Objective To recognize hereditary IgM glomerulopathy. Methods Three patients with hereditary IgM glomerulopathy were report first time in China. Genealogic investigation and light, immunofluorescence and electron microscopy were performed. Literatures concerned were reviewed. Results Kidney biopsies from three patients all revealed diffuse mesangial proliferative glomerulonephritis with intense IgM and C3 deposits. These siblings might be suffering from a hitherto undescribed clinico-pathological entity. Genealogic investigation enabled us to discover 10 additional affected members with clinical glomerulonephritis in the kindred of the family. The clinical picture was that of hematuria and/or proteinuria, meanwhile one patient died of uremia. Conclusion Genetic factors may be involved in the mechanism of IgM nephropathy .

Phospholipase A2 receptor,a transmembrane protein located on glomerular podocytes,had been proved to be the target antigen in idiopathic membranous nephropathy and provided us a new road to uncover the mechanisms of its pathogenesis.In this review,we described the structure and physiological function of phospholipase A2 receptor and highlighted the role of its autoantibody in idiopathic membranous nephropathy.Finally we suggested several possible aspects of its future clinical application.( Chin J Lab Med,2012,35:792-794 )

Objective To investigate the hepatitis C virus(HCV) and hepatitis B virus(HBV) infection in hemodialysis patients in several hemodialysis centers in Beijing.Methods HCV RNA,HBV DNA(PCR) and the serum virus antibody(ELISA) were detected in 225 uremia patients.50 volunteers and the employers in hemodialysis center were also as controls.The relationship between the infection of hepatitis virus and the dialysis time,blood infusion and hepatic function was analysed.Results 37 patients(16.4%) were positive in HCV RNA,3 patients(1.33%) positive in HBV DNA.The logistic analysis showed that blood infusion and the time of hemodialysis were the risk factors.3 patients(3/99,3.0%) were found to be infected with both hepatitis B and C,with disorder of liver function and clinical symptoms.8.1%(8/99) of patients with positive HBcAb were infected with HCV.Conclusion The prevalence of HCV infection in hemodialysis patients is serious.Hemodialysis time and times of blood transfusion are the major ways to transmit HCV.

Objective To observe the effects of high glucose(HG) on the expression of PAI-1 /tPA and the activation of NF-KB in human proximal tubular cell(HKC) ,and explore whether these effects can be reversed by lovastatin. Methods Chromogenic substance was used to show the activity of tPA and PAI-1. The expression of PAI-1 mRNA was examined by RT-PCR. Immunoblotting and laser confocal microscopy were applied to examine the expression of p65 in nuclear. Results HG could up-regulated the activity of PAI-1 from(8. 23?0. 02) to (8.40?0. 07) IU/ml, and down-regulated the activity of tPA from (6. 22?0. 52) to (4. 9?0. 11) IU/ml (control vs HG, P

Objectives To study the relationship of atypical membranous nephropathy (AMN) with idiopathic membranous nephropathy (IMN) and lupus membranous nephropathy (LMN), and to explore the predictive clinical and pathological features for LMN diagnosis. Methods The patients undergone renal biopsy in PUMCH between 2003 and 2006 were selected, and were divided into group AMN (n=28), IMN (n=100) and LMN (n=45). Clinical manifestations and pathological features were compared among three groups retrospectively. The intensity of glomerular IgG subclasses was analyzed by immunohistoehemical staining among three groups semi- quantitatively. The spatial arrangement of IgG and C3 deposits was investigated by immunofluorescenee double staining among three groups by eonfocal laser scanning microscopy. Results (1) The onset age of AMN was (38±17) years and female/male ratio(F/M) was 2.5:1 in group LMN and IMN. The onset age was significantly different among three groups (P<0.01), and F/M ratio was significantly different between AMN and IMN (P=0.017). (2) The incidence of most extra-renal manifestations was less than 20% in AMN except for hematological disorder (21.4%) and serum anti-SSA antibody positivity(40.7%). (3) The incidence of subendothelial electron densedeposits in either LMN or AMN was significantly higher than that in IMN (P<0.01). (4) The percentage of IgG3 predominance in AMN and LMN giomeruli was 78.9% and 73.9%, respectively, while the percentage of IgC,-4 predominance in IMN was 61.1%. The difference was significant(IMN vs AMN and LMN, P<0.01). (5) IMN had an overlapping distribution of IgG and C3 in subepithelial deposition, which was rarely found in AMN or LMN. (6) Among the indexes differentiating LMN and IMN, the high sensitive one was non-IgC,4 predominance in glomeruli (91.3%), while the high specific ones included subendothelial electron dense deposits (100.0%), serum anti-SSA antibody (95.5%), glomendar IgG3 predominance (94.4%). Conclusions AMN with serum ANA positivity is similar to LMN in respect to pathological features and glomerular IgG subclasses, although it has few extra-renal clinical manifestations. It may represent a latent subgroup of lupus nephritis.

