Reactome | Heparanase 2 (HPSE2) binds heparan sulfate proteoglycans

Heparanase 2 (HPSE2) binds heparan sulfate proteoglycans

Stable Identifier

R-HSA-1678694

Type

Reaction [binding]

Species

Homo sapiens

Compartment

plasma membrane, extracellular region

ReviewStatus

5/5

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Heparanase 2 (HPSE2) binds heparan sulfate proteoglycans

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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