Results 31 to 40 of about 2,390 (199)

Integrated high-content quantification of intracellular ROS levels and mitochondrial morphofunction [PDF]

open access: yes, 2016
Oxidative stress arises from an imbalance between the production of reactive oxygen species (ROS) and their removal by cellular antioxidant systems.
A Boveris   +130 more
core   +2 more sources

PRIMARY (GENETICALLY DETERMINED) DILATION CARDIOMYOPATHY IN A PATIENT WITH NOVEL MUTATION OF LAMIN GENE: CLINICAL AND MORPHOLOGICAL MANAGEMENT

open access: yesКардиоваскулярная терапия и профилактика, 2017
The primary diagnosis of “dilation cardiomyopathy” is syndromal, and demands clarification of nosological origins. In the article, the specifics of such diagnostics is discussed.
O. V. Blagova   +7 more
doaj   +1 more source

Consequences of Lmna Exon 4 Mutations in Myoblast Function

open access: yesCells, 2020
Laminopathies are causally associated with mutations on the Lamin A/C gene (LMNA). To date, more than 400 mutations in LMNA have been reported in patients.
Déborah Gómez-Domínguez   +11 more
doaj   +1 more source

The telomeric protein AKTIP interacts with A- and B-type lamins and is involved in regulation of cellular senescence [PDF]

open access: yes, 2016
AKTIP is a shelterin-interacting protein required for replication of telomeric DNA. Here, we show that AKTIP biochemically interacts with A- and B-type lamins and affects lamin A, but not lamin C or B, expression.
Astrologo, Letizia   +14 more
core   +2 more sources

Mouse models of the laminopathies [PDF]

open access: yesExperimental Cell Research, 2007
The A and B type lamins are nuclear intermediate filament proteins that comprise the bulk of the nuclear lamina, a thin proteinaceous structure underlying the inner nuclear membrane. The A type lamins are encoded by the lamin A gene (LMNA). Mutations in this gene have been linked to at least nine diseases, including the progeroid diseases Hutchinson ...
Colin L, Stewart   +3 more
openaire   +2 more sources

In silico synchronization reveals regulators of nuclear ruptures in lamin A/C deficient model cells [PDF]

open access: yes, 2016
The nuclear lamina is a critical regulator of nuclear structure and function. Nuclei from laminopathy patient cells experience repetitive disruptions of the nuclear envelope, causing transient intermingling of nuclear and cytoplasmic components.
Corne, Tobias   +5 more
core   +2 more sources

Structure and stability of the lamin A tail domain and HGPS mutant [PDF]

open access: yes, 2011
Hutchinson–Gilford progeria syndrome (HGPS) is a premature aging syndrome caused by the expression and accumulation of a mutant form of lamin A, Δ50 lamin A.
Buehler, Markus J   +3 more
core   +1 more source

Thematic Review Series: Lipid Posttranslational Modifications. Prelamin A, Zmpste24, misshapen cell nuclei, and progeria—new evidence suggesting that protein farnesylation could be important for disease pathogenesis

open access: yesJournal of Lipid Research, 2005
Prelamin A undergoes multistep processing to yield lamin A, a structural protein of the nuclear lamina. Prelamin A terminates with a CAAX motif, which triggers farnesylation of a C-terminal cysteine (the C of the CAAX motif), endoproteolytic release of ...
Stephen G. Young   +2 more
doaj   +1 more source

Structural and Mechanical Aberrations of the Nuclear Lamina in Disease

open access: yesCells, 2020
The nuclear lamins are the major components of the nuclear lamina in the nuclear envelope. Lamins are involved in numerous functions, including a role in providing structural support to the cell and the mechanosensing of the cell.
Merel Stiekema   +3 more
doaj   +1 more source

In memoriam: Kuan-Teh Jeang, MD PhD (1958–2013) [PDF]

open access: yes, 2013
published_or_final_versio
Jin, D, Shi, YB, Wu, TC
core   +1 more source

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