Protein farnesyltransferase inhibitors and progeria.

Nuclear lamins: key regulators of nuclear structure and activities. J Cell Mol Med (2009) 1.70

Treatment with a farnesyltransferase inhibitor improves survival in mice with a Hutchinson-Gilford progeria syndrome mutation. Biochim Biophys Acta (2007) 1.33

Farnesyl diphosphate analogues with omega-bioorthogonal azide and alkyne functional groups for protein farnesyl transferase-catalyzed ligation reactions. J Org Chem (2007) 1.21

Protein farnesylation inhibitors cause donut-shaped cell nuclei attributable to a centrosome separation defect. Proc Natl Acad Sci U S A (2011) 1.10

Absence of progeria-like disease phenotypes in knock-in mice expressing a non-farnesylated version of progerin. Hum Mol Genet (2010) 1.09

Prenylome profiling reveals S-farnesylation is crucial for membrane targeting and antiviral activity of ZAP long-isoform. Proc Natl Acad Sci U S A (2013) 0.99

Prediction and evaluation of protein farnesyltransferase inhibition by commercial drugs. J Med Chem (2010) 0.96

Farnesyltransferase inhibitor treatment restores chromosome territory positions and active chromosome dynamics in Hutchinson-Gilford progeria syndrome cells. Genome Biol (2011) 0.96

All-trans retinoic acid and rapamycin normalize Hutchinson Gilford progeria fibroblast phenotype. Oncotarget (2015) 0.85

Molecular ageing in progeroid syndromes: Hutchinson-Gilford progeria syndrome as a model. Immun Ageing (2009) 0.83

Increasing the length of progerin's isoprenyl anchor does not worsen bone disease or survival in mice with Hutchinson-Gilford progeria syndrome. J Lipid Res (2008) 0.80

Reversal of age-dependent nuclear morphology by inhibition of prenylation does not affect lifespan in Caenorhabditis elegans. Nucleus (2010) 0.77

Progeria: a rare genetic premature ageing disorder. Indian J Med Res (2014) 0.75

The knockout mouse project. Nat Genet (2004) 7.80

Glycosylphosphatidylinositol-anchored high-density lipoprotein-binding protein 1 plays a critical role in the lipolytic processing of chylomicrons. Cell Metab (2007) 5.04

BayGenomics: a resource of insertional mutations in mouse embryonic stem cells. Nucleic Acids Res (2003) 4.83

A public gene trap resource for mouse functional genomics. Nat Genet (2004) 3.96

Linking lipid metabolism to the innate immune response in macrophages through sterol regulatory element binding protein-1a. Cell Metab (2011) 3.49

The International Gene Trap Consortium Website: a portal to all publicly available gene trap cell lines in mouse. Nucleic Acids Res (2006) 3.40

Blocking protein farnesyltransferase improves nuclear blebbing in mouse fibroblasts with a targeted Hutchinson-Gilford progeria syndrome mutation. Proc Natl Acad Sci U S A (2005) 3.30

ESCRT-III dysfunction causes autophagosome accumulation and neurodegeneration. Curr Biol (2007) 3.27

Zmpste24 deficiency in mice causes spontaneous bone fractures, muscle weakness, and a prelamin A processing defect. Proc Natl Acad Sci U S A (2002) 3.26

Lamins A and C but not lamin B1 regulate nuclear mechanics. J Biol Chem (2006) 3.02

Chylomicronemia with a mutant GPIHBP1 (Q115P) that cannot bind lipoprotein lipase. Arterioscler Thromb Vasc Biol (2009) 2.99

Laminopathies and the long strange trip from basic cell biology to therapy. J Clin Invest (2009) 2.94

Lamin B1 is required for mouse development and nuclear integrity. Proc Natl Acad Sci U S A (2004) 2.89

Prelamin A, Zmpste24, misshapen cell nuclei, and progeria--new evidence suggesting that protein farnesylation could be important for disease pathogenesis. J Lipid Res (2005) 2.88

Blocking protein farnesyltransferase improves nuclear shape in fibroblasts from humans with progeroid syndromes. Proc Natl Acad Sci U S A (2005) 2.85

GPIHBP1 is responsible for the entry of lipoprotein lipase into capillaries. Cell Metab (2010) 2.75

A protein farnesyltransferase inhibitor ameliorates disease in a mouse model of progeria. Science (2006) 2.71

A farnesyltransferase inhibitor improves disease phenotypes in mice with a Hutchinson-Gilford progeria syndrome mutation. J Clin Invest (2006) 2.39

Heterozygosity for Lmna deficiency eliminates the progeria-like phenotypes in Zmpste24-deficient mice. Proc Natl Acad Sci U S A (2004) 2.39

Prelamin A and lamin A appear to be dispensable in the nuclear lamina. J Clin Invest (2006) 2.23

Antioxidants accelerate lung cancer progression in mice. Sci Transl Med (2014) 2.23

Lowering plasma cholesterol levels halts progression of aortic valve disease in mice. Circulation (2009) 2.20

Calcific aortic valve stenosis in old hypercholesterolemic mice. Circulation (2006) 2.13

GPIHBP1: an endothelial cell molecule important for the lipolytic processing of chylomicrons. Curr Opin Lipidol (2007) 2.01

