منابع مشابه
Accumulation of mutant lamin A causes progressive changes in nuclear architecture in Hutchinson-Gilford progeria syndrome.
Hutchinson-Gilford progeria syndrome (HGPS) is a premature aging disorder, commonly caused by a point mutation in the lamin A gene that results in a protein lacking 50 aa near the C terminus, denoted LADelta50. Here we show by light and electron microscopy that HGPS is associated with significant changes in nuclear shape, including lobulation of the nuclear envelope, thickening of the nuclear l...
متن کاملProgressive vascular smooth muscle cell defects in a mouse model of Hutchinson-Gilford progeria syndrome.
Children with Hutchinson-Gilford progeria syndrome (HGPS) suffer from dramatic acceleration of some symptoms associated with normal aging, most notably cardiovascular disease that eventually leads to death from myocardial infarction and/or stroke usually in their second decade of life. For the vast majority of cases, a de novo point mutation in the lamin A (LMNA) gene is the cause of HGPS. This...
متن کاملSevere Osteolysis of the Mandibular Angle and Total Condylolysis in Progressive Systemic Sclerosis
Progressive systemic sclerosis is an autoimmune disease characterized by gradual deposition of abnormal collagen which causes fibrous changes of the skin and visceral organs such as lung, heart, and kidney, which lead to serious complication. Microangiopathy and firm skin cause extrinsic pressure and obliteration of the vessels that lead to ischemia and destruction of the underlying bone. Saliv...
متن کاملHutchinson-Gilford Progeria Syndrome
The Hutchinson-Gilford syndrome or progeria is a laminopathy generated by mutations that affect LMNA gene. This produces an abnormal protein named progerine which alters the formation of the cellular membrane inducing premature aging of all cells. In the present review aspects related to the pathophysiology and clinical characteristics of this syndrome are shown.
متن کاملCurrent concepts in osteolysis.
The most frequent cause of failure after total hip replacement in all reported arthroplasty registries is peri-prosthetic osteolysis. Osteolysis is an active biological process initiated in response to wear debris. The eventual response to this process is the activation of macrophages and loss of bone. Activation of macrophages initiates a complex biological cascade resulting in the final commo...
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ژورنال
عنوان ژورنال: American Journal of Roentgenology
سال: 1967
ISSN: 0361-803X,1546-3141
DOI: 10.2214/ajr.100.1.75