Myelin protein zero explained

Myelin protein zero (P0, MPZ) is a single membrane glycoprotein^[1] which in humans is encoded by the MPZ gene. P0 is a major structural component of the myelin sheath in the peripheral nervous system (PNS).^[2] Myelin protein zero is expressed by Schwann cells and accounts for over 50% of all proteins in the peripheral nervous system, making it the most common protein expressed in the PNS. Mutations in myelin protein zero can cause myelin deficiency and are associated with neuropathies like Charcot–Marie–Tooth disease and Dejerine–Sottas disease.^[3]

Structure

Symbol:	Myelin-PO_C
Myelin-PO_C

Notes and References

1. Magnaghi V, Cavarretta I, Galbiati M, Martini L, Melcangi RC . Neuroactive steroids and peripheral myelin proteins . Brain Research. Brain Research Reviews . 37 . 1–3 . 360–71 . November 2001 . 11744100 . 10.1016/s0165-0173(01)00140-0 . 8004545 .
2. Shy ME . Peripheral neuropathies caused by mutations in the myelin protein zero . Journal of the Neurological Sciences . 242 . 1–2 . 55–66 . March 2006 . 16414078 . 10.1016/j.jns.2005.11.015 . 32802793 .
3. Shy ME, Jáni A, Krajewski K, Grandis M, Lewis RA, Li J, Shy RR, Balsamo J, Lilien J, Garbern JY, Kamholz J . Phenotypic clustering in MPZ mutations . Brain . 127 . Pt 2 . 371–84 . February 2004 . 14711881 . 10.1093/brain/awh048 . free .