کد مقاله | کد نشریه | سال انتشار | مقاله انگلیسی | نسخه تمام متن |
---|---|---|---|---|
10756659 | 1050387 | 2013 | 6 صفحه PDF | دانلود رایگان |
عنوان انگلیسی مقاله ISI
Chorein, the protein responsible for chorea-acanthocytosis, interacts with β-adducin and β-actin
دانلود مقاله + سفارش ترجمه
دانلود مقاله ISI انگلیسی
رایگان برای ایرانیان
کلمات کلیدی
CHACMALDI-TOFPMFCBBHEK293PBS-TChoreinFBSVPS13AMEMEchorea-acanthocytosisCoomassie Brilliant Blue - Coomassie درخشان آبیβ-actin - β-اکتینPeptide mass fingerprint - اثر انگشت پپتیدهMinimum Essential Medium Eagle - حداقل عقاب متوسط ضروریmatrix-assisted laser desorption ionization-time of flight - زمان یونیزاسیون لیزر جذب لیزر ماتریس-زمان پروازfetal bovine serum - سرم جنین گاوCo-Immunoprecipitation - هم ایمن زداییhuman embryonic kidney 293 - کلیه جنینی انسان 293
موضوعات مرتبط
علوم زیستی و بیوفناوری
بیوشیمی، ژنتیک و زیست شناسی مولکولی
زیست شیمی
پیش نمایش صفحه اول مقاله
![عکس صفحه اول مقاله: Chorein, the protein responsible for chorea-acanthocytosis, interacts with β-adducin and β-actin Chorein, the protein responsible for chorea-acanthocytosis, interacts with β-adducin and β-actin](/preview/png/10756659.png)
چکیده انگلیسی
Chorea-acanthocytosis (ChAc) is an autosomal, recessive hereditary disease characterized by striatal neurodegeneration and acanthocytosis, and caused by loss of function mutations in the vacuolar protein sorting 13 homolog A (VPS13A) gene. VPS13A encodes chorein whose physiological function at the molecular level is poorly understood. In this study, we show that chorein interacts with β-adducin and β-actin. We first compare protein expression in human erythrocyte membranes using proteomic analysis. Protein levels of β-adducin isoform 1 and β-actin are markedly decreased in erythrocyte membranes from a ChAc patient. Subsequent co-immunoprecipitation (co-IP) and reverse co-IP assays using extracts from chorein-overexpressing human embryonic kidney 293 (HEK293) cells, shows that β-adducin (isoforms 1 and 2) and β-actin interact with chorein. Immunocytochemical analysis using chorein-overexpressing HEK293 cells demonstrates co-localization of chorein with β-adducin and β-actin. In addition, immunoreactivity of β-adducin isoform 1 is significantly decreased in the striatum of gene-targeted ChAc-model mice. Adducin and actin are membrane cytoskeletal proteins, involved in synaptic function. Expression of β-adducin is restricted to the brain and hematopoietic tissues, corresponding to the main pathological lesions of ChAc, and thereby implicating β-adducin and β-actin in ChAc pathogenesis.
ناشر
Database: Elsevier - ScienceDirect (ساینس دایرکت)
Journal: Biochemical and Biophysical Research Communications - Volume 441, Issue 1, 8 November 2013, Pages 96-101
Journal: Biochemical and Biophysical Research Communications - Volume 441, Issue 1, 8 November 2013, Pages 96-101
نویسندگان
Nari Shiokawa, Masayuki Nakamura, Mieko Sameshima, Akiko Deguchi, Takehiro Hayashi, Natsuki Sasaki, Akira Sano,