PMP2 is a peripheral membrane protein that plays a significant role in the stability and function of the myelin sheath. The myelin sheath is a multilayered membrane structure that insulates axons, facilitating the rapid transmission of nerve impulses. PMP2 binds both the membrane layers of the myelin sheath and monomeric lipids, contributing to the structural integrity and functionality of the sheath .
The protein has a barrel-shaped structure with a cavity that can bind fatty acids and cholesterol. This binding capability suggests that PMP2 may play a role in lipid transport within Schwann cells, which are responsible for the formation and maintenance of the myelin sheath .
Research on PMP2 has provided valuable insights into its role in myelin stability and lipid transport. Structural studies have revealed that PMP2 can bind cholesterol, one of the most abundant lipids in myelin, supporting its potential role in lipid metabolism within the myelin sheath .
Mutations in the PMP2 gene have been associated with Charcot-Marie-Tooth Disease, a hereditary neuropathy characterized by progressive loss of muscle tissue and touch sensation . Understanding the structure and function of PMP2 is crucial for developing therapeutic strategies for such demyelinating diseases.