Hutchinson-Gilford Progeria Syndrome: A premature aging disease caused by LMNA gene mutations

S Gonzalo, R Kreienkamp, P Askjaer - Ageing research reviews, 2017 - Elsevier
Products of the LMNA gene, primarily lamin A and C, are key components of the nuclear
lamina, a proteinaceous meshwork that underlies the inner nuclear membrane and is …

Mechanisms of chromosome congression during mitosis

H Maiato, AM Gomes, F Sousa, M Barisic - Biology, 2017 - mdpi.com
Chromosome congression during prometaphase culminates with the establishment of a
metaphase plate, a hallmark of mitosis in metazoans. Classical views resulting from more …

Arterial calcification in diabetes mellitus: preclinical models and translational implications

JN Stabley, DA Towler - Arteriosclerosis, thrombosis, and vascular …, 2017 - Am Heart Assoc
Diabetes mellitus increasingly afflicts our aging and dysmetabolic population. Type 2
diabetes mellitus and the antecedent metabolic syndrome represent the vast majority of the …

Nuclear envelopathies: a complex LINC between nuclear envelope and pathology

A Janin, D Bauer, F Ratti, G Millat, A Méjat - Orphanet journal of rare …, 2017 - Springer
Since the identification of the first disease causing mutation in the gene coding for emerin, a
transmembrane protein of the inner nuclear membrane, hundreds of mutations and variants …

A tissue engineered blood vessel model of Hutchinson-Gilford progeria syndrome using human iPSC-derived smooth muscle cells

L Atchison, H Zhang, K Cao, GA Truskey - Scientific reports, 2017 - nature.com
Abstract Hutchison-Gilford Progeria Syndrome (HGPS) is a rare, accelerated aging disorder
caused by nuclear accumulation of progerin, an altered form of the Lamin A gene. The …

High-throughput imaging for the discovery of cellular mechanisms of disease

G Pegoraro, T Misteli - Trends in Genetics, 2017 - cell.com
High-throughput imaging (HTI) is a powerful tool in the discovery of cellular disease
mechanisms. While traditional approaches to identify disease pathways often rely on …

Biomechanical strain exacerbates inflammation on a progeria‐on‐a‐chip model

J Ribas, YS Zhang, PR Pitrez, J Leijten, M Miscuglio… - small, 2017 - Wiley Online Library
Organ‐on‐a‐chip platforms seek to recapitulate the complex microenvironment of human
organs using miniaturized microfluidic devices. Besides modeling healthy organs, these …

Chemical screening identifies ROCK as a target for recovering mitochondrial function in Hutchinson‐Gilford progeria syndrome

HT Kang, JT Park, K Choi, HJC Choi, CW Jung… - Aging cell, 2017 - Wiley Online Library
Hutchinson‐Gilford progeria syndrome (HGPS) constitutes a genetic disease wherein an
aging phenotype manifests in childhood. Recent studies indicate that reactive oxygen …

Current insights into LMNA cardiomyopathies: Existing models and missing LINCs

D Brayson, CM Shanahan - Nucleus, 2017 - Taylor & Francis
The nuclear lamina is a critical structural domain for the maintenance of genomic stability
and whole-cell mechanics. Mutations in the LMNA gene, which encodes nuclear A-type …

New tricks for human farnesyltransferase inhibitor: cancer and beyond

J Wang, X Yao, J Huang - Medchemcomm, 2017 - pubs.rsc.org
Human protein farnesyltransferase (FTase) catalyzes the addition of a C15-farnesyl lipid
group to the cysteine residue located in the COOH-terminal tetrapeptide motif of a variety of …