (HU). Airway responsiveness (AR) to inhaled methacholine, together with leukocyte counts in bronchoalveolar lavage fluid (BALF) and in tracheal epithelium, was also examined before and at 30 min, 1, 3 and 6 hr after saline or ET inhalation. In saline inhalation groups, AR and PMN counts in BALF and in tracheal epithelium did not differ from control animals at any time points. However, in ET inhalation groups AR increased significantly at 1 and 3 hr and returned to the pre-exposure level at 6 hr. This period of hyperresponsiveness was associated with an increment of PMN migration into tracheal epithelium. However, the increment of PMN counts in BALF was delayed to 3 and 6 hr after ET. In HU treated animals, AR and PMN counts in BALF and in tracheal epithelium did not change during 6 hr after ET inhalation. These results suggest that the hyperresponsiveness induced by ET inhalation either depends upon PMN migration into the tracheal epithelium.

Administration, Inhalation↗
PubMed1986

A family with beta-galactosidase deficiency: three adults with atypical clinical patterns.

Three adult patients in a single family showed severe myoclonus, ataxia, and pyramidal signs. Enzymatic analysis of lymphocytes, plasma, and cultured skin fibroblasts showed marked deficiency of beta-galactosidase activity, more profound with GM1 ganglioside than with another natural substrate, asialofetuin. Other lysosomal hydrolases were normal. Although the physical signs were similar to those of types 1 and 2 GM1 gangliosidosis, none had bony abnormalities.

Adult↗
PubMed1986

Atypical adult GM1 gangliosidosis: biochemical comparison with other forms of primary beta-galactosidase deficiency.

We studied beta-galactosidase in skin fibroblasts from patients with different forms of beta-galactosidase deficiency: adult GM1 gangliosidosis, type 1 GM1 gangliosidosis, and Morquio B syndrome. Enzyme properties in the adult cases differed from the other disorders and also from normal controls. Genetic hybridization studies indicated that all three forms belong to the same complementation group. Therefore, the adult disorder must be due to a mutation of the structural gene for beta-galactosidase, which is allelic to the mutations in type 1 GM1 gangliosidosis and Morquio B syndrome.

Fibroblasts↗
PubMed1985

A microassay for acid beta-galactosidase activity toward asialofetuin.

To study the enzymatic properties of beta-galactosidase from the patients with a beta-galactosidase deficiency such as GM1 gangliosidosis, determination of enzymatic activity with naturally occurring substrates, asialofetuin in addition to another natural substrate, GM1 ganglioside, is essentially required. With a previously reported, simple and sensitive fluorometric assay for GM1 ganglioside beta-galactosidase using high performance liquid chromatography (HPLC), optimal reaction conditions were determined for the assay of acid beta-galactosidase activity toward asialofetuin in skin fibroblast homogenates. Under these conditions, reduced enzymatic activities could be detected in cultured skin fibroblasts from patients with type 1 and 3 GM1 gangliosidoses and mucopolysaccharidosis IV-B (Morquio B syndrome). This method was applicable to study of the enzymatic properties of the mutant beta-galactosidase and provided an alternative to assays employing radioactive or artificial substrates.

Asialoglycoproteins↗
PubMed1984

Application of a GM1 ganglioside beta-galactosidase microassay method to diagnosis of GM1 gangliosidosis.

The enzymatic diagnosis of GM1 gangliosidosis, including the diagnosis of heterozygosity, requires a microassay of GM1 ganglioside beta-galactosidase activity in lymphocytes and cultured skin fibroblasts. We have adopted high-performance liquid chromatography (HPLC) to the assay of this enzyme and can measure the activity in crude samples fluorometrically. Reaction conditions were examined to determine those optimal for the assay of GM1 ganglioside beta-galactosidase activity in lymphocyte and skin fibroblast homogenates. Under these optimal conditions, reduced enzymatic activities could be detected in lymphocytes and cultured skin fibroblasts from three patients with GM1 gangliosidosis. Thus, this assay can be used for the diagnosis, rather than the usual assays employing radioactive or artificial substrates.

Adult↗
PubMed1984

A fluorometric microassay procedure for monitoring the enzymatic activity of GM1-ganglioside beta-galactosidase by use of high-performance liquid chromatography.

For the measurement of the enzymatic activity of GM1-ganglioside (II3 NeuAcGgOse4Cer, galactosyl-N-acetylgalactosaminyl-(N-acetylneuraminosyl) galactosyl-glucosylceramide) beta-galactosidase in crude enzyme samples, a microassay using nonradioisotopic GM1-ganglioside was devised. To reduce the volume of the reaction mixture and eliminate the interferences due to the fluorescent contaminants in the reaction mixture, NADH, a product after the oxidation of the released galactose with NAD and beta-galactose dehydrogenase, was fluorometrically estimated by use of high-performance liquid chromatography. By this method, as little as 10 pmol of galactose can be detected. Using rat brain homogenates as an enzyme sample, the several parameters were reexamined to define the optimal conditions for the assay. This assay method was also applied to human cultured skin fibroblast homogenates, and it was found that this method can be used for the diagnosis of GM1-gangliosidosis, instead of the usual method using the radioisotope-labeled natural substrate.

Animals↗