Normal Bone Matrix Mineralization but Altered Growth Plate Morphology in the Lmna^G609G/G609G Mouse Model of Progeria.

ABSTRACT: Hutchison-Gilford progeria syndrome (HGPS) is a rare genetic disease caused by a mutation in LMNA, the gene encoding A-type lamins, leading to premature aging with severely reduced life span. HGPS is characterized by growth deficiency, subcutaneous fat and muscle issues, wrinkled skin, alopecia, and atherosclerosis. Patients also develop a bone phenotype with reduced bone mineral density, osteolysis and striking demineralization of long bones. To further clarify the tissue modifications in HGPS, we characterized bone mineralization in the Lmna^G609G/G609G progeria mouse model. Femurs from 8-week-old mice and humeri from 15-week-old mice were analyzed using quantitative backscattered electron imaging to assess bone mineralization density distribution, osteocyte lacunae sections an

SUBMITTER: Blouin S 

PROVIDER: S-EPMC12339082 | biostudies-literature | 2024 Nov

REPOSITORIES: biostudies-literature