Inheirted metabolic disodrers(IMD)involves in multiple substance dysbolism,which usually results in irreversible neurological lesions because of various categories and complicated clinical manifestations.In resent years,IMD became one of the hot spots in medical domain around the world,original diagnostic technique and management progressed unceasingly.This paper provides an overview of the traditio-nal detection and treatment about IMD,and reviews the new techniques such as gene analysis,gene chip,organ transplantation and enzyme replacement therapy at the same time.

0.05)than 20 of those well-cared patients.Conclusion:The results from the tests by the YWG neuropsychological training system indicate it can demonstrate the severity of the schizophrenia and the cognitive dysfunction of the schizophrenics;therefore,these tests can be applied to clinic.

Protein tyrosine phosphatase (PTP) 1B is a potential target for the treatment of diabetes and obesity. We have previously identified the benzoyl sulfathiazole derivative II as a non-competitive PTP1B inhibitor with in vivo insulin sensitizing effects. Preliminary SAR study on this compound series has been carried out herein, and thirteen new compounds have been designed and synthesized. Among them, compound 10 exhibited potent inhibition against human recombinant PTP1B with the IC50 value of 3.97 micromol x L(-1), and is comparable to that of compound II.
Humans ; Protein Tyrosine Phosphatase, Non-Receptor Type 1 ; antagonists & inhibitors ; Structure-Activity Relationship ; Sulfathiazoles ; chemistry ; pharmacology

Objective To investigate the immunogenicity and cross protective effects of two novel HCV DNA vaccines in a mice model.Methods Two self-replicating alphavirus vector-based HCV DNA vaccines, pSCK CE1E2Y and pSCK H155, were constructed based on the genes encoding the structural pro-teins (Core, E1 and E2) and structural and NS3 fusion proteins (Core, E1 , E2 and NS3) of a HCV strain isolated from a Chinese patient (genotype 1b, Hebei strain), respectively.Western blot analysis was per-formed to detect the expression of fusion antigens.The BALB/c mice were intradermally immunized with the recombinant DNA vaccines by using electroporation.The immune responses induced in mice and the cross protective effects of the recombinant DNA vaccines were evaluated.Results The DNA vaccines effectively expressed the target antigens in vitro.The antigen-specific antibody responses and specific T cell immune re-sponses were induced in mice by the immunization of replicative DNA vaccines.However, no effective cross protection was provided by either of the DNA vaccines in the surrogate challenge model based on a recombi-nant heterologous HCV (JFH1, 2a) vaccinia virus strain.Conclusion Although no effective cross protec-tion was observed, both of the two replicative DNA vaccines could induce strong humoral and cellular im-mune responses against multi-target antigens of HCV strains.This study has paved the way for further inves-tigation on the development of novel HCV vaccines.

Poly(ADP-ribose)polymerase-1 (PARP-1) plays a significant role in the DNA repair process by catalyzing the transfer of ADP-ribose from NAD+ to its receptors. It is a promising anticancer drug target and many PARP-1 inhibitors have been developed and used in the clinical trial. In this work, a series of 3-(2-oxo-2-substituted acetamido)benzamides have been synthesized and their inhibitory activities against PARP-1 were evaluated. Of all the tested compounds, six compounds displayed inhibitory activities with IC50 values ranging from 0.23 to 5.78 µmol.L-1 . The binding pose of compound 5a was predicted using molecular docking to facilitate further structural modification.
Antineoplastic Agents ; Benzamides ; chemistry ; DNA Repair ; Drug Design ; Humans ; Molecular Docking Simulation ; Poly(ADP-ribose) Polymerase Inhibitors ; chemical synthesis ; chemistry ; Poly(ADP-ribose) Polymerases

Adenoma, Sweat Gland ; pathology ; Adult ; Female ; Humans ; Immunohistochemistry ; Leg ; pathology ; Skin Neoplasms ; pathology

