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CASE REPORT : Melkersson-Rosenthal Syndrome with Genitalia Involved in a 12-Year-Old Boy
( Zhaowei Chu ),( Yanting Liu ),( Huan Zhang ),( Weihui Zeng ),( Songmei Geng ) 대한피부과학회 2016 Annals of Dermatology Vol.28 No.2
Melkersson.Rosenthal syndrome (MRS) is an uncommon granulomatous disease characterized by the triad of relapsing facial paralysis, orofacial swelling, and fissured tongue. Genital swelling in MRS is rarely reported. We presented the first case of complete MRS with genital swelling in a child. Biopsy examinations of both the child`s lower lip and penis showed noncaseating granuloma and intralymphatic granuloma infiltration. No symptoms or signs of other systemic disease (Crohn`s disease or sarcoidosis) were observed after 2 years of follow-up. Genetic screening for CARD15/NOD2 in this patient showed negative, which further confirmed the diagnosis of MRS. Eleven other cases of suspected complete or incomplete MRS with genitalia involved were reviewed. Our case emphasizes the specific clinical feature of MRS with genitalia involved, which was genetically different from Crohn’s disease and could be an independent entity. Lymphatic obstruction is responsible for localized edema in MRS. (Ann Dermatol 28(2) 232∼236, 2016)
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Regulatory Role of PopN and Its Interacting Partners in Type III Secretion of Pseudomonas aeruginosa
Yang, Hongjing,Shan, Zhiying,Kim, Jaewha,Wu, Weihui,Lian, Wei,Zeng, Lin,Xing, Laijun,Jin, Shouguang American Society for Microbiology 2007 Journal of Bacteriology Vol.189 No.7
<B>ABSTRACT</B><P>The type III secretion system (T3SS) of <I>Pseudomonas aeruginosa</I> plays a significant role in pathogenesis. We have previously identified type III secretion factor (TSF), which is required for effective secretion of the type III effector molecules, in addition to the low calcium signal. TSF includes many low-affinity high-capacity calcium binding proteins, such as serum albumin and casein. A search for the TSF binding targets on the bacterial outer membrane resulted in identification of PopN, a component of the T3SS that is readily detectable on the bacterial cell surface. PopN specifically interacts with Pcr1, and both <I>popN</I> and <I>pcr1</I> mutants have a constitutive type III secretion phenotype, suggesting that the two proteins form a complex that functions as a T3SS repressor. Further analysis of the <I>popN</I> operon genes resulted in identification of protein-protein interactions between Pcr1 and Pcr4 and between Pcr4 and Pcr3, as well as between PopN and Pcr2 in the presence of PscB. Unlike <I>popN</I> and <I>pcr1</I> mutants, <I>pcr3</I> and <I>pcr4</I> mutants are totally defective in type III secretion, while a <I>pcr2</I> mutant exhibits reduced type III secretion. Interestingly, PopN, Pcr1, Pcr2, and Pcr4 are all secreted in a type III secretion machinery-dependent manner, while Pcr3 is not. These findings imply that these components have important regulatory roles in controlling type III secretion.</P>
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