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Biomedical subjects

K Inui

Publications and source records attributed to K Inui.

At least 541 records · Page 30Linked to original sources

A simple method for the isolation of basolateral plasma membrane vesicles from rat kidney cortex. Enzyme activities and some properties of glucose transport.

A procedure for preparing basolateral membrane vesicles from rat renal cortex was developed by differential centrifugation and Percoll density gradient centrifugation, and the uptake of D-[3H] glucose into these vesicles was studied by a rapid filtration technique. (Na+ + K+)-ATPase, the marker enzyme for basolateral membranes, was enriched 22-fold compared with that found in the homogenate. The rate of D-glucose uptake was almost unaffected by Na+ gradient (no overshoot).

Alkaline Phosphatase↗

Properties of sulfatases in cultured skin fibroblasts of multiple sulfatase deficient patients.

Various sulfatase activities were assayed in cultured skin fibroblasts from patients with multiple sulfatase deficiency (MSD). MSD cell lines displayed deficiencies of arylsulfatase A and iduronate sulfatase, but activities of arylsulfatase B, N-acetylgalactosamine 6-sulfate sulfatase and N-acetylglucosamine 6-sulfate sulfatase were within normal ranges, but not consistently. Arylsulfatase A, minor anionic arylsulfatase and N-acetylgalactosamine 6-sulfate sulfatase in MSD cell lines had similar Km, pH optima, inhibitory or activator sensitivity to that of normal skin fibroblasts. Arylsulfatase B in MSD cell lines also had properties similar to that of normal skin fibroblasts, except an abnormal heat stability. From our results, we conclude that properties of arylsulfatase A, minor anionic arylsulfatase and N-acetylgalactosamine 6-sulfate sulfatase in MSD fibroblasts were intact. On the other hand, arylsulfatase B in MSD might be a functionally abnormal enzyme.

Cell Line↗

Hypoglycemia in a child with hepatoblastoma.

A five-year-old boy with hepatoblastoma, who showed a severe hypoglycemia as an initial symptom, was studied. After resection of tumors, hypoglycemia disappeared and did not recur despite recurrence of tumor. A high concentration of insulin-like activity (ILA) was found in preoperative serum and in the tumor extract. Hypoglycemia in this patient seemed to depend mainly on ILA and in part on liver enzyme activities related to carbohydrate metabolism.

Carcinoma, Hepatocellular↗

Amino acid and hexose transport by cultured crypt cells from rat small intestine.

The characteristics of amino acid and sugar transport in intestinal crypt epithelial cells have been examined by measuring substrate uptake in an established epithelial cell line. These cells (IEC-6 cells) have been characterized as derived from rat small intestinal crypt cells on the basis of morphological criteria (J. Cell. Biol. 80: 248-265, 1979). Amino acid transport appeared to be mediated by both Na+-dependent and Na+-independent systems. Hexose uptake was stereospecific and Na+ independent, and was markedly inhibited by phloretin and cytochalasin B. Since glucocorticoids are known to have profound effects on maturation of the intestinal epithelium in vivo, their effects on transport properties of the cultured crypt cells were studied. Hydrocortisone, while completely inhibiting cell growth, increased the initial uptake rates of various hexoses, while having little or nor effect on the initial rate of amino acid uptake. The increased hexose uptake appeared to be due to a change in Vmax rather than Km. Appearance of the Na+-dependent hexose transport system, which is present in differentiated enterocytes, was not elicited by in vitro treatment with glucocortcoids.

Amino Acids↗

Studies on alpha-ketoglutaric aciduria in type I glycogenosis.

Urinary excretion of the organic acids in patients with type I and III glycogenosis was investigated. In all patients with type I glycogenosis, urinary alpha-ketoglutarate concentration ws about 10 times the normal value. alpha-Ketoglutaric aciduria was not improved by the acute or prolonged administration of a large dose of factors for pyruvate- and alpha-ketoglutarate dehydrogenase complex. On the other hand, the level of alpha-ketoglutarate in the urine from type I patients decreased in conjunction with the decrease of plasma lactate and pyruvate concentration after repeated oral glucose loading. Oral citrate loading brought an increased excretion of alpha-ketoglutarate in type I glycogenosis. It is possible that alpha-ketoglutarate dehydrogenase in the rate-limiting step in tricarboxylic acid cycle and in patients with glycogenosis type I, the excessive excretion of alpha-ketoglutarate may be caused by the limited activity of alpha-ketoglutarate dehydrogenase with excessive substrate.

