The matrilins are a recently discovered family of non-collagenous extracellular matrix proteins. During embryogenesis, all matrilins are expressed in skeletal tissues. Additionally, matrilin-2 and -4 are expressed in the dermis and in connective tissues of internal organs, e.g. of the lung and kidney. After birth, the expression of matrilin-1 and -3 remains specific for cartilage and bone whereas matrilin-2 and -4 display a broader tissue distribution and could be detected in epithelial, muscle, and nervous tissue as well as in loose and dense connective tissue. In epiphyseal cartilage of growing long bones, matrilin-1 and -3 are present in all cartilage regions, in contrast to matrilin-2, which is expressed in the proliferative and the upper hypertrophic zones. Similarly matrilin-4 was detected all over the epiphyseal cartilage, with the weakest expression in the hypertrophic zone. Although it was shown that matrilin-1 and -3 can form hetero-oligomers and are often co-localized in tissue, clear differences in their spatial distribution could be demonstrated by double-immunolabelling. During joint development matrilin-2 and matrilin-4 are present at the developing joint surface, while in articular cartilage of 6-week-old mice all matrilins are only weakly expressed.