LINE-1 RNA causes heterochromatin erosion and is a target for amelioration of senescent phenotypes in progeroid syndromes
Constitutive heterochromatin is responsible for genome repression of DNA enriched in
repetitive sequences, telomeres, and centromeres. During physiological and pathological …
repetitive sequences, telomeres, and centromeres. During physiological and pathological …
Splicing-directed therapy in a new mouse model of human accelerated aging
FG Osorio, CL Navarro, J Cadiñanos… - Science translational …, 2011 - science.org
Hutchinson-Gilford progeria syndrome (HGPS) is caused by a point mutation in the LMNA
gene that activates a cryptic donor splice site and yields a truncated form of prelamin A …
gene that activates a cryptic donor splice site and yields a truncated form of prelamin A …
Depleting the methyltransferase Suv39h1 improves DNA repair and extends lifespan in a progeria mouse model
Abstract A de novo G608G mutation in LMNA gene leads to Hutchinson–Gilford progeria
syndrome. Mice lacking the prelamin A-processing metalloprotease, Zmpste24, recapitulate …
syndrome. Mice lacking the prelamin A-processing metalloprotease, Zmpste24, recapitulate …
Inhibition of DNA damage response at telomeres improves the detrimental phenotypes of Hutchinson–Gilford Progeria Syndrome
Hutchinson–Gilford progeria syndrome (HGPS) is a genetic disorder characterized by
premature aging features. Cells from HGPS patients express progerin, a truncated form of …
premature aging features. Cells from HGPS patients express progerin, a truncated form of …
Histone H4 lysine 16 hypoacetylation is associated with defective DNA repair and premature senescence in Zmpste24-deficient mice
V Krishnan, MZY Chow, Z Wang… - Proceedings of the …, 2011 - National Acad Sciences
Specific point mutations in lamin A gene have been shown to accelerate aging in humans
and mice. Particularly, a de novo mutation at G608G position impairs lamin A processing to …
and mice. Particularly, a de novo mutation at G608G position impairs lamin A processing to …
Molecular pathology of rare progeroid diseases
Progeroid syndromes (PSs) are characterized by the premature onset of age-related
pathologies. The genetic mutations underlying PSs are functionally linked to genome …
pathologies. The genetic mutations underlying PSs are functionally linked to genome …
Deregulated expression of mammalian lncRNA through loss of SPT6 induces R-loop formation, replication stress, and cellular senescence
Extensive tracts of the mammalian genome that lack protein-coding function are still
transcribed into long noncoding RNA. While these lncRNAs are generally short lived, length …
transcribed into long noncoding RNA. While these lncRNAs are generally short lived, length …
Hallmarks of progeroid syndromes: lessons from mice and reprogrammed cells
D Carrero, C Soria-Valles… - Disease models & …, 2016 - journals.biologists.com
Ageing is a process that inevitably affects most living organisms and involves the
accumulation of macromolecular damage, genomic instability and loss of heterochromatin …
accumulation of macromolecular damage, genomic instability and loss of heterochromatin …
Human progeroid syndromes, aging and cancer: new genetic and epigenetic insights into old questions
CL Ramirez, J Cadinanos, I Varela, JMP Freije… - Cellular and molecular …, 2007 - Springer
Disorders in which individuals exhibit certain features of aging early in life are referred to as
segmental progeroid syndromes. With the progress that has been made in understanding …
segmental progeroid syndromes. With the progress that has been made in understanding …
Targeting of NAT10 enhances healthspan in a mouse model of human accelerated aging syndrome
Abstract Hutchinson-Gilford Progeria Syndrome (HGPS) is a rare, but devastating genetic
disease characterized by segmental premature aging, with cardiovascular disease being the …
disease characterized by segmental premature aging, with cardiovascular disease being the …