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Biomedical subjects

H Hirono

Publications and source records attributed to H Hirono.

At least 19 recordsLinked to original sources
PubMed1995

Isolation of cDNA encoding the human liver phosphorylase kinase alpha subunit (PHKA2) and identification of a missense mutation of the PHKA2 gene in a family with liver phosphorylase kinase deficiency.

X-linked liver glycogenosis (XLG) due to liver phosphorylase kinase (PHK) deficiency is the most frequent liver glycogen storage disease. The affected patients present in early childhood with hepatomegaly and growth retardation. We isolated and determined the structure of human liver alpha subunit of PHK (PHKA2) cDNA. The 3705 base pair open reading frame encodes a polypeptide of 1235 amino acid residues, and the deduced amino acid sequence shows 93 and 68% homology to that of rabbit liver alpha subunit of PHK and human muscle alpha subunit of PHK, respectively. We identified a missense mutation, a valine substitution for glycine at amino acid 193, in the PHKA2 gene of a family with XLG.

Amino Acid Sequence↗
PubMed1991

Possible essentially of docosahexaenoic acid in Japanese monkey neonates: occurrence in colostrum and low biosynthetic capacity in neonate brains.

The importance of mother's milk as a source of docosahexaenoic acid (DHA) in Japanese monkey neonates was investigated. The DHA content in monkey colostrum total lipids was 2.2%, similar to or slightly higher than in humans. A comparison of the biosynthetic capacity of brain microsomes from monkeys of different age (up to 10 years) showed that chain elongation/desaturation of linolenic acid and eicosapentaenoic acid in neonates was significantly less pronounced than in adults. In particular, the formation of DHA, which is the product of delta 4 desaturase, was negligible. These results suggest that milk is an important source of DHA in Japanese monkey neonates.

Aging↗
PubMed1989

Micronucleus test with methyl methanesulfonate administered by intraperitoneal injection and oral gavage.

The effects of 2 routes of administration, intraperitoneal injection (i.p.) and oral gavage (p.o.), in the micronucleus test were evaluated using methyl methanesulfonate (MMS) and 2 strains of mice (MS/Ae and CD-1). A small-scale acute toxicity study and a pilot micronucleus experiment were carried out first. On the basis of the results obtained, a final micronucleus test was performed at doses of 20, 40, 80, and 160 mg/kg (i.p.) and 40, 80, 160, and 320 mg/kg (p.o.), with a 24-h sampling time. MMS induced micronucleated polychromatic erythrocytes (MNPCEs) in both routes in both mouse strains under the conditions used. At 40 and 80 mg/kg, MMS induced a higher number of MNPCEs by the i.p. route in both strains. A 160 mg/kg MMS dose induced higher numbers of MNPCEs by the p.o. route in MS/Ae mice. The route-related difference with MMS on the basis of mg/kg disappeared when the difference was determined on the basis of a ratio of the LD50. In practice, both i.p. and p.o. routes are acceptable as routes of administration in the micronucleus test using this chemical.

Administration, Oral↗
PubMed1986

Many isoforms of fast muscle troponin T from chicken legs.

Troponin T from fast muscle of chicken legs was found to be composed of about 40 kinds of isoforms by two-dimensional polyacrylamide gel electrophoresis in conjunction with immunoblotting tests with an antiserum to chicken breast muscle troponin T. Almost all of the isoforms were found in the myofibril preparation and troponin preparation from the leg muscle, and they showed complex-forming ability with troponin I and troponin C. These isoforms existed in most of the fast muscle except pectoralis and posterior latissimus dorsi muscles, and they changed in composition during development. The breast muscle troponin T also showed different types of isoforms in the period soon after hatching. Since proteolysis was completely inhibited during two-dimensional gel electrophoresis and since the many isoforms were observed consistently in various muscles of chicken leg, they are most probably products of mRNAs generated by differential gene splicing.

Animals↗
PubMed1984

Lipids of liver, kidney, spleen and muscle in a case of generalized deficiency of cytochrome b5 reductase in congenital methemoglobinemia with mental retardation.

The lipid compositions of liver, kidney, spleen and muscle in a case of generalized deficiency of cytochrome b5 reductase in congenital methemoglobinemia with mental retardation were analyzed and compared with age-matched controls. The contents of cholesterol and phospholipids (mumol/g) in each organ were within the normal range. Diminished proportions of unsaturated fatty acids were observed in ethanolamine phosphoglycerides (EPG) of the liver. The proportion of linoleic acid decreased to less than half of the normal level in EPG of liver, kidney and spleen.

