کد مقاله کد نشریه سال انتشار مقاله انگلیسی نسخه تمام متن
1951765 1537964 2006 10 صفحه PDF دانلود رایگان
عنوان انگلیسی مقاله ISI
Get the balance right: Mitofusins roles in health and disease
موضوعات مرتبط
علوم زیستی و بیوفناوری بیوشیمی، ژنتیک و زیست شناسی مولکولی زیست شیمی
پیش نمایش صفحه اول مقاله
Get the balance right: Mitofusins roles in health and disease
چکیده انگلیسی

Mitochondria are highly dynamic organelles exhibiting an elaborate morphology and fine structure. Fusion and fission processes contribute to the maintenance and dynamics of mitochondrial morphology. The Mitofusins, a class of evolutionary conserved GTPases of the mitochondrial outer membrane, are essential for the controlled fusion of mitochondrial membranes. Genetic and biochemical data propose a model in which functional domains, such as the GTPase domain and the C-terminally located coiled coil structure, act in an orchestrated manner to coordinate the tethering and mitochondrial outer membrane fusion. In addition, recent reports shed new light on the physiological importance of Mitofusin function suggesting a role in mitochondrial metabolism, apoptosis as well as cellular signalling. Mutations identified in the human Mfn2 gene from patients with the peripheral neuropathy Charcot–Marie–Tooth Type 2A invoke a direct correlation between mitochondrial morphology and function.

ناشر
Database: Elsevier - ScienceDirect (ساینس دایرکت)
Journal: Biochimica et Biophysica Acta (BBA) - Molecular Cell Research - Volume 1763, Issues 5–6, May–June 2006, Pages 490–499
نویسندگان
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