کد مقاله | کد نشریه | سال انتشار | مقاله انگلیسی | نسخه تمام متن |
---|---|---|---|---|
5905164 | 1159854 | 2016 | 9 صفحه PDF | دانلود رایگان |
عنوان انگلیسی مقاله ISI
Extensive macrophage accumulation in young and old Niemann-Pick C1 model mice involves the alternative, M2, activation pathway and inhibition of macrophage apoptosis
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کلمات کلیدی
enzyme-liked immunosorbent assayGM-CSFS1PHPBCDH & EBALNiemann–Pick C1GFAPNSENPC2NPC1PBSNeuron-specific enolase - enolase اختصاصی نورونLung pathology - آسیب شناسی ریهSphingosine-1-phosphate - اسپینگسین-1-فسفاتTerminal deoxynucleotidyl transferase-mediated dUTP nick end labeling - ترمینال deoxynucleotidyl transferase-mediated dUTP نام نهایی پایان نامهELISA - تست الیزاTUNEL - تونلstandard error of the mean - خطای استاندارد میانگینbronchoalveolar lavage - لارو برونکلو آلوئولارFoamy macrophages - ماکروفاژ فوئیgranulocyte macrophage colony-stimulating factor - ماکروفاژ گرانولوسیت عامل کلونی تحریک کنندهPhosphate-buffered saline - محلول نمک فسفات با خاصیت بافریSEM - مدل معادلات ساختاری / میکروسکوپ الکترونی روبشیPAS - نهHematoxylin and Eosin - هماتوکسیلین و ائوزینhydroxypropyl-beta-cyclodextrin - هیدروکسی پروپیل بتا سیکلوکودکسترینglial fibrillary acid protein - پروتئین اسید فیبریلیری گلیالcholesterol - کلسترول
موضوعات مرتبط
علوم زیستی و بیوفناوری
بیوشیمی، ژنتیک و زیست شناسی مولکولی
ژنتیک
پیش نمایش صفحه اول مقاله

چکیده انگلیسی
We have studied the pathophysiology of lung disease which occurs in two mouse models of Niemann-Pick C1 disease. We utilized Npc1â/â mice transgenic for normal gene expression in glia or neurons and glia at ages several fold the usual and a mouse model of the juvenile form of NPC1, a point mutation, at one age to confirm some findings. Lung weights, as per cent of body weight, increase much more than liver and spleen weights. Although pulmonary function parameters only vary for hysteresis between young and older Npc1â/â mice, they are markedly different than those found in normal control mice. Cholesterol accumulation continued in the older mice but sphingosine-1-phosphate was not increased. Bronchoalveolar lavage (BAL) showed a massive increase (26Â Ã) in the number of macrophages. Histologic examination from the older, transgenic Npc1â/â mice showed small foci of alveolar proteinosis and evidence of hemorrhage, as well as dense macrophage accumulation. A large subset of macrophages was immunopositive for Fizz1 or arginase-1, markers of the alternative activation pathway, while no Fizz1 or arginase-1 positive macrophages were found in wild-type mice. The percentage of marker positive macrophages was relatively stable at 5-10% at various ages and within the 2 transgenic models. Phosphohistone H3 and Ki67 showed low levels of proliferation of these macrophages. Apoptosis was prominent within lung capillary endothelial cells, but limited within macrophages. Thus, activation of the alternative pathway is involved in Niemann-Pick C1 associated pulmonary macrophage accumulation, with low proliferation of these cells balanced by low levels of apoptosis.
ناشر
Database: Elsevier - ScienceDirect (ساینس دایرکت)
Journal: Gene - Volume 578, Issue 2, 10 March 2016, Pages 242-250
Journal: Gene - Volume 578, Issue 2, 10 March 2016, Pages 242-250
نویسندگان
Gail Deutsch, Akshay Muralidhar, Ellen Le, Ivan A. Borbon, Robert P. Erickson,