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Loss of podocalyxin causes a novel syndromic type of congenital nephrotic syndrome
강희경,이모세,이경분,Michael Hughes,권보상,이상문,Kelly M McNagny,안요한,고정민,하일수,최무림,정해일 생화학분자생물학회 2017 Experimental and molecular medicine Vol.49 No.-
Many cellular structures directly imply specific biological functions. For example, normal slit diaphragm structures that extend from podocyte foot processes ensure the filtering function of renal glomeruli. These slits are covered by a number of surface proteins, such as nephrin, podocin, podocalyxin and CD2AP. Here we report a human patient presenting with congenital nephrotic syndrome, omphalocele and microcoria due to two loss-of-function mutations in PODXL, which encodes podocalyxin, inherited from each parent. This set of symptoms strikingly mimics previously reported mouse Podxl−/− embryos, emphasizing the essential function of PODXL in mammalian kidney development and highlighting this patient as a human PODXL-null model. The results underscore the utility of current genomics approaches to provide insights into the genetic mechanisms of human disease traits through molecular diagnosis.
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Sheka Yagub Aloyouni,Charis-Patricia Segeritz,Ashley M. Sherrid,Matthew J. Gold,Daniela I. M. Loeffler,Marie-Renée Blanchet,Bing Cai,Jeremy Hirota,Kelly M. McNagny,Tobias R. Kollmann 대한천식알레르기학회 2014 Allergy, Asthma & Immunology Research Vol.6 No.4
Purpose: Asthma is a chronic respiratory disorder that leads to inflammation and narrowing of the airways. Its global prevalence has attained epidemiclevels and treatment options that reach beyond temporary relief of symptoms are urgently needed. Since the processes leading to clinicallysymptomatic asthma start early in life, we set out to systematically evaluate a neonatal immunotherapeutic based on Listeria monocytogenes (Lm)for the control of allergic sensitization. Methods: We modified Lm to express the model allergen, ovalbumin (OVA), and tested the ability of neonatalimmunization with this strain to control allergic sensitization in a mouse model of OVA-induced asthma. Mice were immunized as newborns withlive or heat killed LmOVA or live Lm, followed 6 weeks later by allergic sensitization with OVA. In order to determine whether the TH1-polarizing effectof this vaccine vector inadvertently may exacerbate development of certain TH1-driven allergic diseases, mice immunized as newborns were assessedin a model of adult hypersensitivity pneumonitis (HP). Results: Both LmOVA and Lm-control vaccines were highly effective in providinglong-lasting protection from airway inflammation after only one immunization given perinatally. Serum antibody levels and lung cytokine productionsuggest that this prophylactic strategy is associated with an allergen specific TH1-dominated response. Specifically, LmOVA vaccinated mice displayedsignificantly elevated OVA-specific serum IgG2a, but no difference in anti-OVA IgE antibodies and only slightly decreased anti-OVA IgG1 antibodies. Importantly, Lm-based neonatal vaccination did not exacerbate Th1/Th17 driven HP, arguing against broad spectrum immune skewing. Conclusions:Our findings highlight the promise of early life Lm-based immunomodulatory interventions as a prophylactic strategy for allergic asthma.
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