Progerin expression in humans: Implications for natural ageing
So-Mi Kang1, Soyoung Park2, Tae-Gyun Woo2
1Institute of Systems Biology, Pusan National University, Busan 46241, Republic of Korea; Department of Molecular Biology, College of Natural Science, Pusan National University, Busan 46241, Republic of Korea.
Abstract:
Progerin, a truncated lamin A isoform generated by cryptic LMNA splicing, is the pathogenic driver of Hutchinson-Gilford Progeria Syndrome (HGPS) and has been implicated as a putative marker in natural ageing. Low-level progerin arises in normal tissues, particularly skin, vasculature, and blood-derived cells, where it contributes to nuclear deformation, chromatin disorganization, DNA damage, telomere attrition, mitochondrial stress, stem cell exhaustion, and premature senescence. These cellular effects align with damage accumulation and senescence-based theories of ageing, while HGPS illustrates accelerated convergence of these mechanisms. Endogenous suppressors, including WRN helicase and telomere-protective factors, modulate progerin levels and mitigate its harmful consequences, highlighting the existence of regulatory pathways buffering nuclear lamina stress. Although its detection in normal ageing is constrained by low abundance and tissue specificity, progerin provides domain-rich information about vascular and dermal ageing, complementing systemic measures such as epigenetic clocks. Standardized ultrasensitive assays, longitudinal tissue-resolved studies, and interventional tests targeting upstream drivers or reinforcing endogenous inhibitors are key to validating progerin as part of multimodal biomarker panels. These insights may collectively position progerin as a mechanistic link between premature ageing and physiological ageing, positioning it as a potential component of biomarker strategies.
Related Concept Videos
The Effect of Aging on Tissues
Aging
Cellular Clock Theory
The cellular clock theory posits that the human lifespan is closely tied to the finite capacity of cells to divide, a phenomenon governed by telomeres, which are protective caps at the ends of...
Pharmacodynamics in Geriatric Patients: Effects of Age
Replicative Cell Senescence


