Perlecan/perlecan
Perlecan is a large HSPG, present in virtually all BMs which consists of a core protein of molecular weight 470 kDa to which three long chains (each approximately 70-100 kDa) of glycosaminoglycans (often heparan sulfate, HS but can be chondroitin sulfate, CS) are attached. It interacts with basement membrane components such as laminin-1 and collagen IV but had no effect on the lining and deposition of this major basement membrane components. It also interacts with cell adhesion molecules such as β1 integrin but does not affect its expression. Inactivation of the perlecan gene in mice results in embryonic lethality and prenatal death. Similarly, the functional null mutation in the perlecan gene in humans, termed dyssegmental dysplasia, Silverman-Handmaker type (DDSH), is characterized by either stillbirth or death within a few days after birth. Studies in monolayer cultures revealed that perlecan binds and modulates the activity of a variety of growth factors, including FGF7 and GM-CSF that are implicated in human skin formation. Perlecan regulates both the survival and terminal differentiation steps of keratinocytes. Perlecan deposition in the basement membrane zone correlated with formation of multilayered epidermis.
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