Cerebral antioxidant enzyme increase associated with learning deficit in type 2 diabetes rats.

In this study, we examined alterations in the enzymatic antioxidant defenses associated with learning deficits induced by type 2 diabetes, and studied the effects of the peroxisome proliferator-activated receptor γ agonist pioglitazone on these learning deficits. Learning ability was assessed by visual discrimination tasks in Otsuka Long-Evans Tokushima Fatty (OLETF) rats, as a model of spontaneous type 2 diabetes. Levels of the antioxidant enzymes glutathione peroxidase (GPx), Cu(2+)-Zn(2+) superoxide dismutase (CuZn-SOD) and manganese SOD were measured in the cortex, hippocampus and striatum.

Threshold values of visceral fat and waist girth in Japanese obese children.

Background: In order to define the diagnostic criteria for visceral adipose tissue (VAT) accumulation and abdominal obesity in Japanese youths, a cross-sectional, multicenter study was conducted.

Methods: Subjects were 194 boys and 96 girls ranging in age from 6 to 15 years. Obese youths were classified according to the occurrence of abnormal values in serum triglyceride, alanine aminotransferase or insulin level.

An MCA/MR syndrome with hypocholesterolemia due to familial hypobetalipoproteinemia: report of a second patient with Nguyen syndrome.

[Nguyen et al. (2003); Am J Med Genet 121A: 109-112] reported a boy with severe hypocholesterolemia due to autosomal dominant hypobetalipoproteinemia (ADBHL) associated with severe growth retardation, mental deficiency, epicanthal folds, a short nose with low nasal bridge and anteverted nares and bilateral partial cutaneous syndactyly of toes 2 and 3. Many of these manifestations resembled those in a mild form of Smith-Lemli-Opitz syndrome (SLOS).

Plasma levels of orexin-a and leptin in obese children.

The present study was designed to determine the plasma level of orexin and its relationship with other metabolic and anthropometric markers in obese children. Forty-seven obese Japanese children, consisting of 31 boys and 16 girls, were enrolled in the study. Their ages were 10.

Decrease in serum adiponectin level due to obesity and visceral fat accumulation in children.

Objective: To determine whether serum adiponectin is decreased in obesity and is restored toward normal level after treatment in children.

Research Methods And Procedures: Subjects were 53 Japanese obese children, 33 boys and 20 girls (6 to 14 years old), and 30 age-matched nonobese controls for measuring adiponectin (16 boys and 14 girls). Blood was drawn after an overnight fast, and the obese children were subjected to anthropometric measurements including waist and hip circumferences and skinfold thicknesses.

A variant of Fine-Lubinsky syndrome: a Japanese boy with profound deafness, cataracts, mental retardation, and brachycephaly without craniosynostosis.

A Japanese boy with a hearing deficit, cataracts, mental retardation, and brachycephaly without craniosynostosis is described. We believe that this patient represents a variant of the Fine-Lubinsky syndrome, and is the first report from a racial group other than Caucasian.

Increased serum cholesteryl ester transfer protein in obese children.

Objective: To determine whether serum cholesteryl ester transfer protein (CETP), which is one of the physiologically active gene products secreted from adipose tissue, is increased and associated with atherogenic lipoprotein profile in obese children.

Research Methods And Procedures: Subjects were 42 consecutive outpatient Japanese obese children, 29 boys and 13 girls, ranging in age from 5 to 14 years, and 25 age-matched non-obese children, 13 boys and 12 girls, as the control group for measuring CETP mass. Blood was drawn after an overnight fast and, at the same time, and anthropometric measurements including height, body weight, waist girth, hip girth, and triceps and subscapular skinfold thicknesses were taken.

Threshold values of visceral fat measures and their anthropometric alternatives for metabolic derangement in Japanese obese boys.

Objective: To determine whether the direct measure of visceral adipose tissue (VAT) by computed tomography (CT) is a superior diagnostic criterion to the anthropometric surrogates and more classical criteria of obesity.

Design: Cross-sectional, clinical study. Obese boys were classified according to the occurrence of abnormal values in either serum triglyceride, alanine aminotransferase or insulin level.

Induction of glutathione peroxidase in response to inactivation by nitric oxide.

