basement membrane-specific heparan sulfate proteoglycan core protein; HSPG; perlecan; PLC (HSPG2)
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Function
- integral component of basement membranes
- responsible for the fixed negative electrostatic charge of basement membrane
- role in the charge-selective ultrafiltration properties of basement membrane
- serves as an attachment substrate for cells N-glycosylated & O-glycosylated
- purified perlecan has a strong tendency to aggregate in dimers or stellate structures
- interacts with other basement membrane components such as laminin, prolargin & collagen typ-4
- interacts with COL13A1, FGFBP1 & VWA1
Structure
- contains three heparan sulfate chains
- contains 4 EGF-like domains
- contains 22 Ig-like C2-type domain (immunoglobulin-like)
- contains 11 laminin EGF-like domains
- contains 3 laminin G-like domains
- contains 3 laminin IV type A domains
- contains 4 LDL-receptor class A domains
- contains 1 SEA domain
Compartment
Expression
found in the basement membranes
Pathology
- defects in HSPG2 are the cause of
More general terms
References
- ↑ UniProt http://www.uniprot.org/uniprot/P98160.html
- ↑ Atlas of Genetics & Cytogenetics in Oncology & Haematology http://atlasgeneticsoncology.org//genes/HSPG2ID40890ch1p36.html
- ↑ Wikipedia; Note: perlecan entry http://en.wikipedia.org/wiki/perlecan
- ↑ GeneReviews https://www.genecards.org/cgi-bin/carddisp.pl?gene=HSPG2
- ↑ Ruoslahti E, Yamaguchi Y. Proteoglycans as modulators of growth factor activities. Cell. 1991 Mar 8;64(5):867-9. Review. PMID: https://www.ncbi.nlm.nih.gov/pubmed/2001586
- ↑ Noonan DM, Fulle A, Valente P, Cai S, Horigan E, Sasaki M, Yamada Y, Hassell JR. The complete sequence of perlecan, a basement membrane heparan sulfate proteoglycan, reveals extensive similarity with laminin A chain, low density lipoprotein-receptor, and the neural cell adhesion molecule. J Biol Chem. 1991 Dec 5;266(34):22939-47. PMID: https://www.ncbi.nlm.nih.gov/pubmed/1744087
Database
- UniProt: http://www.uniprot.org/uniprot/P98160.html
- Entrez gene: http://www.ncbi.nlm.nih.gov/sites/entrez?db=gene&cmd=Retrieve&dopt=Graphics&list_uids=3339
- Kegg: http://www.genome.jp/dbget-bin/www_bget?hsa:3339
- OMIM: https://mirror.omim.org/entry/142461
- OMIM: https://mirror.omim.org/entry/224410
- OMIM: https://mirror.omim.org/entry/255800