LaminA Mouse Model
| Starin Name | C57BL/6J‑Lmna<sup>tm1(G609G)</sup> |
|---|---|
| Strain Background | C57BL/6J |
Applications
- Aging & Senescence Mechanism Research: Investigating nuclear lamina dysfunction, DNA damage accumulation, telomere attrition, and senescence‑associated secretory phenotype (SASP) in accelerated aging.
- Age‑Related Cardiovascular & Metabolic Disease: Modeling vascular calcification/stiffness, lipodystrophy, dyslipidemia, and cardiomyopathy associated with laminopathies.
- Geroprotective & Anti‑Aging Drug Screening: Preclinical evaluation of farnesyltransferase inhibitors (FTIs), splicing modulators (e.g., morpholino antisense oligos), senolytics, and antioxidant therapies targeting progerin‑induced pathology.
- Musculoskeletal & Dermal Aging: Studying osteoporosis, sarcopenia, dermal thinning, and alopecia mechanisms in the context of nuclear envelope defects.
- Biomarker Validation: Quantifying senescence markers (e.g., SA‑β‑Gal in hippocampus/brain, p16INK4a, γ‑H2AX foci) in a physiologically relevant premature‑aging background.
Key Features
- Clinically Relevant LMNA Mutation: Harbors the canonical LmnaG609G (human LMNAG608G/progerin‑inducing) point mutation that activates a cryptic splice site → expression of truncated prelamin A (progerin), faithfully mimicking human HGPS molecular pathogenesis.
- Robust Progeroid Phenotype: Homozygotes display overt premature‑aging signs—alopecia, dwarfism, osteolysis, subcutaneous fat loss, vascular sclerosis, organ fibrosis, chronic low‑grade inflammation—with accelerated mortality (median ~100 d).
- Short Lifecycle & Clear Readouts: Rapid disease onset post‑puberty enables time‑/cost‑efficient drug efficacy studies; phenotype penetrance is high and uniform across littermates.
- Defined Genetic Background: Established on C57BL/6J inbred background ensuring genetic homogeneity and compatibility with standard aged‑mouse cohorts and transgenic crosses.
Strain Description
Lamin A Premature Aging (LMNAG609G) Knock-in Mouse Model — C57BL/6J‑Lmnatm1(G609G)/MCL
This model carries a homozygous or heterozygous LMNA G608G(mouse G609G, equivalent to human LMNAc.1824C>T, p.Gly606Gly which activates a cryptic splice site causing Δ50/progerin expression) point mutation on the C57BL/6J background. It recapitulates the classic Hutchinson‑Gilford Progeria Syndrome (HGPS) phenotype—including growth retardation, alopecia, osteoporosis, lipodystrophy, vascular stiffening, and markedly shortened lifespan (~100 days in homozygotes)—providing a well‑established in vivoplatform for aging research and geroprotective drug screening.
FAQ
Game-changing benefits？
While competitors highlight germline efficiency gains, shorter timelines and enhanced 3Rs animal welfare benefits for their technologies, these are merely incremental improvements over traditional approaches. In sharp contrast, our proprietary technology delivers fully pure, homogeneous lineages—every single cell of the mice is derived exclusively from totipotent ES cells, with guaranteed 100% germline transmission efficiency. To experience these unparalleled benefits firsthand, enquire about your custom mouse model project with us or order embryos for in-house validation at your facility.
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