The zinc metalloprotease ZMPSTE24 plays a critical role in nuclear lamin biology by cleaving the prenylated and carboxylmethylated 15-amino acid tail from the C-terminus of prelamin A to yield mature lamin A. A defect in this proteolytic event, caused by a mutation in the lamin A gene (LMNA) that eliminates the ZMPSTE24 cleavage site, underlies the premature aging disease Hutchinson-Gilford Pro...

Mutants of lamin A cause diseases including the Hutchinson-Gilford progeria syndrome (HGPS) characterized by premature aging. Lamin A undergoes a series of processing reactions, including farnesylation and proteolytic cleavage of the farnesylated C-terminal domain. The role of cleavage is unknown but mutations that affect this reaction lead to progeria. Here we show that interphase serine 22 ph...

يمسج للاتعا نوسينشتاه ايريجورب دروفليج ةمزلاتم دعت يفحق بسانت مدعب فصتي .دللجا ةشاشهل مزلام رطيسم .رويطلا هطقتلا روهظو ،كفلا رغصو ،يننستلا رخأتو ،يهجو ينج يف افون يد ينج ةرفط وه يسيئرلا يثارولا بيعلا نأ امك 16 رمعلا نم غلبي ضيرم ةلاح ريرقتلا اذه فصي .LMNA يذلاو نوسينشتاه ايريجورب دروفليج ضارعأ روهظ عم ماع ضارعأ ترهظ امك .ريبك لكشب تايبدلأا يف ةلالحا فصو تم ةفلتخلما تاصيخشتلا ةشقانم تم امك .ضيرل...

Hutchinson-Gilford Progeria (HGPS) and Werner syndromes are diseases that clinically resemble some aspects of accelerated aging. HGPS is caused by mutations in theLMNA gene resulting in post-translational processing defects that trigger Progeria in children. Werner syndrome, arising from mutations in the WRN helicase gene, causes premature aging in young adults. What are the molecular mechanism...

The mutation responsible for Hutchinson Gilford Progeria Syndrome (HGPS) causes abnormal nuclear morphology. Previous studies show that free radicals and reactive oxygen species play major roles in the etiology and/or progression of neurodegenerative diseases and aging. This study compares oxidative stress responses between progeric and normal fibroblasts. Our data revealed higher ROS levels in...

Cell biologists love to think outside the box, pursuing many surprising twists and unexpected turns in their quest to unravel the mysteries of how cells work. But can cell biologists think outside the bench? We are certain that they can, and clearly some already do. To encourage more cell biologists to venture into the realm of translational research on a regular basis, we would like to share a...