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Biomedical subjects

I Inoue

Publications and source records attributed to I Inoue.

At least 199 records · Page 11Linked to original sources
PubMed1987

Increased urinary excretion of argininosuccinate in type II citrullinemia.

Although argininosuccinate is a product of the catalytic action of deficient argininosuccinate synthetase in citrullinemia, its concentration was found to be elevated in the urine of patients with type II citrullinemia. Urinary argininosuccinate was identified by two methods; its conversions to anhydride by boiling in an acidic solution and to arginine by the enzymatic action of argininosuccinate lyase. Oral administration of citrulline to patients with type II citrullinemia and control subjects increased urinary argininosuccinate levels. These phenomena are consistent with our previous findings on type II citrullinemia (Adv Exp Med Biol 1983;153:63-76,J Clin Biochem Nutr 1986;1:129-142), namely that renal argininosuccinate synthetase which plays a role in arginine synthesis is not deficient in patients with type II citrullinemia; and that serum arginine levels in patients with type II citrullinemia are rather higher than the controls, and increase after the oral administration of citrulline. The organ-specific deficiency of argininosuccinate synthetase in type II citrullinemia is further confirmed by this paper.

Administration, Oral↗
PubMed1987

Accumulation of large neutral amino acids in the brain of sparse-fur mice at hyperammonemic state.

Sparse-fur mice which are deficient in ornithine transcarbamylase, the second-step enzyme in the urea cycle, were examined for hyperammonemia and its relationship with encephalopathy. We compared amino acid concentrations in the serum and brain of spf mice with those of control mice. Unlike hepatic encephalopathy we could not find marked amino acid changes in the serum of spf mice besides low levels of citrulline and arginine. But in the brain of spf mice, glutamine was increased strikingly during hyperammonemia, and a concomitant accumulation of large neutral amino acids such as tyrosine, phenylalanine, methionine, and histidine was observed. The accumulation of these large neutral amino acids in the brain was not influenced by 24-hr fasting which caused increases in branched chain amino acids in the serum. From these results, we conclude that the accumulation of the large neutral amino acid in the brain of hyperammonemic state is caused by uptake of ammonia in the brain and the subsequent accumulation of glutamine, but is not influenced by a decreased ratio of branched chain amino acids to aromatic amino acids in the serum.

Aging↗
PubMed1987

Plasma concentrations of immunoreactive-atrial natriuretic polypeptide in patients on hemodialysis.

Plasma concentrations of immunoreactive (IR)-atrial natriuretic polypeptide (ANP) were measured before and after hemodialysis (HD) as well as isolated ultrafiltration (UF) in 9 patients with end-stage renal disease. There were significant falls in plasma concentrations of IR-ANP during both UF (from 78.6 +/- 109.7 to 45.4 +/- 56.8 pg/ml; mean +/- SD; p less than 0.025) and HDs (from 84.7 +/- 48.6 to 35.0 +/- 28.4 (p less than 0.01) on first HD; from 73.7 +/- 74.2 to 31.8 +/- 21.8 pg/ml (p less than 0.01) on later HD). There were distinct positive correlations between blood pressures and plasma concentrations of IR-ANP. These results support the view that ANP is secreted mainly by the expansion of blood volume. The fall in plasma concentrations of IR-ANP after HD seems to be caused by the decrease of blood volume, but not by removal due to dialysis of the peptide. However, the physiological role of ANP in patients with end-stage renal disease remains unknown.

Atrial Natriuretic Factor↗
PubMed1987

Molecular basis of enzyme abnormalities in urea cycle disorders. With special reference to citrullinemia and argininosuccinic aciduria.

This paper deals with enzymological, immunochemical and molecular genetic analyses of citrullinemia and argininosuccinic aciduria. Citrullinemia has been classified by Saheki et al. [J. inher. Metab. Dis. 8: 155-156, 1985] into three types from the properties of the deficient argininosuccinate synthetase (ASS) of the patients. Analysis of hepatic mRNA coding for ASS revealed certain characteristics in type II and III citrullinemic patients whose hepatic ASS protein was low. A newly developed enzyme-linked immunosorbent assay (ELISA) of argininosuccinate lyase (ASL) protein showed that 8 out of ten cases of argininosuccinic aciduria had no detectable ASL protein in the liver, erythrocytes, cultured skin fibroblasts or cultured amniocytes.

