Effect of alloxan diabetes on cholesterol flux into aorta.
Explore the source record for details and available documents.
Animals↗
Biomedical subjects
I Matsuda
Publications and source records attributed to I Matsuda.
Cholesterol flux into aorta in experimental nephrosis.
Explore the source record for details and available documents.
Animals↗
Bilirubin and urobilin excretion into stools in infancy.
Explore the source record for details and available documents.
Bilirubin↗
Subcellular distribution of acid alpha-glucosidase in fibroblasts and of antigenically cross-reactive material in Pompe's disease fibroblasts.
From fibroblasts of two cases of Pompe's disease (acid alpha-glucosidase deficiency), one of the childhood type (RH-SF-1) and one of the adult type (RH-SF-2), and normal fibroblasts, antigenically cross-reactive material and acid alpha-glucosidase were immunoprecipitated and analysed by immunoelectrotransfer blotting. The acid alpha-glucosidase and antigenically cross-reactive material (which reacts with antibody raised against normal acid alpha-glucosidase) revealed a precursor form of molecular weight 97,000 and two major components of 79,000 and 76,000. When monensin was added to the fibroblast culture, the two major components of normal acid alpha-glucosidase were decreased, whereas the large molecular weight precursor was increased. On the other hand, the 97,000 molecular weight component of cross-reactive material in the Pompe's fibroblasts (RH-SF-1 and RH-SF-2) was only slightly increased on monensin treatment. The fibroblasts were pulse-chase labelled with [2-H3] mannose and 32Pi. The cross-reactive material and acid alpha-glucosidase were precipitated with anti acid alpha-glucosidase antibody, and after sodium dodecyl sulphate-polyacrylamide gel electrophoresis (SDS-PAGE), fluorography was performed. The radiolabel of 3H in the cross-reactive material of RH-SF-1 and -2 was weak, and 32P in the cross-reactive material of both fibroblasts was very weak when compared with those of the acid alpha-glucosidase. The radiolabel of 32P in the cross-reactive material of RH-SF-1 was extremely weak. Immunofluorescence histochemistry revealed a granular localization of acid alpha-glucosidase in the normal fibroblast cytoplasm, and a diffuse distribution of cross-reactive material in the cytoplasm of RH-SF-1 and -2. Immuno-electron microscopic examinations showed a normal acid alpha-glucosidase localization on the inner side of the lysosomal membrane and also diffusely in the lysosome; when treated with monensin, it was present on the trans part of the Golgi apparatus. Antigenically cross-reactive material, however, was found in the cytoplasm and endoplasmic reticulum. Some lysosomal localization was observed sporadically. Even after monensin treatment, it was not demonstrated on the Golgi apparatus.
Antigens↗
Demonstration of acid alpha-glucosidase in different types of Pompe disease by use of an immunochemical method.
The nature of mutant acid alpha-glucosidase (AAG) in muscle was studied in 6 patients with Pompe disease, consisting of 2 each of the infantile, childhood and adult types. Anti-human liver AAG rabbit antibody prepared in the present study was confirmed to be monospecific by immunodiffusion, immunotitration and immunohistochemical methods. It was found by the immunodiffusion and enzyme immunoassay methods using this antibody that the mutation produced a normal amount of enzyme protein but the latter was an inactive form, suggesting structural gene mutation in 5 of the 6 cases. In the remaining childhood type case there was no detectable amount of enzyme protein, suggesting that the mutation causes a reduction in the amount of the enzyme protein or synthesis of unstable enzyme protein. Similarly, the enzyme activity of AAG was markedly reduced in all patients, but that of neutral alpha-glucosidase was the least reduced in the adult type, medium in the childhood type, and the most reduced in the infantile type.
Adult↗
Carnitine prevents Reye-like syndrome in atypical carnitine deficiency.
