کد مقاله کد نشریه سال انتشار مقاله انگلیسی نسخه تمام متن
2132033 1086670 2007 13 صفحه PDF دانلود رایگان
عنوان انگلیسی مقاله ISI
Structural and functional analysis of the sarcoglycan–sarcospan subcomplex
موضوعات مرتبط
علوم زیستی و بیوفناوری بیوشیمی، ژنتیک و زیست شناسی مولکولی تحقیقات سرطان
پیش نمایش صفحه اول مقاله
Structural and functional analysis of the sarcoglycan–sarcospan subcomplex
چکیده انگلیسی

Sarcospan is a component of the dystrophin–glycoprotein complex that forms a tight subcomplex with the sarcoglycans. The sarcoglycan–sarcospan subcomplex functions to stabilize α-dystroglycan at the plasma membrane and perturbations of this subcomplex are associated with autosomal recessive limb-girdle muscular dystrophy. In order to characterize protein interactions within this subcomplex, we first demonstrate that sarcospan forms homo-oligomers within the membrane. Experiments with a panel of site-directed mutants reveal that proper structure of the large extracellular loop is an important determinant of oligo formation. Furthermore, the intracellular N- and C-termini contribute to stability of sarcospan-mediated webs. Point mutation of each cysteine residue reveals that Cys 162 and Cys 164 within the large extracellular loop form disulfide bridges, which are critical for proper sarcospan structure. The extracellular domain of sarcospan also forms the main binding site for the sarcoglycans. We propose a model whereby sarcospan forms homo-oligomers that cluster the components of the dystrophin–glycoprotein complex within the membrane.

ناشر
Database: Elsevier - ScienceDirect (ساینس دایرکت)
Journal: Experimental Cell Research - Volume 313, Issue 4, 15 February 2007, Pages 639–651
نویسندگان
, , , , ,