To determine effect of nitric oxide (NO) on cellular glutathione peroxidase (GPX) level in living cells, we measured the activity, protein and mRNA of GPX in rat kidney (KNRK) cells under a high NO condition. Combined treatment of lipopolysaccharide (LPS, 1 microgram/ml) and tumor necrosis factor-alpha (TNF-alpha, 50 ng/ml) synergistically enhanced (23-folds) nitrite production from KNRK cells. This was suppressed by an inducible NO synthase (iNOS) inhibitor (aminoguanidine, N-nitro-L-arginine methylester hydrochloride) and arginase.

Effect of obesity and troglitazone on expression of two glutathione peroxidases: cellular and extracellular types in serum, kidney and adipose tissue.

To determine the effect of obesity on expression of cellular- (C-) and extracellular (EC-) glutathione peroxidase (GPX) in serum, kidney and adipose tissue, we measured GPX in serum, kidneys and adipose tissue of the obese Otsuka-Long-Evans-Tokushima Fatty (OLETF) rat and its lean counterpart (LETO). We also investigated the effect of troglitazone. Five each of OLETF and LETO rats were fed diet with or without 0.

Troglitazone inhibits the expression of inducible nitric oxide synthase in adipocytes in vitro and in vivo study in 3T3-L1 cells and Otsuka Long-Evans Tokushima Fatty rats.

The aim of this study was to determine the mechanism of troglitazone action on nitric oxide (NO) production via inducible NO synthase (iNOS) in adipocytes in vitro and in vivo. The treatment of 3T3-L1 adipocytes with the combination of lipopolysaccharide (LPS), tumor necrosis factor-alpha and interferon-gamma synergistically induced de novo iNOS expression leading to enhanced NO production. The NO production was inhibited by co-treatment with aminoguanidine or N-nitro-L-arginine methylester hydrochloride.

Changes in serum leptin concentration during behavioral therapy in obese children.

To determine the pathophysiological implications of serum leptin level in obesity, we monitored the changes in serum leptin level during outpatient treatment with life style modification in children. Fifty-five obese Japanese children (34 boys and 21 girls; mean age, 9.64 years) were studied.

Decreased activity of plasma cholesteryl ester transfer protein in children and adolescents with insulin-dependent diabetes mellitus.

The aims of the present study were to determine whether the activity of cholesteryl ester transfer protein (CETP) is altered in the plasma of children and adolescents with insulin-dependent diabetes mellitus (IDDM), and whether high-density lipoprotein-cholesterol (HDL-C) levels reflect CETP activity. Plasma CETP activity was measured by a micromethod for radioisotopic assay, using exogenous lipoproteins as donor and acceptors. The study subjects were 22 Japanese children and adolescents with IDDM (8M, 14F) with a mean age of 13.

Effect of peroxisome proliferator on extracellular glutathione peroxidase in rat.

Glutathione peroxidase (GPX) activity measured using tert-butyl hydroperoxide as a substrate detects solely cellular/classical GPX (cGPX) in rat liver and kidney, and extracellular/plasma glutathione peroxidase (EC-GPX) in rat serum. To investigate the effect of peroxisome proliferator on EC-GPX, we measured activities of GPX and catalase in rat liver, kidney and serum, and then we performed immunoblot and Northern blot analyses in the kidney. Rats were fed on a diet containing either 2% (w/w) di-2-ethylhexyl phthalate (DEHP) or 0.

Increased peroxisomal fatty acid beta-oxidation and enhanced expression of peroxisome proliferator-activated receptor-alpha in diabetic rat liver.

To determine whether the increased fatty acid beta-oxidation in the peroxisomes of diabetic rat liver is mediated by a common peroxisome proliferation mechanism, we measured the activation of long-chain (LC) and very long chain (VLC) fatty acids catalyzed by palmitoyl CoA ligase (PAL) and lignoceryl CoA ligase and oxidation of LC (palmitic acid) and VLC (lignoceric acid) fatty acids by isotopic methods. Immunoblot analysis of acyl-CoA oxidase (ACO), and Northern blot analysis of peroxisome proliferator-activated receptor (PPAR-alpha), ACO, and PAL were also performed. The PAL activity increased in peroxisomes and mitochondria from the liver of diabetic rats by 2.

Relationships between an index of body fat distribution (based on waist and hip circumferences) and stature, and biochemical complications in obese children.

