Matrilin-3 Is Dispensable for Mouse Skeletal Growth and Development
AUTOR(ES)
Ko, Yaping
FONTE
American Society for Microbiology
RESUMO
Matrilin-3 belongs to the matrilin family of extracellular matrix (ECM) proteins and is primarily expressed in cartilage. Mutations in the gene encoding human matrilin-3 (MATN-3) lead to autosomal dominant skeletal disorders, such as multiple epiphyseal dysplasia (MED), which is characterized by short stature and early-onset osteoarthritis, and bilateral hereditary microepiphyseal dysplasia, a variant form of MED characterized by pain in the hip and knee joints. To assess the function of matrilin-3 during skeletal development, we have generated Matn-3 null mice. Homozygous mutant mice appear normal, are fertile, and show no obvious skeletal malformations. Histological and ultrastructural analyses reveal endochondral bone formation indistinguishable from that of wild-type animals. Northern blot, immunohistochemical, and biochemical analyses indicated no compensatory upregulation of any other member of the matrilin family. Altogether, our findings suggest functional redundancy among matrilins and demonstrate that the phenotypes of MED disorders are not caused by the absence of matrilin-3 in cartilage ECM.
ACESSO AO ARTIGO
http://www.pubmedcentral.nih.gov/articlerender.fcgi?artid=344189Documentos Relacionados
- Mek2 Is Dispensable for Mouse Growth and Development
- Ras-Guanine Nucleotide Exchange Factor Sos2 Is Dispensable for Mouse Growth and Development
- Gadd45γ Is Dispensable for Normal Mouse Development and T-Cell Proliferation
- hnRNP C Is Required for Postimplantation Mouse Development but Is Dispensable for Cell Viability
- Fbx15 Is a Novel Target of Oct3/4 but Is Dispensable for Embryonic Stem Cell Self-Renewal and Mouse Development
