The Emerging Role of Lamin C as an Important LMNA Isoform in Mechanophenotype
Lamin A and lamin C isoforms of the gene LMNA are major structural and mechanotransductive components of the nuclear lamina. Previous reports have proposed lamin A as the isoform with the most dominant contributions to cellular mechanophenotype. Recently,
Rafael D. González-Cruz +3 more
doaj +1 more source
RNA Interference by Single- and Double-stranded siRNA With a DNA Extension Containing a 3′ Nuclease-resistant Mini-hairpin Structure [PDF]
Selective gene silencing by RNA interference (RNAi) involves double-stranded small interfering RNA (ds siRNA) composed of single-stranded (ss) guide and passenger RNAs.
Ahmed +38 more
core +2 more sources
Grancalcin (GCA), a myeloid‐derived protein, is enriched in gingival tissues of periodontitis patients and mouse models. Through interactions with CD44 and activation of MYH9, GCA promotes NF‐κB signaling and exacerbates periodontal inflammation and bone loss.
Min Zhou +6 more
wiley +1 more source
Nuclear shape is affected differentially by loss of lamin A, lamin C, or both lamin A and C
Lamin intermediate filaments form a peripheral meshwork to support nuclear shape and function. Knockout of the LMNA gene that encodes for both lamin A and C results in an abnormally shaped nucleus. To determine the relative contribution of lamin A and C to nuclear shape, we measured nuclear blebbing and circular deviation in separate lamin A and lamin ...
Pho, Mai +3 more
openaire +2 more sources
Mandibuloacral Dysplasia Caused by LMNA Mutations and Uniparental Disomy. [PDF]
Mandibuloacral dysplasia (MAD) is a rare autosomal recessive disorder characterized by postnatal growth retardation, craniofacial anomalies, skeletal malformations, and mottled cutaneous pigmentation.
Bai, Shaochun +5 more
core +3 more sources
NIBAN2/FLII/RREB1 Axis Drives Glioma Stem Cell Malignancy via TLR3 Pathway Activation
NIBAN2, highly expressed in glioma stem‐like cells (GSCs), assembles with FLII and transcription factor RREB1 to form a nuclear complex. This complex transcriptionally activates stemness‐associated genes (e.g., CD44, NANOG) and metabolic enzymes (e.g., LDHA), thereby sustaining both transcriptional and metabolic stemness programs.
Liang liang Shi +14 more
wiley +1 more source
PSMA8‐Containing 20S Proteasome Regulates Spermiogenesis and Male Fertility
PSMA8 assembles s20S proteasome that degrades specific substrates in elongating spermatids. Degradation of s20S‐substrates activates translation of FXR1‐target mRNAs, which are essential for mitochondrial sheath formation and sperm morphogenesis.
Huiwen Cao +7 more
wiley +1 more source
Methionine Restriction Extends Lifespan in Progeroid Mice and Alters Lipid and Bile Acid Metabolism [PDF]
C.L.-O. is supported by grants from the European Union (ERC-2016-ADG, DeAge); Ministerio de Economía y Competitividad (MINECO/FEDER: SAF2014-52413-R and SAF2017-87655-R); Instituto de Salud Carlos III (RTICC); Progeria Research Foundation (PRF2016-66 ...
Bárcena Fernández, Clea +11 more
core +3 more sources
First Generation Proteolysis Targeting Chimeras (PROTACs) for the Treatment of Progeria
We report the first PROTACs designed to degrade progerin, introducing a novel therapeutic approach for progeria. The best compound, UCM‐18142, significantly reduces progerin levels and improves key disease phenotypes in patient‐derived cells and in the LmnaG609G/G609G mouse model, paving the way for new treatment strategies targeting the root cause of ...
Jon Macicior‐Michelena +5 more
wiley +1 more source
Neuron type‐specific increase in lamin B1 contributes to nuclear dysfunction in Huntington’s disease
Lamins are crucial proteins for nuclear functionality. Here, we provide new evidence showing that increased lamin B1 levels contribute to the pathophysiology of Huntington’s disease (HD), a CAG repeat‐associated neurodegenerative disorder.
Rafael Alcalá‐Vida +14 more
doaj +1 more source