The essential medium of B115 composed of 1% tryptone, 0.25% yeast extract and 0.5% sodium chloride was determined by using an orthogonal design. The orthogonal design was also employed in testing the optimum additions. It was composed of 0.1%(NH_(4))_(2)SO_(4),1.4%K_(2)HPO_(4), 0.6% KH_(2)PO_(4) and 0.1% (Na_(3)C_(6)H_(5)O_(7)). The yield of B115 cultured in optimum medium was compared with the one in essential medium. Statistic analysis showed that the growth of B115 was most significantly improved by adding K~(+)、NH~+_(4) and (Na_(3)C_(6)H_(5)O_(7)) to essential medium. The antibacterial effect of Bacillus subtilis strain B115 on pathogenic Aeromonas was studied. The results showed different antibacterial effects of B115 on different aeromonads. There were obvious antibacterial effects on BSK-10 and CL990920, while no effect on the growth of TL970424.

Objective To investigate the full-term newborn′s weight in Zhengzhou city and nearby areas around Zhengzhou in Henan province.Methods Each group newborn′s weight was divided with sex and city.We studied the regularity of full-term newborn′s weight,and examined the cause of the newborn′s weight rising.Results The average newborn′s weight in Zhengzhou was (3449.06?453.97) g,which in nearby areas around Zhengzhou was (3352.07?429.91) g.The average newborn′s weight in Zhengzhou was 86.97 g higher than other cities (P

Objective To evaluate the short and long term efficacy of self-expandable metallic stent implantation through bronchoscope in the management of the patients with malignant airway stenosis.Methods Seventy-four hospitalized patients with malignant airway stenosis during a 10-year period were selected as our subjects from Dec.2002 to Nov.2012 in the Respiratory Department of the Central Hospital of Wuhan.All these patients underwent bronchoscope for nitinol self-expandable metallic stent implantation.Clinical improvement,6min walking distance (6MWT),mMRC index,arterial blood gas,as well as early and late postoperative complications were investigated.Results A total of 77 stents were implanted,including 34 covered metallic stents and 43 uncovered metallic stents.All patients showed the obvious relief of dyspnea after the operation.6MWT at pre-operation was (172 ± 45)m and (288 ± 36)m at 28 week post-operation.Arterial blood gas was (61.34 ±6.44)mmHg at pre-opemtion and (72.12 ±6.87) mmHg at 28 week post-operation.mMRC was 3.56 ± 0.44 at pre-operation and 1.96 ± 0.37 at 28 week post-operation.All these differences were significant(P ＜ 0.01).The operation succeeded in releasing bucking of patients with esophago-tracheal fistula.Symptoms such as fever,cough,pharyngodynia,foreign body sensation and hemoptysis were observed,and all the above side effect could be solved while no serious complications arose.Conclusion Bronchoscopic insertion of metallic airway stents for malignant airway stenosis is a safe and effective procedure that could obviously relieve respiratory distress and improve the quality of life.

Objective To investigate the development strategy of novel T cell based vaccine against HCV infection.Methods BALB/c mice were primed with pSCK-based DNA vaccine and boosted with type 5 adenoviral vector-based vaccine, which expressed the structural proteins ( Core, E1 and E2) de-rived from a Chinese HCV patient (genotype 1b, Hebei strain).Enzyme linked immunospot assay (ELIS-POT) and intracellular cytokine staining ( ICS) were used to analyze the elicited antigen-specific immune re-sponses and the efficacy of cross-protection.Results Immunization of mice with the prime-boost vaccination strategy elicited stronger T cell immune responses against multiple HCV antigens than using the DNA vac-cines alone, especially the IFN-γ-secreting T cell responses against E1 protein as indicated by ELISPOT as-say.ICS data indicated that the prime-boost regimen elicited more TNF-α-producing CD4+and IFN-γ-produ-cing CD8+T cells against E1 protein and high levels of IFN-γ-producing CD4+and CD8+T cells against E2 protein in comparison with immunization with DNA vaccines.Moreover, the prime-boost vaccination was ca-pable of eliciting effective cross-protection in a surrogate challenge model based on a recombinant heterolo-gous HCV (JFH1, 2a) vaccinia virus.Conclusion The prime-boost vaccination using DNA and rAd5-based vaccine expressing HCV structural antigens induced significant cellular immune response and cross-protection in mice, suggesting the possibility of using it as a promising T cell based vaccine against HCV in-fection.