L-PRX dimer binds DRP2

Stable Identifier
Reaction [binding]
Homo sapiens
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L-Periaxin (L-PRX) is a membrane protein that is expressed in Schwann cells of the peripheral nervous system where it acts as a key component of the dystrophin glycoprotein complex (DGC). PRX is required for the proper localization of a dystrophin-related protein DRP2 to sites of apposition between the Schwann cell plasma membrane and the abaxonal surface of the myelin sheath (Sherman et al, 2001; Sherman et al, 2012; Court et al, 2004; Court et al, 2009; reviewed in Masaki and Matsumura, 2010). The spectrin repeats of DRP2 and the basic subdomains of L-PRZ mediate direct interaction between the two proteins. S-PRZ, an alternate isofom that lacks the basic subdomains of L-PRZ, does not interact with DRP2 (Sherman et al, 2001). The complex of L-PRX and DRP2 also interact with other dystrophin-related proteins such as utrophin, as well as dystroglycan and components of the basal lumina, such as laminin-211 (Yamada et al, 1994; Yamada et al, 1996; Sherman et al, 2001).
PRZ and DRP2 have partially overlapping but distinct roles in myelin sheath formation. Both are required for the formation of appositions between the plasma membrane and the myelin sheath and for the formation of Cajal bodies. PRX also contributes to normal Schwann cell elongation and regulation of internode space along the axon, which is required for nerve conduction, while DRP2 is dispensable for these activities (Court et al, 2009; Sherman et al, 2012;)

Literature References
PubMed ID Title Journal Year
11430802 Specific disruption of a schwann cell dystrophin-related protein complex in a demyelinating neuropathy

Gillespie, CS, Fabrizi, C, Brophy, PJ, Sherman, DL

Neuron 2001
8627307 Characterization of dystroglycan-laminin interaction in peripheral nerve

Matsumura, K, Kanazawa, I, Endo, T, Yamada, H, Fukuta-Ohi, H, Anderson, LV, Shimizu, T, Kobata, A, Hori, H, Chiba, A, Campbell, KP

J. Neurochem. 1996
15356632 Restricted growth of Schwann cells lacking Cajal bands slows conduction in myelinated nerves

Fleetwood-Walker, SM, Cottrell, DF, Court, FA, Brophy, PJ, Pratt, T, Ribchester, RR, Garry, EM, Sherman, DL

Nature 2004
19321787 A laminin-2, dystroglycan, utrophin axis is required for compartmentalization and elongation of myelin segments

Wrabetz, L, Hewitt, JE, Feltri, ML, Davies, K, Uncini, A, Court, FA, Patton, BL

J. Neurosci. 2009
7925941 Dystroglycan is a binding protein of laminin and merosin in peripheral nerve

Yamada, H, Matsumura, K, Shimizu, T, Tanaka, T, Campbell, KP

FEBS Lett. 1994
22764250 Drp2 and periaxin form Cajal bands with dystroglycan but have distinct roles in Schwann cell growth

Gillespie, CS, Wu, LM, Grove, M, Brophy, PJ, Sherman, DL

J. Neurosci. 2012
20625412 Biological role of dystroglycan in Schwann cell function and its implications in peripheral nervous system diseases

Matsumura, K, Masaki, T

J. Biomed. Biotechnol. 2010
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