Metformin decreases progerin expression and alleviates pathological defects of Hutchinson–Gilford progeria syndrome cells
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چکیده
منابع مشابه
Inhibiting farnesylation of progerin prevents the characteristic nuclear blebbing of Hutchinson-Gilford progeria syndrome.
Hutchinson-Gilford progeria syndrome (HGPS) is a rare genetic disorder that is characterized by dramatic premature aging and accelerated cardiovascular disease. HGPS is almost always caused by a de novo point mutation in the lamin A gene (LMNA) that activates a cryptic splice donor site, producing a truncated mutant protein termed "progerin." WT prelamin A is anchored to the nuclear envelope by...
متن کاملEfficient progerin clearance through autophagy induction and SRSF-1 downregulation in Hutchinson-Gilford Progeria Syndrome
Hutchinson-Gilford progeria syndrome (HGPS) is an extremely rare premature and accelerated aging disease caused by a de novo point mutation in LMNA encoding A-type lamins. Progerin, a truncated and toxic form of prelamin A, accumulates in HGPS cells nuclei and is a hallmark of the disease. We show that progerin is sequestered, together with other proteins (lamins B1/B2, emerin), into abnormally...
متن کاملInterruption of progerin-lamin A/C binding ameliorates Hutchinson-Gilford progeria syndrome phenotype.
Hutchinson-Gilford progeria syndrome (HGPS) is a rare autosomal dominant genetic disease that is caused by a silent mutation of the LMNA gene encoding lamins A and C (lamin A/C). The G608G mutation generates a more accessible splicing donor site than does WT and produces an alternatively spliced product of LMNA called progerin, which is also expressed in normal aged cells. In this study, we det...
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Background: It is well known that there is a close relationship between elevated androgen plasma levels and the ultrasound findings of stromal hypertrophy in polycystic ovary syndrome (PCOS). The objective of this study was to investigate the effects metformin on the hyperandrogenism and ovarian volume in PCOS. Methods: The study is an unrandomized clinical trial with before–after desig...
متن کاملNaı̈ve adult stem cells from patients with Hutchinson-Gilford progeria syndrome express low levels of progerin in vivo
Vera Wenzel*, Daniela Roedl*, Diana Gabriel*, Leslie B. Gordon, Meenhard Herlyn, Reinhard Schneider, Johannes Ring and Karima Djabali Department of Dermatology and IMETUM, Technische Universität München, Munich, Germany Department of Pediatrics, Alpert Medical School of Brown University, and Hasbro Children’s Hospital, Providence, USA Molecular and Cellular Oncogenesis Program, The Wistar Insti...
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ژورنال
عنوان ژورنال: npj Aging and Mechanisms of Disease
سال: 2016
ISSN: 2056-3973
DOI: 10.1038/npjamd.2016.26