特异性This antibody recognizes pro and active forms of MMP13. We have data to indicate that this antibody may not cross react with Human. However, this has not been conclusively tested and expression levels may vary in certain cell lines/tissues.
The application notes include recommended starting dilutions; optimal dilutions/concentrations should be determined by the end user.
Use a concentration of 1 µg/ml. Predicted molecular weight: 54 kDa. Incubate for 2 hours at room temperature.
Use at 2 µg/mg of lysate.
功能Degrades collagen type I. Does not act on gelatin or casein. Could have a role in tumoral process.
组织特异性Seems to be specific to breast carcinomas.
疾病相关Defects in MMP13 are the cause of spondyloepimetaphyseal dysplasia Missouri type (SEMD-MO) [MIM:602111]. A bone disease characterized by moderate to severe metaphyseal changes, mild epiphyseal involvement, rhizomelic shortening of the lower limbs with bowing of the femora and/or tibiae, coxa vara, genu varum and pear-shaped vertebrae in childhood. Epimetaphyseal changes improve with age. Defects in MMP13 are the cause of metaphyseal anadysplasia type 1 (MANDP1) [MIM:602111]. Metaphyseal anadysplasia consists of an abnormal bone development characterized by severe skeletal changes that, in contrast with the progressive course of most other skeletal dysplasias, resolve spontaneously with age. Clinical characteristics are evident from the first months of life and include slight shortness of stature and a mild varus deformity of the legs. Patients attain a normal stature in adolescence and show improvement or complete resolution of varus deformity of the legs and rhizomelic micromelia.
序列相似性Belongs to the peptidase M10A family. Contains 4 hemopexin-like domains.
结构域The conserved cysteine present in the cysteine-switch motif binds the catalytic zinc ion, thus inhibiting the enzyme. The dissociation of the cysteine from the zinc ion upon the activation-peptide release activates the enzyme.
细胞定位Secreted > extracellular space > extracellular matrix.