Xingnaojing injection has been widely used in the treatment of cerebral vascular diseases, through the following mechanisms: improving the blood-brain barrier (BBB) permeability, anti-oxidant free radical damage, inhibition of excitatory amino acids (EAA) toxicity and calcium overload, inhibition of apoptosis, reducing cerebral edema and inhibition of autophagy. Thus, the paper summarized its progress.

Objective To observe the expressions and distribution of transient receptor potential cation channel 6 (TRPC6) and integrin-linked kinase (ILK) in the glomeruli of renal biopsytissue of patients with proteinuric kidney diseases,and to investigate the effect of TRPC6 over-expression on ILK in vitro.Methods The archival histological specimens of patients admitted to Tangdu hospital from 2012 to 2013,with 24-hour urinary protein over 1 g,were collected.The expressions and distribution of TRPC6 and ILK in the glomeruli of renal biopsy tissue were observed by immunohistochemistry.MPC5 podocytes were cultured in vitro and they were stimulated with 10-7 mol/L ADR for 12,24 and 36 h.The pcDNA3.1(+)-TRPC6 plasmid and pcDNA3.1(+) were transfected into MPC5 podocytes by liposome 2000 reagent to establish the TRPC6 overexpression group and the negative control group respectively.Western blotting was used to detect the expressions of TRPC6 and ILK protein.Results There were 14 cases of membranous nephropathy,13 cases of focal segmental glomerulosclerosis (FSGS),15 cases of membranoproliferative glomerulonephritis,12 cases of mesangial proliferative glomerulonephritis,10 cases of hyperplastic sclerosis nephritis,15 cases of IgA nephropathy,13 cases of purpura nephritis,15 cases of lupus nephritis,13 cases of hypertensive renal injury,14 cases of diabetic nephropathy and 9 cases of normal renal tissue included.In glomerulus,TRPC6 was expressed mainly in podocytes,and the expressions of TRPC6 in these renal tissues were higher than that in normal renal tissues (all P ＜ 0.05),except for hypertensive nephropathy.ILK was expressed in podocytes and the mesangial areas.The expressions of ILK in FSGS,lupus nephritis and diabetic nephropathy were higher than that in normal kidney tissue (all P ＜ 0.05),while the other renal tissues was high but showed no statistical difference with normal kidney tissue (all P ＞ 0.05).The expressions of TRPC6 and ILK were positively correlated in renal tissues of FSGS and diabetic nephropathy (r=0.906,P ＜ 0.001;r=0.783,P=0.001 respectively).The expressions of TRPC6 and ILK protein in 24 and 36 h stimulating with ADR were significantly higher than that in the control group (all P ＜ 0.05).The expression of ILK in the TRPC6 overexpression group was significantly higher than that in the normal control group (P ＜ 0.05).Conclusions The expressions of TRPC6 and ILK increase in the glomeruli of patients with kidney diseases with proteinuria being the main manifestation,especially in FSGS and diabetic nephropathy.The up-regulation of TRPC6 can increase the expression of ILK protein,which may be involved in podocyte injury.

OBJECTIVE: To summarize the genetic characteristics of 671 Chinese pedigrees affected with Duchenne/Becker muscular dystrophy (DMD/BMD). METHODS: Clinical data of the pedigrees were collected. Multiplex PCR, multiple ligation dependent probe amplification (MLPA), next generation sequencing (NGS), Sanger sequencing and long read sequencing were used to detect the variant of DMD gene in the probands and their mothers, and prenatal diagnosis was provided for high risk pregnant women. RESULTS: Among 178 pedigrees analyzed by multiplex PCR, 44 variants of the DMD gene were detected, with the genetic diagnosis attained in 110 pedigrees. Among 493 pedigrees analyzed by MLPA in combination with NGS or Sanger sequencing, 294 pathogenic/possible pathogenic variants were identified, among which 45 were unreported previously, and the genetic diagnosis attained in 484 pedigrees. Structural variants of the DMD gene were identified in two pedigrees by long-read sequencing. Among 444 probands, 341 have inherited the DMD gene variant from their mothers (76.8%). Among 390 women with a high-risk, 339 have opted to have natural pregnancy and 51 chose preimplantation genetic testing for monogenetic disease (PGT-M). The detection rate of neonatal patients and carriers following natural pregnancy was significantly higher than that for PGT-M. CONCLUSION Combined application of MLPA, NGS, Sanger sequencing and long-read sequencing is an effective strategy to detect DMD/BMD. PGT-M can effectively reduce the risk of fetuses. Above finding has expanded the spectrum of DMD gene variants and provided a basis for reproductive intervention for pregnancies with a high risk for DMD/BMD.
China ; Dystrophin/genetics* ; Exons ; Female ; Genetic Testing ; Humans ; Infant, Newborn ; Multiplex Polymerase Chain Reaction ; Muscular Dystrophy, Duchenne/genetics* ; Mutation ; Pedigree ; Pregnancy ; Prenatal Diagnosis