Cytochrome Reductases↗
PubMed1983

Adipose fatty acid composition in a case of generalized deficiency of cytochrome b5 reductase in congenital methemoglobinemia with mental retardation.

The fatty acid composition of the triglyceride in adipose tissue in a case of generalized deficiency of cytochrome b5 reductase in congenital methemoglobinemia with mental retardation was analyzed and compared with age-matched controls. The proportions of linoleic acid, linolenic acid and arachidonic acid were decreased to less than half of normal level. There was a decrease in total unsaturated fatty acids and an increase in palmitic acid. These results revealed an undeveloped pattern of fatty acid composition in adipose tissue in the patient.

Adipose Tissue↗
PubMed1980

Lipids of myelin, white matter and gray matter in a case of generalized deficiency of cytochrome b5 reductase in congenital methemoglobinemia with mental retardation.

The lipid classes and fatty acid compositions of myelin, white matter and gray matter were analyzed in a case of generalized deficiency of cytochrome b5 reductase in congenital methemoglobinemia with mental retardation. When compared with normal data, the percentage of 24:1 was considerably decreased and diminished unsaturation was observed in cerebrosides, whereas the sum of 24:0 and 24:1 was the same as in normals. The ratio of hydroxy fatty acids to total fatty acids in cerebrosides was low. The contents of cholesterol and phospholipids in white matter were reduced to 80% of the normal, whereas cerebroside was reduced to 48% of the normal.

Brain Chemistry↗
PubMed1978

Effects of dietary folate deficiency on developmental increase of myelin lipids in rat brain.

Rats were fed a folic acid deficient purified diet from day 12 of gestation throughout the lactational period. Offsprings were fed the same diet after weaning. Control rats were given 170 microgram of folic acid per day per rat supplemented to the same diet, which was fed ad libitum or by pair-feeding. At 3 and 6 weeks of age, myelin was isolated from rat brains. It was found that in comparison with the controls, myelin yield was significantly decreased as well as the brain weight in the folic acid deficient rats at 6 weeks of age. There were no differences of gross composition of myelin, protein, ratio of cholesterol, glycolipids, phospholipids, and total lipid with or without folate deficiency either at 3 or 6 weeks of age. The hydroxy fatty acid composition of myelin lipids in brain was not changed with folate deficiency at 3 or 6 weeks of age. The developmental increase of the percentages of 22:6, 22:4, and 20:1 in nonhydroxy fatty acids of myelin lipids from the folic acid deficient rats were significantly lower at 6 weeks of age in comparison with the controls. The n-3:n-6 ratio in myelin fatty acids from the folic acid deficient rat brains was abnormally low at 3 weeks of age and was not increased at even 6 weeks of age. The implications of these findings are that folic acid may play an important role in desaturation or chain elongation of polyunsaturated fatty acids in the brain of developing rats.

Animals↗
PubMed1977

Essential fatty acid deficiency induced by total parenteral nutrition and by medium-chain triglyceride feeding.

In hospitalized infants receiving either prolonged total parenteral nutrition without fat or a formula of medium-chain triglyceride, the fatty acid composition of platelet, red blood cell, and plasma lipids was determined. The results showed that the changes in the fatty acid composition occurred not only in plasma but also in platelets and red blood cells, and the decrease in linoleic and arachidonic acid and the concurrent increase in 5,8,11-eicosatrienoic acid were confirmed to be dramatic evidence of essential fatty acid deficiency. There was no effect of essential fatty acid deficiency upon the phospholipid distribution in red blood cells or plasma.

Animals↗
PubMed1976

Lipid composition of myelin in multiple sclerosis.

Myelin was isolated from histologically normal white matter and plaques from MS patients and from white matter of neurologically normal controls. No difference was found in the total lipid content. There were no detectable deficits in MS myelin of phosphoglycerides, plasmalogens or sphingolipids. Gangliosides and lysolecithin were not detected. Analysis of the fatty aldehyde composition of the phosphoglycerides and the fatty acid composition of the cholesteryl esters, phosphoglycerides and sphingolipids did not show any differences between the normal and MS myelin.

Cerebrosides↗