Results 71 to 80 of about 5,061 (213)
Nuclear envelope defects cause stem cell dysfunction in premature-aging mice [PDF]
et al.Nuclear lamina alterations occur in physiological aging and in premature aging syndromes. Because aging is also associated with abnormal stem cell homeostasis, we hypothesize that nuclear envelope alterations could have an important impact on stem ...
Espada, Jesús, Pendás, Alberto M.
core +2 more sources
SUMOylation regulates mitochondrial processes, but its impact on protein import remains unclear. TOM40 is identified, a mitochondrial outer membrane channel protein, as a substrate of deSUMOylase SENP6. TOM40 SUMOylation disrupts outer membrane complex assembly, inhibits protein import, and compromises mitochondrial homeostasis.
Liubing Hu +13 more
wiley +1 more source
Laminopathies: what can humans learn from fruit flies
Lamin proteins are type V intermediate filament proteins (IFs) located inside the cell nucleus. They are evolutionarily conserved and have similar domain organization and properties to cytoplasmic IFs. Lamins provide a skeletal network for chromatin, the
Marta Pałka +6 more
doaj +1 more source
Myopathic lamin mutations impair nuclear stability in cells and tissue and disrupt nucleo-cytoskeletal coupling [PDF]
Lamins are intermediate filament proteins that assemble into a meshwork underneath the inner nuclear membrane, the nuclear lamina. Mutations in the LMNA gene, encoding lamins A and C, cause a variety of diseases collectively called laminopathies.
Dialynas, George +8 more
core
Increased release and activity of matrix metalloproteinase-9 in patients with mandibuloacral dysplasia type A, a rare premature ageing syndrome [PDF]
Mandibuloacral dysplasia type A (MADA; OMIM 248370), a rare disorder caused by mutation in the LMNA gene, is characterized by post-natal growth retardation, craniofacial and skeletal anomalies (mandibular and clavicular hypoplasia, acroosteolysis ...
Borgiani, P +9 more
core +1 more source
The Inner Nuclear Membrane Has a Unique Lipid Signature
The inner nuclear membrane (INM) has a distinct lipid profile, most notably characterized by high enrichment of phosphatidylserine (PS). In this review, Yang Niu and Tamas Balla summarize recent advances in elucidating the INM's lipid composition and related metabolic pathways, while elaborating further on their potential functional roles.
Yang Niu, Tamas Balla
wiley +1 more source
Emerging perspectives on laminopathies
Laminopathies are a group of inherited disorders caused by mutations in the lamin A/C gene, and can affect diverse organs or tissues, or can be systemic, causing premature aging. In the present review, we report on the composition and structure of the nuclear lamina and the role of lamins in nuclear mechanics and their involvement in human diseases ...
Lattanzi, Giovanna +6 more
openaire +5 more sources
Atrial TR as more favourable prognosis except in advanced patients. Abstract Background and Aims Although the classification of secondary tricuspid regurgitation (STR) by atrial or ventricular aetiology (A‐STR or V‐STR) carries prognostic importance, the confounding effects of New York Heart Association (NYHA) class have not yet been elucidated.
Corentin Bourg +9 more
wiley +1 more source
Targeted Regulation of Nuclear Lamins by Ubiquitin and Ubiquitin-Like Modifiers
Nuclear lamins (NLs) are essential components of the animal cell nucleus involved in the regulation of a plethora of molecular and cellular processes.
Michael Blank
doaj +1 more source
Recent Advances in Animal and Human Pluripotent Stem Cell Modeling of Cardiac Laminopathy [PDF]
published_or_final_versio
Jiang, Y +7 more
core +1 more source

