scholarly journals Age-related dystrophic changes in corneal endothelium from DNA repair-deficient mice

Aging Cell ◽  
2013 ◽  
Vol 12 (6) ◽  
pp. 1122-1131 ◽  
Author(s):  
Danny S. Roh ◽  
Yiqin Du ◽  
Michelle L. Gabriele ◽  
Andria R. Robinson ◽  
Laura J. Niedernhofer ◽  
...  
Keyword(s):  
Dna Repair ◽  
Corneal Endothelium ◽  
Age Related ◽  
Deficient Mice

scholarly journals Impact of telomerase ablation on organismal viability, aging, and tumorigenesis in mice lacking the DNA repair proteins PARP-1, Ku86, or DNA-PKcs

2004 ◽  
Vol 167 (4) ◽  
pp. 627-638 ◽  
Author(s):  
Silvia Espejel ◽  
Peter Klatt ◽  
Josiane Ménissier-de Murcia ◽  
Juan Martín-Caballero ◽  
Juana M. Flores ◽  
...  
Keyword(s):  
Dna Repair ◽  
Aging Process ◽  
Telomere Shortening ◽  
Age Related ◽  
Dna Repair Proteins ◽  
Telomere Function ◽  
Telomere Capping ◽  
The Impact ◽  
Parp 1 ◽  
Deficient Mice

The DNA repair proteins poly(ADP-ribose) polymerase-1 (PARP-1), Ku86, and catalytic subunit of DNA-PK (DNA-PKcs) have been involved in telomere metabolism. To genetically dissect the impact of these activities on telomere function, as well as organismal cancer and aging, we have generated mice doubly deficient for both telomerase and any of the mentioned DNA repair proteins, PARP-1, Ku86, or DNA-PKcs. First, we show that abrogation of PARP-1 in the absence of telomerase does not affect the rate of telomere shortening, telomere capping, or organismal viability compared with single telomerase-deficient controls. Thus, PARP-1 does not have a major role in telomere metabolism, not even in the context of telomerase deficiency. In contrast, mice doubly deficient for telomerase and either Ku86 or DNA-PKcs manifest accelerated loss of organismal viability compared with single telomerase-deficient mice. Interestingly, this loss of organismal viability correlates with proliferative defects and age-related pathologies, but not with increased incidence of cancer. These results support the notion that absence of telomerase and short telomeres in combination with DNA repair deficiencies accelerate the aging process without impacting on tumorigenesis.

scholarly journals Crosstalk between Different DNA Repair Pathways Contributes to Neurodegenerative Diseases

Biology ◽  
2021 ◽  
Vol 10 (2) ◽  
pp. 163
Author(s):  
Swapnil Gupta ◽  
Panpan You ◽  
Tanima SenGupta ◽  
Hilde Nilsen ◽  
Kulbhushan Sharma
Keyword(s):  
Dna Repair ◽  
Neurodegenerative Diseases ◽  
3D Models ◽  
Model Systems ◽  
Spinocerebellar Ataxias ◽  
C Elegans ◽  
Cellular Processes ◽  
Age Related ◽  
Damage Response ◽  
Lateral Sclerosis

Genomic integrity is maintained by DNA repair and the DNA damage response (DDR). Defects in certain DNA repair genes give rise to many rare progressive neurodegenerative diseases (NDDs), such as ocular motor ataxia, Huntington disease (HD), and spinocerebellar ataxias (SCA). Dysregulation or dysfunction of DDR is also proposed to contribute to more common NDDs, such as Parkinson’s disease (PD), Alzheimer’s disease (AD), and Amyotrophic Lateral Sclerosis (ALS). Here, we present mechanisms that link DDR with neurodegeneration in rare NDDs caused by defects in the DDR and discuss the relevance for more common age-related neurodegenerative diseases. Moreover, we highlight recent insight into the crosstalk between the DDR and other cellular processes known to be disturbed during NDDs. We compare the strengths and limitations of established model systems to model human NDDs, ranging from C. elegans and mouse models towards advanced stem cell-based 3D models.

scholarly journals Growth Retardation, DNA Repair Defects, and Lack of Spermatogenesis in BRCA1-Deficient Mice

2006 ◽  
Vol 26 (24) ◽  
pp. 9571-9571 ◽  
Author(s):  
Victoria L. Cressman ◽  
Dana C. Backlund ◽  
Anna V. Avrutskaya ◽  
Steven A. Leadon ◽  
Virginia Godfrey ◽  
...  
Keyword(s):  
Dna Repair ◽  
Growth Retardation ◽  
Deficient Mice

scholarly journals A low glycemic diet protects disease-prone Nrf2-deficient mice against age-related macular degeneration

2020 ◽  
Vol 150 ◽  
pp. 75-86 ◽  
Author(s):  
Sheldon Rowan ◽  
Shuhong Jiang ◽  
Min-Lee Chang ◽  
Jonathan Volkin ◽  
Christa Cassalman ◽  
...  
Keyword(s):  
Macular Degeneration ◽  
Age Related Macular Degeneration ◽  
Age Related ◽  
Deficient Mice
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