When lamins go bad: nuclear structure and disease

KH Schreiber, BK Kennedy - Cell, 2013 - cell.com
Mutations in nuclear lamins or other proteins of the nuclear envelope are the root cause of a
group of phenotypically diverse genetic disorders known as laminopathies, which have …

Mouse models of laminopathies

H Zhang, JE Kieckhaefer, K Cao - Aging cell, 2013 - Wiley Online Library
The A‐and B‐type lamins are nuclear intermediate filament proteins in eukaryotic cells with
a broad range of functions, including the organization of nuclear architecture and interaction …

Identification of mitochondrial dysfunction in Hutchinson–Gilford progeria syndrome through use of stable isotope labeling with amino acids in cell culture

J Rivera-Torres, R Acin-Perez, P Cabezas-Sánchez… - Journal of …, 2013 - Elsevier
Hutchinson–Gilford progeria syndrome (HGPS) is a rare segmental premature aging
disorder that recapitulates some biological and physical aspects of physiological aging. The …

Lipodystrophy-Linked LMNA p.R482W Mutation Induces Clinical Early Atherosclerosis and In Vitro Endothelial Dysfunction

G Bidault, M Garcia, MC Vantyghem… - … and Vascular Biology, 2013 - Am Heart Assoc
Objective—Some mutations in LMNA, encoding A-type lamins, are responsible for Dunnigan-
type-familial partial lipodystrophy (FPLD2), with altered fat distribution and metabolism. The …

Arterial ageing

SJ Lee, SH Park - Korean circulation journal, 2013 - synapse.koreamed.org
Arterial ageing is characterized by age associated degeneration and sclerosis of the media
layer of the large arteries. However, besides ageing, clinical conditions, which enhance …

Disruption of lamin B1 and lamin B2 processing and localization by farnesyltransferase inhibitors

SA Adam, V Butin-Israeli, MM Cleland, T Shimi… - Nucleus, 2013 - Taylor & Francis
Lamin A and the B-type lamins, lamin B1 and lamin B2, are translated as pre-proteins that
are modified at a carboxyl terminal CAAX motif by farnesylation, proteolysis and …

Targeting protein prenylation in progeria

SG Young, SH Yang, BSJ Davies, HJ Jung… - Science translational …, 2013 - science.org
A clinical trial of a protein farnesyltransferase inhibitor (lonafarnib) for the treatment of
Hutchinson-Gilford progeria syndrome (HGPS) was recently completed. Here, we discuss …

Use of microarray hybrid capture and next-generation sequencing to identify the anatomy of a transgene

AJ DuBose, ST Lichtenstein, N Narisu… - Nucleic Acids …, 2013 - academic.oup.com
Transgenic animals are extensively used to model human disease. Typically, the transgene
copy number is estimated, but the exact integration site and configuration of the foreign DNA …

Synthesis of heterocyclic compounds through palladium-catalyzed C–H cyclization processes

K Inamoto - Chemical and Pharmaceutical Bulletin, 2013 - jstage.jst.go.jp
Herein, we describe our development of synthetic methods for heterocyclic compounds
based on the palladium-catalyzed carbon–hydrogen bond (C–H) functionalization …

Ring‐Expansion Reaction of Isatins with Ethyl Diazoacetate Catalyzed by Dirhodium (II)/DBU Metal‐Organic System: En Route to Viridicatin Alkaloids

R Paterna, V André, MT Duarte… - European Journal of …, 2013 - Wiley Online Library
We present here the NHC‐dirhodium (II)/DBU‐catalyzed ring expansion reaction of isatins
with ethyl diazoacetate. This new one‐pot protocol yields the ethyl 3‐hydroxy‐2 (1H) …