Amino Acid Metabolism, Inborn Errors↗
PubMed1987

Hyperornithinemia, hyperammonemia, and homocitrullinuria: case report and biochemical study.

Two siblings with hyperornithinemia, hyperammonemia, and homocitrullinuria are reported. The clinical picture included protein intolerance, mental retardation, seizures, and stuporous episodes. One patient had cerebellar ataxia, myoclonus, convulsive seizure, and muscular weakness in both legs. Isolated liver mitochondria in the patient revealed that ornithine transport and citrulline synthesis were decreased, but urea cycle enzymes and ornithine aminotransferase were normal. Ornithine metabolism was decreased in cultured skin fibroblasts.

Adolescent↗
PubMed1987

Immunoenzymatic studies of ornithine transcarbamylase (OTC) in liver of patients with OTC deficiency by enzyme linked immunosorbent assay (ELISA).

Antigen contents of ornithine transcarbamylase (OTC) protein in the liver of 5 patients with OTC deficiency and mutant mice were studied by ELISA. OTC activities in the liver tissues of 2 patients were 13.1% and 5.2% of the controls, respectively. The 2 patients' antigen contents of OTC protein were decreased in parallel with the enzyme activities. OTC antigen contents were not detected in the liver tissues of remaining 3 patients who had the complete type of OTC deficiency. Enzyme activities, kinetic properties and antigen contents of the OTC deficient mutant mice were the same as the results reported previously (Briand et al. 1982). The ELISA method for the assay of OTC antigen contents used in this study is more sensitive as compared with the radial immunodiffusion technique.

Animals↗
PubMed1987

Effects of changes in dietary sodium intake and saline infusion on plasma atrial natriuretic peptide in hypertensive patients.

Plasma concentrations of immunoreactive (IR)-atrial natriuretic polypeptide (hANP) were measured by radioimmunoassay in 9 essential hypertensive patients after alteration of salt intake and acute saline infusion. Daily salt intake was altered every one week in the order of 15g/day, 3g/day, and 7g/day. On the last day of the first week, 1500 ml of 0.9% saline was infused intravenously over one hour. Plasma concentrations of IR-hANP tended to decrease, although not significant, by salt restriction. Further, there were significant positive correlations between changes in plasma concentrations of IR-hANP and those of several variables such as body weight, systolic blood pressure, and creatinine clearance. Plasma concentrations of IR-hANP rose significantly (p less than 0.05) from 50.7 +/- 20.1 (Mean +/- SEM) pg/ml to 119.0 +/- 48.8 after acute saline infusion. Although there was significant correlation between mean blood pressure and the increase in sodium excretion by saline infusion, this increase was unrelated to the rise in plasma concentrations of IR-hANP. These results suggest that the release of ANP is stimulated mainly by expansion of extracellular fluid volume in hypertensive patients. However, natriuretic and hypotensive effects attributable to the changes of ANP release could not be elucidated.

Adult↗
PubMed1987

[A phase II study of vindesine for pretreated non-small cell lung cancer].

A phase II evaluation of vindesine (VDS) was performed in 16 patients with non-small cell lung cancer (ten patients with adenocarcinoma, six patients with squamous cell carcinoma, and one patient with large cell carcinoma). All except one of the patients had had prior chemotherapy. VDS at a dose of 3 mg/m2 was given intravenously every week for more than three weeks. Among 16 evaluable patients, two patients with pretreated adenocarcinoma of the lung showed partial response. The response rate for VDS was 12.5%. Toxic effects included leukopenia (94%), anemia (44%), thrombopenia (13%), alopecia (38%), peripheral neurotoxicity (38%), liver injury (19%), constipation (13%), anorexia (13%), nausea (13%), stomatitis (6%) and fever (6%).

Adult↗