Heparanase 2 (HPSE2) binds heparan sulfate proteoglycans

Stable Identifier
R-HSA-1678694
Type
Reaction [binding]
Species
Homo sapiens
Compartment
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Heparanase 2 (HPSE2) (McKenzie et al. 2000) is a membrane-bound protein with ~40% sequence similarity to heparanase (HPSE). While HPSE2 binds HS proteoglycans strongly, it has no endoglycosidase activity and cannot cleave heparan sulfate (HS) from HS proteoglycan. Studies of cultured cells suggest that HPSE2 may also block targeting of HPSE and HS-proteoglycan to lysosomes (Levy-Adam et al. 2010). Defects in HPSE2 are the cause of urofacial syndrome (UFS) (MIM:236730) (Daly et al. 2010, Pang et al. 2010).

Literature References
PubMed ID Title Journal Year
20560210 Mutations in HPSE2 cause urofacial syndrome

Woolf, AS, Black, GC, Stuart, H, Burgu, B, Donnai, D, Lewis, M, Newman, WG, Shalev, S, McKenzie, EA, Gener, B, Aydogdu, O, Derbent, M, Garcia-Minaur, S, Hilton, E, Daly, SB, Long, DA, Kerr, B, Urquhart, JE, Smith, R, Reardon, W, Kammerer, RA

Am J Hum Genet 2010
20560209 Loss-of-function mutations in HPSE2 cause the autosomal recessive urofacial syndrome

Ochoa, B, Voelckel, MA, Mei, L, Zhang, S, Xiong, WC, She, JX, Gu, W, Yang, P, Fisher, RB, Wang, CY, Pang, J, Zhang, Y, Zhong, J, Agundez, JA, Hawkins-Lee, B

Am J Hum Genet 2010
11027606 Cloning and expression profiling of Hpa2, a novel mammalian heparanase family member

Tyson, K, McKenzie, E, Smith, P, Stamps, A, Stubberfield, C, Turner, P, Barry, E, Patel, S, Page, M, Hircock, M, Barry, R, Terrett, J

Biochem Biophys Res Commun 2000
20576607 Heparanase 2 interacts with heparan sulfate with high affinity and inhibits heparanase activity

Cohen-Kaplan, V, Naroditsky, I, Arvatz, G, Doweck, I, Feld, S, Ilan, N, Gross, M, Shteingauz, A, Levy-Adam, F, Vlodavsky, I

J Biol Chem 2010
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