کد مقاله | کد نشریه | سال انتشار | مقاله انگلیسی | نسخه تمام متن |
---|---|---|---|---|
2816633 | 1159946 | 2014 | 5 صفحه PDF | دانلود رایگان |
![عکس صفحه اول مقاله: Lysosome-associated protein 1 (LAMP-1) and Lysosome-associated protein 2 (LAMP-2) in a larger family carrier of Fabry disease Lysosome-associated protein 1 (LAMP-1) and Lysosome-associated protein 2 (LAMP-2) in a larger family carrier of Fabry disease](/preview/png/2816633.png)
• Lymphocytes expressed the highest levels of LAMP-1 than LAMP-2.
• Phagocytes expressed the highest levels of LAMP-2 than LAMP-1.
• FD patients' phagocytes expressed more LAMP-2 than the controls' phagocytes.
• LAMP-2 expression in phagocytes also diminished continuously over ERT.
This study investigated the potential relationship between the expression levels of lysosome-associated membrane proteins (LAMP) 1 and 2 and responses to enzyme replacement therapy (ERT) in the members of a single family with Fabry disease (FD). LAMP levels were assessed by flow cytometry in leukocytes from 17 FD patients who received an eight-month course of ERT course and 101 healthy individuals. We found that phagocytic cells from the FD patients had higher expression levels of both LAMP-1 and LAMP-2, relative to the levels in phagocytes from the healthy controls (p = 0.001). Furthermore, the LAMP-1 and LAMP-2 levels in phagocytes from the FD carriers continuously decreased with ERT administration to reach levels similar to those in healthy controls. We suggest that LAMP-1 and LAMP-2 could be used as additional markers with which to assess ERT effectiveness in FD.
Journal: Gene - Volume 536, Issue 1, 15 February 2014, Pages 118–122