کد مقاله کد نشریه سال انتشار مقاله انگلیسی نسخه تمام متن
2839462 1165151 2006 8 صفحه PDF دانلود رایگان
عنوان انگلیسی مقاله ISI
Defective translation initiation causes vanishing of cerebral white matter
موضوعات مرتبط
علوم زیستی و بیوفناوری بیوشیمی، ژنتیک و زیست شناسی مولکولی پزشکی مولکولی
پیش نمایش صفحه اول مقاله
Defective translation initiation causes vanishing of cerebral white matter
چکیده انگلیسی

Leukoencephalopathy with vanishing white matter (VWM) is one of the most prevalent inherited white-matter disorders, especially in Caucasian populations. VWM is unusual because of its sensitivity to febrile infections and minor head trauma. The basic defect of this enigmatic brain disease resides in the regulation of initiation of protein synthesis. Recently, undue activation of the unfolded-protein response has emerged as an important factor in the pathophysiology of VWM. Here, we discuss the mechanisms that might be responsible for the selective involvement of the brain white matter in VWM. At present, VWM research is in need of an animal model to study disease mechanisms and therapeutic interventions.

ناشر
Database: Elsevier - ScienceDirect (ساینس دایرکت)
Journal: - Volume 12, Issue 4, April 2006, Pages 159–166
نویسندگان
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