The world's first wiki where authorship really matters (Nature Genetics, 2008). Due credit and reputation for authors. Imagine a global collaborative knowledge base for original thoughts. Search thousands of articles and collaborate with scientists around the globe.

wikigene or wiki gene protein drug chemical gene disease author authorship tracking collaborative publishing evolutionary knowledge reputation system wiki2.0 global collaboration genes proteins drugs chemicals diseases compound
Hoffmann, R. A wiki for the life sciences where authorship matters. Nature Genetics (2008)



Gene Review

Hspg2  -  perlecan (heparan sulfate proteoglycan 2)

Mus musculus

Synonyms: AI852380, Basement membrane-specific heparan sulfate proteoglycan core protein, HSPG, Pcn, Plc, ...
Welcome! If you are familiar with the subject of this article, you can contribute to this open access knowledge base by deleting incorrect information, restructuring or completely rewriting any text. Read more.

Disease relevance of Hspg2


Psychiatry related information on Hspg2


High impact information on Hspg2

  • Syndecan-1 is a cell-surface, heparan-sulphate proteoglycan (HSPG) predominantly expressed by epithelial cells [7].
  • Through the generation of AAV2-AAV8 hybrids and the creation of site-directed mutations, we mapped the domain that directs the activation of T cells to the RXXR motif on VP3, which was previously shown to confer binding of the virion to heparan sulfate proteoglycan (HSPG) [8].
  • Heparan sulphate, or the heparan sulphate proteoglycan perlecan, has been identified in all amyloids and implicated in the earliest stages of inflammation-associated (AA) amyloid induction [9].
  • Here we show that perlecan, a multifunctional heparan sulfate proteoglycan concentrated at the NMJ, is the unique acceptor molecule for collagen-tailed AChE at sites of nerve-muscle contact and is the principal mechanism for localizing AChE to the synaptic basal lamina [10].
  • Absence of acetylcholinesterase at the neuromuscular junctions of perlecan-null mice [10].

Chemical compound and disease context of Hspg2


Biological context of Hspg2


Anatomical context of Hspg2


Associations of Hspg2 with chemical compounds

  • The HS at the NH(2) terminus of perlecan was removed while the core protein remained intact [16].
  • The expression of perlecan mRNA decreased substantially during skeletal muscle differentiation, in contrast to the increase in transcripts for specific skeletal muscle proteins such as myogenin and creatine kinase [21].
  • Blot overlay assays have shown that perlecan binds alpha-DG in a calcium and heparin-sensitive manner [22].
  • The loss of the basement membrane proteoglycan perlecan and the podocyte protein nephrin in the diabetic state was not prevented in the PKC-beta(-/-) mice as previously demonstrated in the nonalbuminuric diabetic PKC-alpha(-/-) mice [23].
  • By 7 weeks, only animals infused with A beta plus HSPG demonstrated compaction of the Congo red material from amorphous, wispy deposits (at 1 week) to stellate deposits resembling a Maltese cross [24].

Physical interactions of Hspg2

  • This competition may result in the presentation of perlecan-bound growth factors such as HB-GAM to effect synaptic induction [22].
  • Crystal structure and mutational analysis of a perlecan-binding fragment of nidogen-1 [19].
  • Perlecan is a specific proteoglycan that binds to amyloid precursor protein and beta-amyloid peptide, is present within amyloid deposits, and has been implicated in plaque formation [2].
  • These results suggest that dystroglycan is essential for the assembly of a synaptic basement membrane, most notably by localizing AChE through its binding to perlecan [25].
  • In the present study we investigated the effect of the isoform and the amount of apoE per particle, as well as the role of LPL on the binding of beta-VLDL to HSPG [26].

Co-localisations of Hspg2


Regulatory relationships of Hspg2

  • The binding of beta-VLDL to heparan sulfate proteoglycans (HSPG) has been reported to be stimulated by both apoE and lipoprotein lipase (LPL) [26].

Other interactions of Hspg2

  • We have solved the crystal structure at 2.0 A resolution of the mouse nidogen-1 G2-perlecan IG3 complex [20].
  • Both stimulatory and inhibitory effects of perlecan on FGF-2 signaling have been reported [16].
  • However, at 24 weeks and in the LDL receptor heterozygous background, the presence of a perlecan knockout allele did not significantly alter lesion size [27].
  • Most notably, HIP/RPL29 accumulated in decidua, where it displayed a pattern complementary to that of pericellular deposition of the HS proteoglycan, perlecan [28].
  • Furthermore, perlecan is present in muscle lysate immunoprecipitated with an anti-DG antibody [22].