A patient with repeated episodes of a Reye-like syndrome was studied. Serum and muscle carnitine levels were normal, but there was an apparent accumulation of muscle lipid and glycogen. Ragged-red fibers were present in the muscle. Prolonged fasting (20 hours) induced hypoglycemia, lactic acidosis, an increase in free fatty acids, and hyperammonemia. There was an accompanying sizeable reduction in the serum free carnitine level. Fasting with L-carnitine administration resulted in milder changes in these laboratory measures. Administration of L-carnitine, (100 mg/kg/day) led to clinical improvement as evidenced by fewer attacks and a normal Gowers sign.
Biopsy↗
Carnitine status in Reye and Reye-like syndromes.
Fourteen children with the following Reye and Reye-like syndromes were studied to determine each patient's carnitine status: valproate-induced Reye-like attack, ornithine transcarbamylase deficiency, systemic carnitine deficiency, methylmalonic acidemia, and propionic acidemia. Reduced free carnitine and increased serum and urine acylcarnitine levels were found in all patients except for 2 with Reye syndrome, in whom serum creatinine levels were mildly elevated and serum free carnitine levels were not reduced. The renal free carnitine reabsorption rate was reduced in all cases. The free carnitine content of autopsied liver samples were reduced in 2 Reye syndrome patients, 2 OTC deficiency patients, and in a single systemic carnitine deficiency patient. The observed secondary free carnitine deficiency may be a factor in the pathogenesis of Reye and Reye-like syndromes.
Carnitine↗
Conversion of thyroxine into tri-iodothyronine in zinc deficient rat liver.
Hepatic thyroxine (T4) to tri-iodothyronine (T3) conversion was measured in six animal groups: Group A was fed a severe zinc-deficient diet (1.98 ppm) for 5 weeks; group B was a pair-fed control group for group A; group C was fed a less severe zinc-deficient diet (6.10 ppm) for 5 weeks; group D was a pair-fed control group for group C; group E was fed a zinc-supplemented control diet (90.4 ppm) for 5 weeks; and group F was first fed the severe zinc-deficient diet for 5 weeks and then placed on the zinc-supplemented control diet until a body weight corresponding to the final weight of group E was obtained. Serum T3 and T4 levels and T4 to T3 conversion were significantly reduced in group A. A significant positive correlation was observed between T4 to T3 conversion and alcohol dehydrogenase (zinc-metalloenzyme) activity in liver tissue from the six groups. Thus, it appeared that an appropriate amount of zinc might be one of the factors in thyroxine to tri-iodothyronine conversion in liver tissue normally.
Alcohol Dehydrogenase↗
A single case of hypophosphatemic rickets with hypercalciuria.
A single case of hypophosphatemic rickets with hypercalciuria and an elevated level of serum 1,25 dihydroxyvitamin D is reported. The characteristic features (genu valgum, rickets, short stature, increased renal phosphate excretion, decreased serum phosphorus level, elevated serum alkaline phosphatase level, and normal serum calcium level) were comparable to those in hypophosphatemic vitamin D resistant rickets. Massive doses of 1 alpha-hydroxyvitamin D were not effective for the rickets and the biochemical defect in this patient. Long-term phosphate supplementation on its own resulted in the reversal of all clinical and biochemical abnormalities except for the decreased ratio between the maximum tubular reabsorption rate for phosphorus and the glomerular filtration rate. In this patient, the concentration of serum 1,25 dihydroxyvitamin D seemed to be controlled by the concentration of serum phosphorus rather than by the serum parathyroid hormone level. It is noted that this is the first case of a single hypophosphatemic rickets with hypercalciuria.
Calcitriol↗
Vitamin K deficiency in breast-fed infants at one month of age.
PIVKA-II (protein induced by vitamin K absence or antagonist-II) was measured, as an indicator of vitamin K deficiency, in breast-fed infants of approximately 1 month of age. The infants consisted of three different groups: untreated (group 1); those given 5 mg vitamin K once at birth (group 2); and those given 5 mg twice, at birth and at 14 days after birth (group 3). At 1 month of age, the rate of PIVKA-II-positive infants and their PIVKA-II levels were significantly reduced in group 3 as compared with the levels of the other two groups, whereas these parameters were similar between groups 1 and 2. This observation suggested that vitamin K administration once at birth may be unsafe by 1 month of age. An additional administration of vitamin K seemed to be necessary for complete prevention of vitamin K deficiency, causing severe bleeding in breast-fed infants of approximately 1 month of age.
