Perlecan
| Perlecan | ||
|---|---|---|
| Properties of human protein | ||
| Mass / length primary structure | 4370 amino acids | |
| Secondary to quaternary structure | Dimer; star-shaped homooligomers | |
| Identifier | ||
| Gene name | HSPG2 | |
| External IDs | ||
| Occurrence | ||
| Homology family | Perlecan | |
| Parent taxon | Vertebrates | |
Perlecan is a proteoglycan in vertebrates that binds to other proteins as a structural protein in the basement membrane of various epithelial types . In particular, it has a filter function by forming a network-like structure with pores and is responsible for maintaining a negative electrical charge. In addition, heparan sulfate proteoglycans (HSPG) such as Syndecan 1 and Perlecan take part in lipid transport through chylomicrons into the epithelium by forming the anchor for the enzyme lipoprotein lipase , which cleaves the triglycerides from the chylomicrons. Mutations at HSPG2 - gene are the cause of the Schwartz-Jampel syndrome (a myotonia disease infection) and the Dyssegmentale dysplasia from the Silverman-hand Maker type.
The core protein is 600 kDa in size and has 2–15 carbohydrate chains . These consist of the glycosaminoglycan heparan sulfate . Due to the sulfate residues, Perlecan is strongly negatively charged and prevents a. in the basement membrane of the renal glomeruli the passage of anionic (i.e. negatively charged) serum proteins such as albumin . As a result, these proteins are not filtered and excreted unnecessarily by the kidney , but remain in the blood.
literature
- Joachim Rassow , Karin Hauser, Roland Netzker, Rainer Deutzmann: Dual Biochemistry Series . 2nd Edition. Thieme, Stuttgart 2008, ISBN 978-3-13-125352-1 , p. 405-406 .
Individual evidence
- ↑ UniProt P98160
- ↑ D'Eustachio / reactome: chylomicron ⇒ TG-depleted chylomicron + 50 long-chain fatty acids + 50 diacylglycerols ( page no longer available , search in web archives ) Info: The link was automatically marked as defective. Please check the link according to the instructions and then remove this notice.
Web links
- OrphaNet: Schwartz-Jampel syndrome