Objective To study the effects of acyl-coenzyme A cholesterol acyltransferase inhibitor(ACATI, 58-035) on cholesterol homeostasis in lipid-loaded human mesangial cell line (HMCL) . Methods Oil red O was used to examine the lipid droplet . MTT was performed to detect the cell proliferation . High performance liquid chromatography (HPLC) was used to measure intracellular free cholesterol (FC) and cholesterol ester (CE) . Western blot was used to demonstrate the protein level . Real-time PCR was performed to detect the mRNA expression .Results In HMCL loaded with 100 mg/L of low density lipoprotein (LDL), 10 mg/L 58-035 significantly inhibitted the formation of lipid droplets and CE mass (ratios over control, 1 .91±0 .36 and 1 .07±0 .30 respectively, P＜0 .01) without toxic effect . It further enhanced the ACAT1 protein expression (ratios over control, 1 .27 and 1 .77 respectively) without significant influence on mRNA level, since the activity of ACAT1 pl promoter by transient transfection was not affected by either LDL or ACATI . 100 mg/L LDL down-regulated the mRNA of LDL receptor (LDLR) (ratio over control, 0 .08±0 .02, P＜0 .01)and up-regulated that of ATP binding cassette transporter 1(ABCA1)(ratio over control, 2 .97±0 .39, P＜0 .01) . The mRNA expression of ABCA1 was further upregulated (ratio over control 4 .41±1 .27, compared with LDL group P＜0 .05), and LDLR was further down-regulated (ratio over control 0 .04±0 .005, compared with LDL group P＜0 .01) in the presence of 10 mg/L ACATI 58-035 . Conclusions The CE mass in lipid-loaded HMCL can be decreased in the presence of ACATI, accompanied by further down-regulation of LDLR mRNA expression and up-regulation of ABCA1 protein and mRNA expression . It is physiological feedback to inhibit free cholesterol accumulation to maintain cholesterol homeostasis .

Objective To analysis the clinical and pathological features, results of laboratory tests and prognosis of nephrotic syndrome (NS) in patients with non-heamatological maligancy. Methods The data were collected from 25 patients who presented with NS around the diagnosis of non-heamatological malignancy. Results Twenty-five cases were investigated (age: (56.6±17.7) years; male/female ratio: 20/5). Malignancy and NS occurred within one year in 92% patients. There was a wide distribution of malignancy with involvement of 36% in digestive system and 20% in respiratory system. Ten patients (40%) presented with NS as their initial manifestation. Heamaturia appeared in 67% patients and acute renal insufficiency was complicated in 12% cases before treatments. Some other non-specific laboratory tests were found including elevated serum gamma-globulin in 50% and anemia not related with renal failure in 28% cases. Membranous nephropathy was the most common pathological changes in 67% cases. Although NS still continued for several weeks in 8 of 9 cases after surgery and/or chemical therapy, glucocorticoids was helpful to achieve the remission in these patients. However, no remission was achieved in patients without the treatment for malignancy. Conclusion Malignancy may present with NS as its initial manifestation. It should be inspected routinely and regularly in elder patients with NS, especially in those with membranous nephropathy, as well as gamma-globulinemia and anemia.