Biomarkers↗
Acarboxy prothrombin (PIVKA-II) as a marker of hepatoblastoma in infants.
We evaluated plasma PIVKA-II (protein induced by vitamin K absence or antagonist-II, acarboxy prothrombin) levels in three infants with hepatoblastoma as a tumor marker. PIVKA-II levels were highly elevated in all three patients. Vitamin K administration, performed in two patients, resulted in only moderate reduction of PIVKA-II levels. Chemotherapy against tumor cells reduced the PIVKA-II levels without exception. Immunohistochemical study of the liver tissue indicated the presence of PIVKA-II in the hepatoblastoma cell. These findings suggest that elevated PIVKA-II in these patients was not due to nutritional vitamin K deficiency, but to excess production of tumor cells. A measurement of plasma PIVKA-II may be useful as a new marker of hepatoblastoma.
Biomarkers↗
A prospective survey of serial maternal serum zinc levels and pregnancy outcome.
Serum zinc levels were monitored serially in pregnant women. Compared with those of age-matched nonpregnant women, their serum zinc levels were significantly decreased in the first trimester (p less than 0.005) and further decreased in the second trimester (p less than 0.005) but then remained unchanged until delivery. Cord blood zinc levels and the ratio of cord blood to maternal serum zinc at delivery were significantly higher in preterm than term (greater than or equal to 37 weeks) infants (p less than 0.05, p less than 0.05). The maternal serum zinc level in the second trimester was not related to the birth weights of the infants. Mothers with serum zinc levels of less than 65 micrograms/dl in the last trimester showed a higher incidence of birth weights of less than the 50th percentile (p less than 0.005) and also of less than the 25th percentile (p less than 0.005) on term delivery. The incidences of birth weights of less than the 10th percentile on term delivery were similar for mothers with serum zinc levels of greater than or equal to 65 micrograms/dl. This observation suggested that a relatively lower birth weight may be associated with lower maternal serum zinc levels in the third trimester, but an additional factor (factors) is also involved in the outcome of low birth weight infants.
Birth Weight↗
Enzyme-linked immunosorbent assay for apolipoprotein B on dried blood spot derived from newborn infant: its application to neonatal mass screening for hypercholesterolemia.
Hypercholesterolemia is a risk factor for atherosclerotic cardiovascular disease, and early diagnosis and treatment should be given attention. We developed a simple and sensitive enzyme-linked immunosorbent assay (ELISA) for apolipoprotein B (apoB) on a dried blood spot (DBS). The specificity of this assay was investigated by constructing curves for other proteins. The cross-reactivity was negligible. The mean intra- and intercoefficients of variation were 5.2 and 7.8%, respectively. We used phosphate buffer with Triton X-100 for extracting apoB on DBS. The elution of apoB was stable up to 30 days after bleeding. The correlation between plasma and DBS apoB was r = 0.94, p less than 0.005, n = 55. Using this method, we screened 2,500 babies between 5 and 7 days of age. The mean +/- SD of DBS apoB was 75 +/- 15 U. Seven hypercholesterolemic neonates were detected. Family studies of these neonates disclosed one definite and two suspected heterozygotes for familial hypercholesterolemia. Four other neonates showed no related familial background. This ELISA method for assaying apoB should prove to be a useful tool for large mass screening for hypercholesterolemia, particularly in the newborn.
Apolipoproteins B↗
Nutritional copper deficiency in severely handicapped patients on a low copper enteral diet for a prolonged period: estimation of the required dose of dietary copper.
Six cases of nutritional copper deficiency were identified in a nursery institute for severely handicapped patients. All had been on prolonged enteral feeding of a copper-deficient diet and all had abnormalities related to the copper deficiency. Two of them had leukopenia, with or without macrocytic and normochromic anemia. After the oral administration of copper, the clinical and laboratory observations including neutrophil counts and serum copper and ceruloplasmin levels showed a complete recovery. The dietary copper requirement was estimated to be approximately 20 micrograms/kg/day for these patients, based on the correlation between the dietary copper intake and the levels of copper in the serum.