Objective: To determine the clinical utility of a new age-adjusted measure of body fat distribution (based on waist and hip circumferences) and stature, in relation to biochemical complications in obese children.

Design: Cross-sectional, clinical study. The formula to calculate the common standard deviation score (SDS) of waist-to-hip ratio/height (WHR/Ht) was obtained from the data of control children.

Effect of selenium deficiency on cellular and extracellular glutathione peroxidases: immunochemical detection and mRNA analysis in rat kidney and serum.

To determine the effect of selenium (Se) deficiency on expression of glutathione peroxidase (GSH-Px) 1 and 2, we measured GSH-Px activity in rat serum, liver and kidneys, serum immunoreactive GSH-Px 2, and the mRNAs of kidney GSH-Px 1 and 2. We purified rat GSH-Px 2 and raised polyclonal antibodies. Immunoreactive GSH-Px 2 was measured by rocket immunoelectrophoresis.

New age-adjusted measure of body fat distribution in children and adolescents: standardization of waist-hip ratio using multivariate analysis.

Objective: To explore a new anthropometric index of body fat distribution adjusted for ages ranging from 6-15 y in both boys and girls.

Design: Sex, age, and 11 anthropometric variables were subjected to principal component analysis. Based on these results, we developed a new anthropometric index, namely an age-adjusted measure of body-fat distribution.

Screening and detection of gene mutations in Japanese patients with Fabry disease by non-radioactive single-stranded conformation polymorphism analysis.

We have applied non-radioactive polymerase chain reaction (PCR)-single-stranded conformation polymorphism (SSCP) to the detection of gene mutations causing Fabry disease. Nineteen of 22 known mutations were detected as electrophoretic mobility shifts on PCR-SSCP analysis. Then, DNA from newly diagnosed Japanese patients with the classical form of Fabry disease was subjected to PCR-SSCP analysis, and 4 novel mutations (1 small deletion, 1 nonsense mutation and 2 missense mutations) and 1 neutral polymorphism were identified.

Increased plasma cholesteryl ester transfer activity in obese children.

To determine whether enhanced activity of cholesteryl ester transfer protein (CETP) contributes to the development of atherogenic lipoprotein profiles in obese children, plasma CETP activity was assayed according to a micro-method, by co-incubating lipoprotein-deficient samples with exogenous donor and acceptor lipoproteins. The study subjects were 31 obese children (14 males and 17 females). Serum levels of triglycerides, total cholesterol (TC), low-density lipoprotein cholesterol (LDL-C), TC:high-density lipoprotein (HDL)-C, LDL-C:HDL-C, apolipoprotein (apo) B, and apo B:apo Al were increased in obese children.

Two novel gene mutations (Glu174-->Lys, Phe383-->Tyr) causing the "hepatic" form of carnitine palmitoyltransferase II deficiency.

Carnitine palmitoyltransferase II (CPT II) deficiency has two different clinical forms, one with "hepatic" and the other with "muscular" symptoms. We studied the molecular basis of the "hepatic" form in two Japanese siblings. Their CPT II activity in lymphoblasts was reduced to 3% of the level observed in normal controls.

Increased activity of plasma cholesteryl ester transfer protein in children with end-stage renal disease receiving continuous ambulatory peritoneal dialysis.

The activity of cholesteryl ester transfer protein (CETP) was assayed in sera of children and adolescents on continuous ambulatory peritoneal dialysis (CAPD) by coincubating lipoprotein-deficient samples with exogenous donor and acceptor lipoproteins. Serum levels of triglycerides, total cholesterol, low-density lipoprotein cholesterol and apolipoprotein B and apolipoprotein B/apolipoprotein A1 ratio were increased, while the high-density lipoprotein cholesterol level was decreased. The CETP activity in children on CAPD exceeded that in either adults or control children, and was correlated with low-density lipoprotein cholesterol, apolipoprotein B, and total cholesterol/high-density lipoprotein cholesterol, low-density lipoprotein cholesterol/high-density lipoprotein cholesterol, and apolipoprotein B/apolipoprotein A1 ratios.

MRI of white matter changes in the Sjögren-Larsson syndrome.

We report a case of Sjögren-Larsson syndrome with spastic diplegia and conduction aphasia. MRI demonstrated the white matter changes deep in the cerebral hemispheres. We analyse the MRI findings and compare the results with neuropsychological signs.