Objective To analyze the clinical and pathological characteristics of Alport syndrome in children. Methods Clinical and pathological information gathered from 62 patients during March 1989 to August 2012 was retrospectively analyzed. Results Four autosomal recessive Alport syndromes (AR-AS) and 58 X-linked Alport syndromes (XL-AS) were analyzed. Of the XL-AS, 47 were boys and 11 were girls. Most of patients induced by upper respiratory tract infections, and onset with hematuria and proteinuria. There was no signiifcant gender difference in family history, impaired renal tubular proteins, hypertension, im-paired renal function, hearing loss, ocular abnormalities or renal pathological changes under light microscopy. However, extensive lamination and split of glomerular basement membrane (GBM) dense layers were found in 83.0%male and 18.2%female patients (P=0.000) and the rest patients were presented with limited distribution of typical GBM changes. Proteinuria progressed signiif-cantly with age in XL-AS males (r=0.501, P=0.000). Five XL-AS patients developed to end stage renal disease (ESRD) between 11 to 16 years old. Conclusions XL-AS is the main inherited type and severe changes of GBM are common in XL-AS males. Proteinuria increases remarkably with age. The detection of type IV collagen in renal tissue or skin is helpful to diagnose Alport syndrome and conifrm inheritance modes.

To determine the bufalin concentration in rats' plasma by establishing a high-performance liquid chromatography-tandem mass spectrometry (HPLC-MS/MS) method, and to evaluate and compare the pharmacokinetic characteristics of bufalin-loaded bovine serum albumin nanoparticles (bufalin-BSA-NP) and bufalin.

Objective To study the influence of let-7b on cell proliferation and aerobic glycolysis of human melanoma cell A375.Methods Transfect A375 cell line with hsa-let-7b oligonucleotide or antisense.Glucose and lactate in medium were determined by spectrophotometry at 24 h and 48 h time point after transfection.The cell proliferation was determined by methylthiazol tetrazolium (MTT) assay.Results Over expression of let-7b in melanoma cell reduced cell proliferation notably,compared to the other groups by MTT(P ＜0.05).However,the glucose consumption and lactate production differences were not observed during 24 h or 48 h ( P ＞ 0.05 ),the blank control group transformed about 57％ and 43％ glucose to lactate during 24 h and 48 h.Conclusions Melanoma cell line A375 has notably aerobic glycolysis hallmark,let-7b could inhibit proliferation of melanoma cell line A375,but it may has no influence on glucose metabolism.

Objective To study the effects of constant and intermittent high glucose on the apoptosis of cultured bovine retinal capillary pericytes (BRPs), and to investigate the role of mitochondrial apoptotic pathway in the apoptosis of BRPs. Methods After being cultured under glucose with different concentrations tot 6 days, the change of uhrastructure of BRPs was observed under electronmieroscope, the apoptosis of pericytes was detected by TUNEL method, the mitochondrial inner transmembrane potential (△Ψm) was detected by laser scanning confocal microscopy, the change of cytochrome c (cyt-c) was assayed by spectrophotometer and the expression of apoptotic genes was detected by immunohistochemical method and RT-PCR. Results (1) BRPs showed typical changes of apoptosis in constant and intermittent high glucose concentrations. The apoptosis induced by constant high glucose concentration was more obvious than that by intermittent high glucose. (2) Constant and intermittent high glucose concentrations obviously decreased △Ψm compared with control group. The △Ψm of BRPs was correlated negatively with the apoptotie rate of BRPs (r = - 0.89, P < 0.01) ; (3) Constant and intermittent high glucose concentrations increased the release of cyt-c from mitochondria to cytoplasm, and the concentration of cyt-c in the cytoplasm was correlated positively with the apoptotic rate of BRPs (P < 0.01) ; (4) Constant and intermittent high glucose concentrations increased the expression of proapoptotie gene Bax and decreased the expression of prosurvival gene Bcl-2, resulting in increased Bax/Bcl-2 ratio. Bax/Bcl-2 ratio was negative correlatied with the △Ψm of BRPs, and positively eorrclaticd with the concentration of cyt-c in cytoplasm and apoptotic rate (both P < 0.01).Conclusion Constant and intermittent high glucose concentrations could decrease △Ψm, increase the release of cyt-c and induce the apoptosis of BRPs, the effects being stronger with constant high glucose concentration. The mitochondrial apoptotic pathway plays an important role in the apoptosis of BRPs, in which Bax and Bcl-2 are involved.

In order to investigate the effects of simvastatin on secretion and mRNA expression of interleukin-6 (IL-6) and adiponectin in 3T3-L1 adipocytes, mouse 3T3-L1 adipocytes were stimulated with lipopolysaccharide (LPS). Production and mRNA expression of IL-6 and adiponectin in 3T3-L1 adipocytes were measured using enzyme-linked immunosorbent assay (ELISA) and reverse transcriptase polymerase chain reaction (RT-PCR), respectively. The results showed that simvastatin could significantly suppress LPS-induced IL-6 production and mRNA expression in adipocytes (P<0.05), but increase the LPS-induced adiponectin secretion and mRNA expression in a dose-dependent manner (P<0.05). It was suggested that simvastatin could exert beneficial effects on prevention of obesity-induced metabolic changes in adipocytes.

In order to investigate the effects of simvastatin on secretion and mRNA expression of interleukin-6 (IL-6) and adiponectin in 3T3-L1 adipocytes, mouse 3T3-L1 adipocytes were stimulated with lipopolysaccharide (LPS). Production and mRNA expression of IL-6 and adiponectin in 3T3-L1 adipocytes were measured using enzyme-linked immunosorbent assay (ELISA) and reverse transcriptase polymerase chain reaction (RT-PCR), respectively. The results showed that simvastatin could significantly suppress LPS-induced IL-6 production and mRNA expression in adipocytes (P＜0.05), but increase the LPS-induced adiponectin secretion and mRNA expression in a dose-dependent manner (P＜0.05). It was suggested that simvastatin could exert beneficial effects on prevention of obesity-induced metabolic changes in adipocytes.

OBJECTIVETo evaluate the potential role of insulin-like growth factor-1 receptor mRNA (IGF-IR mRNA) in the onset and development of retinopathy in diabetic rats.

METHODSA diabetic model was duplicated in Wistar rats. The early changes in the retina were examined using light and transmission electron microscopy. Expression of IGF-IR mRNA was analyzed using in situ hybridization.

RESULTSWeak expression of IGF-IR mRNA (5%) was found in retinas of normal rats, but was significantly increased (15% and 18%) in the retinas of diabetic rats after 3 and 6 months of diabetes (P < 0.01). In situ hybridization and morphological study demonstrated that there was a positive correlation between IGF-IR mRNA expression and retinal changes at various stages.

CONCLUSIONIncreased IGF-IR mRNA might play an important role in the onset and development of diabetic retinopathy.

Animals ; Blood Glucose ; analysis ; Diabetic Retinopathy ; etiology ; Glycated Hemoglobin A ; analysis ; Insulin-Like Growth Factor I ; physiology ; Male ; RNA, Messenger ; analysis ; Rats ; Rats, Wistar ; Receptor, IGF Type 1 ; genetics ; Retina ; metabolism ; pathology