Blocking VLDL secretion causes hepatic steatosis but does not affect peripheral lipid stores or insulin sensitivity in mice. J Lipid Res (2008) 1.91

The acidic domain of GPIHBP1 is important for the binding of lipoprotein lipase and chylomicrons. J Biol Chem (2008) 1.91

Functional coupling between the extracellular matrix and nuclear lamina by Wnt signaling in progeria. Dev Cell (2010) 1.90

Abnormal development of the cerebral cortex and cerebellum in the setting of lamin B2 deficiency. Proc Natl Acad Sci U S A (2010) 1.89

Targeting isoprenylcysteine methylation ameliorates disease in a mouse model of progeria. Science (2013) 1.88

Deficiencies in lamin B1 and lamin B2 cause neurodevelopmental defects and distinct nuclear shape abnormalities in neurons. Mol Biol Cell (2011) 1.86

Postprenylation CAAX processing is required for proper localization of Ras but not Rho GTPases. Mol Biol Cell (2005) 1.85

The posttranslational processing of prelamin A and disease. Annu Rev Genomics Hum Genet (2009) 1.81

Inactivation of Icmt inhibits transformation by oncogenic K-Ras and B-Raf. J Clin Invest (2004) 1.81

Reversal of hyperlipidemia with a genetic switch favorably affects the content and inflammatory state of macrophages in atherosclerotic plaques. Circulation (2011) 1.79

Abnormal patterns of lipoprotein lipase release into the plasma in GPIHBP1-deficient mice. J Biol Chem (2008) 1.73

Chylomicronemia elicits atherosclerosis in mice--brief report. Arterioscler Thromb Vasc Biol (2009) 1.72

Agpat6 deficiency causes subdermal lipodystrophy and resistance to obesity. J Lipid Res (2006) 1.68

Eliminating atherogenesis in mice by switching off hepatic lipoprotein secretion. Circulation (2003) 1.68

Mutation of conserved cysteines in the Ly6 domain of GPIHBP1 in familial chylomicronemia. J Lipid Res (2009) 1.67

Differential membrane localization of ERas and Rheb, two Ras-related proteins involved in the phosphatidylinositol 3-kinase/mTOR pathway. J Biol Chem (2005) 1.64

Agpat6--a novel lipid biosynthetic gene required for triacylglycerol production in mammary epithelium. J Lipid Res (2006) 1.62

Chylomicronemia with low postheparin lipoprotein lipase levels in the setting of GPIHBP1 defects. Circ Cardiovasc Genet (2010) 1.60

A small-molecule inhibitor of isoprenylcysteine carboxyl methyltransferase with antitumor activity in cancer cells. Proc Natl Acad Sci U S A (2005) 1.59

Apobec-1 protects intestine from radiation injury through posttranscriptional regulation of cyclooxygenase-2 expression. Gastroenterology (2004) 1.56

Absence of the CAAX endoprotease Rce1: effects on cell growth and transformation. Mol Cell Biol (2002) 1.56

Highly conserved cysteines within the Ly6 domain of GPIHBP1 are crucial for the binding of lipoprotein lipase. J Biol Chem (2009) 1.54

AGPAT6 is a novel microsomal glycerol-3-phosphate acyltransferase. J Biol Chem (2008) 1.50

CD1d function is regulated by microsomal triglyceride transfer protein. Nat Med (2004) 1.49

Disruption of the phosphatidylserine decarboxylase gene in mice causes embryonic lethality and mitochondrial defects. J Biol Chem (2005) 1.48

GGTase-I deficiency reduces tumor formation and improves survival in mice with K-RAS-induced lung cancer. J Clin Invest (2007) 1.47

Prelamin A farnesylation and progeroid syndromes. J Biol Chem (2006) 1.43

Compensatory increase in hepatic lipogenesis in mice with conditional intestine-specific Mttp deficiency. J Biol Chem (2005) 1.43

Filamin B deficiency in mice results in skeletal malformations and impaired microvascular development. Proc Natl Acad Sci U S A (2007) 1.41

Progerin elicits disease phenotypes of progeria in mice whether or not it is farnesylated. J Clin Invest (2008) 1.40

Mouse models of the laminopathies. Exp Cell Res (2007) 1.40

Normal binding of lipoprotein lipase, chylomicrons, and apo-AV to GPIHBP1 containing a G56R amino acid substitution. Biochim Biophys Acta (2007) 1.39

Macrophage-targeted overexpression of urokinase causes accelerated atherosclerosis, coronary artery occlusions, and premature death. Circulation (2004) 1.37

ATP-citrate lyase deficiency in the mouse. J Biol Chem (2003) 1.35

An absence of both lamin B1 and lamin B2 in keratinocytes has no effect on cell proliferation or the development of skin and hair. Hum Mol Genet (2011) 1.35

Increased progerin expression associated with unusual LMNA mutations causes severe progeroid syndromes. Hum Mutat (2007) 1.34

Treatment with a farnesyltransferase inhibitor improves survival in mice with a Hutchinson-Gilford progeria syndrome mutation. Biochim Biophys Acta (2007) 1.33

An accumulation of non-farnesylated prelamin A causes cardiomyopathy but not progeria. Hum Mol Genet (2010) 1.26