Analytical, diagnostic and therapeutic context of Hspg2


  1. Antisense targeting of perlecan blocks tumor growth and angiogenesis in vivo. Sharma, B., Handler, M., Eichstetter, I., Whitelock, J.M., Nugent, M.A., Iozzo, R.V. J. Clin. Invest. (1998) [Pubmed]
  2. Regulation of the heparan sulfate proteoglycan, perlecan, by injury and interleukin-1alpha. García de Yébenes, E., Ho, A., Damani, T., Fillit, H., Blum, M. J. Neurochem. (1999) [Pubmed]
  3. Expression of perlecan proteoglycan in the infarct zone of mouse myocardial infarction. Nakahama, M., Murakami, T., Kusachi, S., Naito, I., Takeda, K., Ohnishi, H., Komatsubara, I., Oka, T., Ninomiya, Y., Tsuji, T. J. Mol. Cell. Cardiol. (2000) [Pubmed]
  4. Increased intimal hyperplasia and smooth muscle cell proliferation in transgenic mice with heparan sulfate-deficient perlecan. Tran, P.K., Tran-Lundmark, K., Soininen, R., Tryggvason, K., Thyberg, J., Hedin, U. Circ. Res. (2004) [Pubmed]
  5. Exploitation of syndecan-1 shedding by Pseudomonas aeruginosa enhances virulence. Park, P.W., Pier, G.B., Hinkes, M.T., Bernfield, M. Nature (2001) [Pubmed]
  6. Distribution of perlecan in mouse hippocampus following intracerebroventricular kainate injections. Shee, W.L., Ong, W.Y., Lim, T.M. Brain Res. (1998) [Pubmed]
  7. Syndecan-1 is required for Wnt-1-induced mammary tumorigenesis in mice. Alexander, C.M., Reichsman, F., Hinkes, M.T., Lincecum, J., Becker, K.A., Cumberledge, S., Bernfield, M. Nat. Genet. (2000) [Pubmed]
  8. Heparin binding directs activation of T cells against adeno-associated virus serotype 2 capsid. Vandenberghe, L.H., Wang, L., Somanathan, S., Zhi, Y., Figueredo, J., Calcedo, R., Sanmiguel, J., Desai, R.A., Chen, C.S., Johnston, J., Grant, R.L., Gao, G., Wilson, J.M. Nat. Med. (2006) [Pubmed]
  9. Arresting amyloidosis in vivo using small-molecule anionic sulphonates or sulphates: implications for Alzheimer's disease. Kisilevsky, R., Lemieux, L.J., Fraser, P.E., Kong, X., Hultin, P.G., Szarek, W.A. Nat. Med. (1995) [Pubmed]
  10. Absence of acetylcholinesterase at the neuromuscular junctions of perlecan-null mice. Arikawa-Hirasawa, E., Rossi, S.G., Rotundo, R.L., Yamada, Y. Nat. Neurosci. (2002) [Pubmed]
  11. Delayed catabolism of apoB-48 lipoproteins due to decreased heparan sulfate proteoglycan production in diabetic mice. Ebara, T., Conde, K., Kako, Y., Liu, Y., Xu, Y., Ramakrishnan, R., Goldberg, I.J., Shachter, N.S. J. Clin. Invest. (2000) [Pubmed]
  12. A cellular deficiency of gangliosides causes hypersensitivity to Clostridium perfringens phospholipase C. Flores-Díaz, M., Alape-Girón, A., Clark, G., Catimel, B., Hirabayashi, Y., Nice, E., Gutiérrez, J.M., Titball, R., Thelestam, M. J. Biol. Chem. (2005) [Pubmed]
  13. WT1 expression induces features of renal epithelial differentiation in mesenchymal fibroblasts. Hosono, S., Luo, X., Hyink, D.P., Schnapp, L.M., Wilson, P.D., Burrow, C.R., Reddy, J.C., Atweh, G.F., Licht, J.D. Oncogene (1999) [Pubmed]
  14. Effect of a specific endothelin A receptor antagonist on murine lupus nephritis. Nakamura, T., Ebihara, I., Tomino, Y., Koide, H. Kidney Int. (1995) [Pubmed]
  15. Expression of the heparan sulfate proteoglycan, perlecan, during mouse embryogenesis and perlecan chondrogenic activity in vitro. French, M.M., Smith, S.E., Akanbi, K., Sanford, T., Hecht, J., Farach-Carson, M.C., Carson, D.D. J. Cell Biol. (1999) [Pubmed]
