کد مقاله | کد نشریه | سال انتشار | مقاله انگلیسی | نسخه تمام متن |
---|---|---|---|---|
8301784 | 1537707 | 2016 | 9 صفحه PDF | دانلود رایگان |
عنوان انگلیسی مقاله ISI
Characterization of a mutant form of human apolipoprotein B (Thr26_Tyr27del) associated with familial hypobetalipoproteinemia
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کلمات کلیدی
DMEMFBSHDLFHBLVLDLDulbecco's modified Eagle's medium - Medal of Eagle اصلاح شده Dulbeccoapo - آپوapolipoprotein - آپولیپوپروتئینProteasomal degradation - تخریب پروتئازوماfetal bovine serum - سرم جنین گاوendoplasmic reticulum - شبکه آندوپلاسمی high density lipoprotein - لیپوپروتئین با چگالی بالاvery low density lipoprotein - لیپوپروتئین چگالی بسیار کم استlow density lipoprotein - لیپوپروتئین چگالی کمLDL - لیپوپروتئین کم چگالی(کلسترول بد)Hypobetalipoproteinemia - هیپوتالپپروتئینمیFamilial hypobetalipoproteinemia - هیپوتالپپروتئینمی خانوادگیpenicillin–streptomycin - پنی سیلین-استرپتومایسین
موضوعات مرتبط
علوم زیستی و بیوفناوری
بیوشیمی، ژنتیک و زیست شناسی مولکولی
زیست شیمی
پیش نمایش صفحه اول مقاله
![عکس صفحه اول مقاله: Characterization of a mutant form of human apolipoprotein B (Thr26_Tyr27del) associated with familial hypobetalipoproteinemia Characterization of a mutant form of human apolipoprotein B (Thr26_Tyr27del) associated with familial hypobetalipoproteinemia](/preview/png/8301784.png)
چکیده انگلیسی
We have previously identified a deletion mutant of human apoB [apoB (Thr26_Tyr27del)] in a subject with primary hypobetalipoproteinemia. The present study determined the effect of Thr26_Tyr27del mutation on apoB secretion using transfected McA-RH7777 cells. Transient or stable transfection of apoB-48 containing the Thr26_Tyr27del mutation showed drastically reduced secretion of the mutant as compared to wild-type apoB-48. No lipoproteins containing the mutant apoB-48 were secreted into the medium. Incubation of transfected cells in a lipid-rich medium in the presence of cycloheximide showed rapid turnover of cell-associated mutant apoB-48 as compared to that of wild-type apoB-48. Immunofluorescence experiments showed that the mutant apoB-48 was mostly localized in the endoplasmic reticulum. Treatment with the proteasomal inhibitor MG132 markedly attenuated the turnover of cell-associated mutant apoB-48, whereas treatment with inhibitors of autophagosomal/lysosomal function (e.g. 3-MA or ammonium chloride) had no effect. Taken together, these results indicated that the defective secretion of the Thr26_Tyr27del mutant was associated with increased intracellular degradation of apoB through the proteasome-dependent pathway.
ناشر
Database: Elsevier - ScienceDirect (ساینس دایرکت)
Journal: Biochimica et Biophysica Acta (BBA) - Molecular and Cell Biology of Lipids - Volume 1861, Issue 4, April 2016, Pages 371-379
Journal: Biochimica et Biophysica Acta (BBA) - Molecular and Cell Biology of Lipids - Volume 1861, Issue 4, April 2016, Pages 371-379
نویسندگان
Lucia Magnolo, Davide Noto, Angelo B. Cefalù, Maurizio Averna, Sebastiano Calandra, Zemin Yao, Patrizia Tarugi,