Adolescent↗

Insulin and glucagon secretion in hepatic glycogenoses.

Insulin and glucagon secretion was investigated in ten patients with hepatic glycogenosis, types I and III, in order to understand the relationship between hypoglycemia and pancreatic function. In all patients, both oral glucose tolerance and intravenous arginine infusion tests revealed hypoinsulinemia. Decreased urinary C-peptide levels with standard food intake also supported hypofunction of pancreatic beta cells. On the contrary, the normal secretion pattern of glucagon in both types indicated in the arginine loading test, intact alpha cells in the pancreas. Persistent hypoinsulinism, which is apparently an adaptation to hypoglycemia, could be an important cause of nutritional dwarfism in both types of glycogenosis. The usefulness of the measurement of urinary C-peptide, which evaluates the pancreatic function and provides management for normal body growth, is discussed.

Blood Glucose↗

Reconstruction of the cervical esophagus by transplantation and revascularization of a small intestinal segment: ten year follow-up.

This study represents a 10 year follow-up of one case of cervical esophagus reconstruction by transplantation of a small intestinal segment. The graft was of normal configuration in esophagoscopy, radiography, and biopsy. The patient is well now and is able to have any food she likes, but she finds it somewhat difficult to take liquid foods. This may be one of the longest recorded follow-up cases in medical history.

Esophagus↗

Endoscopy and intraductal ultrasonography.

Because the prognosis in pancreatic carcinoma is so poor, a reliable method for early detection of tumors is needed. Endoscopic ultrasonography (EUS) is a useful method for precise diagnosis of pancreatic tumors. Recently, peroral pancreatoscopy (POPS) and intraductal ultrasonography (IDUS) have been introduced to make a more detailed diagnosis of pancreatic tumors. These three techniques were performed in 48 patients with pancreatic carcinoma. The ultrasonographic and endoscopic findings of pancreatic carcinoma were studied. EUS, IDUS, and POPS are very useful for differentiating between benign and malignant pancreatic tumors.

Adenocarcinoma, Mucinous↗

NPC1 gene mutations in Japanese patients with Niemann-Pick disease type C.

Complementary and genomic DNAs isolated from the fibroblasts of 10 Japanese (7 late infantile, 2 juvenile, and 1 adult form of the disease) and one Caucasian patient with Niemann-Pick disease type C were analyzed for mutations in the NPC1 gene. Fourteen novel mutations were found including small deletions and point mutations. A one-base deletion and a point mutation caused splicing errors. The mutations were not clustered in any particular region of the gene and were found both in and out of the transmembrane domains. Three patients were homozygous, five were compound heterozygous, and the remaining three were suspected of being compound hetrozygous with an unknown error in one of their NPC1 alleles. Of the 14 mutations, the G1553A substitution that caused a splicing error of exon 9 appeared to be relatively common in Japanese patients, because two patients were homozygous and one patient was compound heterozygous for this mutation.

Adolescent↗

Evaluation of myelination by means of the T2 value on magnetic resonance imaging.

The progress of myelination in the cerebrum was evaluated by visual inspection, and magnetic resonance (MR) imaging and the transverse relaxation time (T2) was calculated from double echo images. Twenty-three pediatric cases, who did not show intracranial organic changes on MR examination, were included. The T2 values in the corpus callosum (CC), frontal deep white matter (FWM), occipital deep white matter (OWM) and centrum semiovale (CS) were calculated, and the changes in these values with age were followed. During the first year of life, a rapid decrease in the T2 value was seen, followed by a more gradual decrease. The T2 value seemed to reach the adult level between 2 and 3 years of life in all areas examined. The T2 values between 2 and 16 years in CC, FWM, OWM and CS were 59.7 +/- 3.6, 64.5 +/- 5.2, 69.8 +/- 4.8 and 66.3 +/- 3.3 ms (mean +/- S.D.), respectively. The T2 values in patients with clinically diagnosed Pelizaeus-Merzbacher disease (PMD) and late onset Krabbe disease were also calculated. In PMD, non-progressive prolongation of the T2 value was observed in all areas. In late onset Krabbe disease, on the other hand, progressive prolongation of the T2 values was mainly demonstrated in OWM and the posterior part of CS. These results suggest that the T2 value in the cerebral white matter allows more objective judgement than visual inspection, and makes it possible to clarify the mechanism underlying abnormal myelination, i.e. progressive or not.

Adolescent↗