Adolescent↗
Age- and sex-related differences in lipoproteins containing apoprotein A-I.
We investigated age- and sex-related differences in the structure and composition of two species of lipoproteins that contain apoprotein (apo) A-I (A-I Lp): lipoproteins containing apo A-I and apo A-II (Lp A-I/A-II), and lipoprotein containing apo A-I but no apo A-II (Lp A-I), which were isolated by immunoaffinity chromatography. Sixty normolipidemic volunteers were assigned to one of three groups based on their ages and sexual maturation (Group A, prepubertal; Group B, puberty; and Group C, postpuberty). In A-I Lp, the levels of total cholesterol, cholesteryl ester, phospholipid, and apo A-I were lower in males during puberty and then remained stable. In Lp A-I/A-II, there were no age- or sex-related changes in lipids or in apo A-II. Levels of apo A-I in the females were lower with advance in age, although significant differences were observed only between pre- and postpubertal subjects. In Lp A-I, the levels of total cholesterol, cholesteryl ester, phospholipid, and apo A-I were lower in males during puberty and remained stable thereafter, as in the case of A-I Lp. Therefore, the age- and sex-related differences observed in A-I Lp appear to be primarily due to the differences in Lp A-I. When we take into account the constancy of Lp A-I/A-II levels in all groups, the physiological function of A-I Lp (high density lipoprotein) in each individual may be limited by the Lp A-I levels.
Adolescent↗
New method for determining total dietary fiber by liquid chromatography.
The molecular weight limit of water-soluble dietary fiber (SDF) determined by the Prosky method was studied by liquid chromatography (LC). It was confirmed that only SDF with an average degree of polymerization of 12 or higher can be determined by the Prosky method. Total dietary fiber (TDF) was determined by 2 additional methods using LC. In the first method, the total quantity of insoluble dietary fiber (IDF) and high molecular weight SDF (HMSDF) was determined according to the modified Prosky method (MES-TRIS buffer-based). The quantitatively collected final filtrate was analyzed by LC for the quantity of low molecular weight SDF (LMSDF), and the 2 quantities were totaled to obtain TDF. TDF values thus determined for rice, polished or unpolished, soybean flour, and pressed barley were higher than those determined by the Prosky method by approximately 6, 3.5, and 3.5%, respectively. In the second method, direct determination by LC analysis was done on samples after enzymatic treatment according to the Prosky method. Results showed that the determination of LMSDF, in particular, was highly accurate and more effective. In both of these methods, the quantity of LMSDF was determined from its chromatographic peak area ratio to glucose as an internal standard, which was produced by hydrolysis.
Carbohydrates↗
A case of rheumatoid arthritis complicated by two different types of lymphoproliferative disorder.
Explore the source record for details and available documents.
Aged↗
Glucose metabolism in pediatric renal transplant recipients: relation to insulin receptors of erythrocytes.
We studied the oral glucose tolerance test (O-GTT) of 13 kidney transplant recipients and compared the results with the insulin binding characteristics of their own erythrocytes. They had mild renal insufficiency with significant increase of serum creatinine concentrations. Body weights were slightly but significantly elevated compared to the controls. All were receiving small doses of prednisone (0.2-0.3 mg/kg/day). Ten of the 13 patients had normal O-GTT and normal binding of 125I-insulin, while the remaining 3 patients showed abnormal O-GTT and significantly reduced maximum binding of 125I-insulin to erythrocytes. Basal insulin concentration and response to O-GTT were significantly elevated in the patients, regardless of O-GTT being normal or abnormal. It is concluded that transplant recipients have an impaired insulin action due to a post-receptor stage abnormality in glucose metabolism which is due perhaps to mild renal insufficiency, mild obesity and prolonged administration of the small dosage of prednisone.
Adolescent↗