Objective To investigate the role of LPL in enhancing VLDL uptake in mesangial cells and modulating VLDL-mesangial interaction. Methods Human wild type LPL (LPLwt), catalytically inactive LPL (LPL194) or control alkaline phosphatase (AP) were expressed in human mesangiai cell line (HMCL) via adenoviral vectors. The expression of LPL mRNA and protein was detected by RT-PCR and immunoehemistry staining, respectively. LPL activity was assayed by radioisotope labeled liposome substrate. Cellular lipid deposition was visualized by oil red O staining and analyzed quantitatively by standard enzymatic procedures. Effect of LPL on HMCL proliferation was evaluated by colorimetric assay using MTr. MCP-1 mRNA and protein levels in treated HMCLs were determined by real-time quantitative BT-PCB and enzyme-linked immunosorbent assay respectively. For adhesion study, HMCLs were treated with VLDL for six hours, followed by one-hour incubation with Tamm-Horsfall protein-1 (THP-1) cells. Results Compared with HMCLs transfected by Ad-AP, the lever of cellular triglyceride content was sharply increased in Ad-LPLwt Wansfected HMCLs [(109.11±5.01) mg/g protein vs (23.98±3.23) mg/g protein,P<0.01] and was slightly increased in Ad-LPL194 transfected HMCLs [(36.33±2.64) mg/g protein vs (23.98±3.23) mg/g protein, P<0.05]. LPLwt amplified VLDL-driven mesangial cells proliferation. Compared to the HMCL-Ad-AP, MCP-1 mRNA and protein expression increasd by 39% (P<0.05) and 171% (P<0.01) in HMCL-Ad-LPLwt, and the amount of THP-1 cells adhering to HMCL-Ad-LPLwt was increased by 1.69-fold (P<0.05), without significant difference between HMCL-Ad-LPLI94 and HMCL-Ad-AP. Conclusions Overexpression of either active or inactive LPL in HMCLs accelerates VLDL-induced triglyceride accumulation, and enzymolysis action of LPL may be the major factor in this process. Active LPL significantly amplifies VLDL-induced proliferative effect on mesangial cells and enhances monocyte adhesion to mesangial cells through up-regulation of MCP-1. Hence, LPL may be an important contribution to initiation and progression of renal injury mediated by triglyceride-rich lipoproteins.

Objective To identify the effect of low salt medium on P44/42 kinase(ERK)、AP-1 in the expression of cyclooxygenase-2 (COX-2) in a mouse macula densa derived cell line (MMDD1 cells). Methods MMDD1 cells were transfected with luciferase reporter plasmid containing AP-1. Luciferase reporter assay were studied in normal salt(NS) and low salt(LS) medium; the expression of C-JUN、C-FOS were analyzed as well by Western Blotting. RT-PCR and Western Blotting were used to detect the mRNA and protein expression of COX-2. Expression of total and phosphorylated ERK was analyzed by Western Blotting in LS solution. The content of PGE2 in the supernatant was examined by ELISA. Results Phosphorylated ERK were markedly increased by LS treatment. The up-regulated COX-2 protein expression with LS was reduced with PD-98059 (ERK inhibitiors) at 20 ?mmol/L, PGE2 release was down-regulated as well. Luciferase activity of AP-1 was stimulated in LS, the up-regulated luciferase activity of AP-1 was attenuated by curcumin at 20 ?mmol/L (AP-1 inhibitor). The expression of C-JUN、C-FOS were increased as well. Low salt medium changed COX-2 mRNA abundance and protein expression was decreasedin medium with curcumin at 20 ?mmol/L.Conclusion Low salt induces the expression of COX-2 in MMDD1 cells through the activation of ERK、AP-1 pathways.

Objective To study the expression of lipoprotein lipase(LPL) in human glomerular mesangial cells and the effect of very low-density lipoprotein(VLDL) on the expression of LPL.Methods LPL mRNA expression,protein synthesis and activity were detected in human glomerular mesangial cells by RT-PCR,Western blot and a radio-chemical analysis respectively.Effect of VLDL on the expression of LPL in mesangial cells was detected by Western blot.Results In human glomerular mesangial cells,a 276 bp band,that was specific for human LPL,was identified by RT-PCR,and the same of a 55 kd band,specific for human LPL by western blot.LPL activity of mesangial cells was also detected in the medium after release by heparin.VLDL stimulated LPL protein synthesis in mesangial cells in a time-and dose-dependent manner.Conclusion LPL is expressed by human mesangial cells and it has catalytic activity.Expression of LPL in mesangial cells is regulated by VLDL.