Perlecan

• OMIM: 142 461
• UniProt: P98160

Perlecan is a proteoglycan in vertebrates, which as a structural protein binds to the basement membrane of various Epitheltypen to other proteins. In particular, it exerts a filter function, by forming a net-like structure having pores, and is responsible for maintaining a negative electric charge. Also heparan sulfate proteoglycans take ( HSPG ) as perlecan and syndecan 1 on lipid transport across the epithelium chylomicrons in part by forming the anchorage for the enzyme lipoprotein lipase which cleaves the triglycerides of chylomicrons. Mutations at HSPG2 gene are the cause of the Schwartz - Jampel syndrome ( a disease myotonia ) and the dysplasia from Silverman - hand maker type.

The core protein has a size of 600 kDa and has 2-15 carbohydrate chains. These consist of the heparan sulfate glycosaminoglycan. By the sulfate radicals Perlecan is highly negatively charged and to prevent, inter alia, in the basal membrane of the kidney glomeruli to the passage of anionic ( negatively charged so ) serum proteins such as albumin. Thus, these proteins are not filtered and unnecessarily excreted by the kidney, but remain in the blood.