  16. Impaired angiogenesis, delayed wound healing and retarded tumor growth in perlecan heparan sulfate-deficient mice. Zhou, Z., Wang, J., Cao, R., Morita, H., Soininen, R., Chan, K.M., Liu, B., Cao, Y., Tryggvason, K. Cancer Res. (2004) [Pubmed]
  17. Release of cell surface-associated basic fibroblast growth factor by glycosylphosphatidylinositol-specific phospholipase C. Bashkin, P., Neufeld, G., Gitay-Goren, H., Vlodavsky, I. J. Cell. Physiol. (1992) [Pubmed]
  18. Internalization of basic fibroblast growth factor at the mouse blood-brain barrier involves perlecan, a heparan sulfate proteoglycan. Deguchi, Y., Okutsu, H., Okura, T., Yamada, S., Kimura, R., Yuge, T., Furukawa, A., Morimoto, K., Tachikawa, M., Ohtsuki, S., Hosoya, K., Terasaki, T. J. Neurochem. (2002) [Pubmed]
  19. Crystal structure and mutational analysis of a perlecan-binding fragment of nidogen-1. Hopf, M., Göhring, W., Ries, A., Timpl, R., Hohenester, E. Nat. Struct. Biol. (2001) [Pubmed]
  20. Structural basis for the high-affinity interaction of nidogen-1 with immunoglobulin-like domain 3 of perlecan. Kvansakul, M., Hopf, M., Ries, A., Timpl, R., Hohenester, E. EMBO J. (2001) [Pubmed]
  21. Expression of perlecan, a proteoglycan that binds myogenic inhibitory basic fibroblast growth factor, is down regulated during skeletal muscle differentiation. Larraín, J., Alvarez, J., Hassell, J.R., Brandan, E. Exp. Cell Res. (1997) [Pubmed]
  22. The relationship between perlecan and dystroglycan and its implication in the formation of the neuromuscular junction. Peng, H.B., Ali, A.A., Daggett, D.F., Rauvala, H., Hassell, J.R., Smalheiser, N.R. Cell Adhes. Commun. (1998) [Pubmed]
  23. Deletion of Protein Kinase C-{beta} Isoform In Vivo Reduces Renal Hypertrophy but Not Albuminuria in the Streptozotocin-Induced Diabetic Mouse Model. Meier, M., Park, J.K., Overheu, D., Kirsch, T., Lindschau, C., Gueler, F., Leitges, M., Menne, J., Haller, H. Diabetes (2007) [Pubmed]
  24. An important role of heparan sulfate proteoglycan (Perlecan) in a model system for the deposition and persistence of fibrillar A beta-amyloid in rat brain. Snow, A.D., Sekiguchi, R., Nochlin, D., Fraser, P., Kimata, K., Mizutani, A., Arai, M., Schreier, W.A., Morgan, D.G. Neuron (1994) [Pubmed]
  25. The dystroglycan complex is necessary for stabilization of acetylcholine receptor clusters at neuromuscular junctions and formation of the synaptic basement membrane. Jacobson, C., Côté, P.D., Rossi, S.G., Rotundo, R.L., Carbonetto, S. J. Cell Biol. (2001) [Pubmed]
  26. Binding of beta-VLDL to heparan sulfate proteoglycans requires lipoprotein lipase, whereas ApoE only modulates binding affinity. de Beer, F., Hendriks, W.L., van Vark, L.C., Kamerling, S.W., van Dijk, K.W., Hofker, M.H., Smelt, A.H., Havekes, L.M. Arterioscler. Thromb. Vasc. Biol. (1999) [Pubmed]
  27. Atherosclerosis in perlecan heterozygous mice. Vikramadithyan, R.K., Kako, Y., Chen, G., Hu, Y., Arikawa-Hirasawa, E., Yamada, Y., Goldberg, I.J. J. Lipid Res. (2004) [Pubmed]
  28. Expression of heparin/heparan sulfate interacting protein/ribosomal protein l29 during the estrous cycle and early pregnancy in the mouse. Julian, J., Das, S.K., Dey, S.K., Baraniak, D., Ta, V.T., Carson, D.D. Biol. Reprod. (2001) [Pubmed]
WikiGenes - Universities