Molecular bases of progeroid syndromes
Abstract
Progeroid syndromes (PSs) constitute a group of disorders characterized by clinical features mimicking physiological aging at an early age. In some of these syndromes, biological hallmarks of aging are also present, whereas in others, a link with physiological aging, if any, remains to be elucidated. These syndromes are clinically and genetically heterogeneous and most of them, including Werner syndrome and Hutchinson-Gilford progeria, are known as 'segmental aging syndromes', as they do not feature all aspects usually associated to physiological aging. However, all the characterized PSs enter in the field of rare monogenic disorders and several causative genes have been identified. These can be separated in subcategories corresponding to (i) genes encoding DNA repair factors, in particular, DNA helicases, and (ii) genes affecting the structure or post-translational maturation of lamin A, a major nuclear component. In addition, several animal models featuring premature aging have abnormal mitochondrial function or signal transduction between membrane receptors, nuclear regulatory proteins and mitochondria: no human pathological counterpart of these alterations has been found to date. In recent years, identification of mutations and their functional characterization have helped to unravel the cellular processes associated to segmental PSs. Recently, several studies allowed to establish a functional link between DNA repair and A-type lamins-associated syndromes, evidencing a relation between these syndromes, physiological aging and cancer. Here, we review recent data on molecular and cellular bases of PSs and discuss the mechanisms involved, with a special emphasis on lamin A-associated progeria and related disorders, for which therapeutic approaches have started to be developed.
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All Zmpste24 enzymatic activity is lost in RD; FC-prelamin A is toxic, especially with the absence of normal lamin A, causing perinatal death.
“All Zmpste24 enzymatic activity is lost in individuals with RD (Zmpste24-/-); the farnesylated and carboxy-methylated prelamin A (FC-prelamin A) is toxic, especially with the absence of normal lamin A, causing perinatal death [29,30].”
From Genomic instability and DNA damage responses in progeria arising from defective maturation of prelamin A (2009) · cites this paper as [29]
Prenyl groups are derived from the cholesterol biosynthetic pathway; statins and amino-bisphosphonates are common drugs for treatment of hypercholesterolemia.
“The prenyl groups are derived from the cholesterol biosynthetic pathway; statins and amino-bisphosphonates are common drugs for treatment of hypercholesterolemia [29].”
From Genomic instability and DNA damage responses in progeria arising from defective maturation of prelamin A (2009) · cites this paper as [29]
Statins and amino-bisphosphonates appear more effective than FTIs in reducing phenotypic markers of laminopathy in model mice and cellular (HGPS, RD) assays.
“These drugs also appear more effective than FTIs in reducing phenotypic markers of laminopathy in model mice and cellular (HGPS, RD) assays [29,67,68].”
From Genomic instability and DNA damage responses in progeria arising from defective maturation of prelamin A (2009) · cites this paper as [29]
A major phenotype-inducing element of progerin and FC-prelamin A is the farnesyl moiety.
“since it is believed that a major phenotype-inducing element of progerin and FC-prelamin A is the farnesyl moiety [14,29].”
From Genomic instability and DNA damage responses in progeria arising from defective maturation of prelamin A (2009) · cites this paper as [29]
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- Authors
- Navarro, C. L., Cau, P., & Lévy, N.
- Affiliations
- Inserm (FR)
- Corresponding author
- Lévy, Nicolas
- Venue
- Human Molecular Genetics
- Publisher
- Oxford University Press (OUP)
- Published
- 2006-10-15
- Volume
- 15
- Issue
- suppl_2
- Pages
- R151-R161
- ISSN
- 0964-6906 (ISSN-L)
- 1460-2083
- OpenAlex
- W2150619381
- Cited by
- 189
- References
- 158
- Citations by year
- 2026: 4 · 2025: 4 · 2024: 2 · 2023: 5 · 2022: 3 · 2021: 11 · 2020: 8 · 2019: 6 · 2018: 9 · 2017: 9 · 2016: 14 · 2015: 8 · 2014: 13 · 2013: 6 · 2012: 12
- FWCI
- 1.19
- Type
- journal article
- Language
- en
- Open access
- closed
- Locations
- Human Molecular Genetics (publishedVersion)
- PubMed (publishedVersion)
- Funders
- Institut National de la Santé et de la Recherche Médicale
- Topics
- Life Sciences › Biochemistry, Genetics and Molecular Biology › Molecular Biology › Nuclear Structure and Function
- Life Sciences › Biochemistry, Genetics and Molecular Biology › Aging › Genetics, Aging, and Longevity in Model Organisms
- Life Sciences › Biochemistry, Genetics and Molecular Biology › Molecular Biology › DNA Repair Mechanisms
- Keywords
- progeroid syndromes, premature aging, Werner syndrome, Hutchinson-Gilford progeria syndrome, lamin A, DNA helicase, DNA repair, mitochondrial dysfunction, cancer, treatment options
- MeSH terms
- Liver X Receptors, Adult, Child, Cockayne Syndrome, DNA Repair, Humans, Mitochondria, Models, Biological, Models, Genetic, Progeria, Syndrome, Werner Syndrome, Receptors, Cytoplasmic and Nuclear, Lamin Type A, RecQ Helicases, Orphan Nuclear Receptors
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