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Myelin proteolipid protein (PLP or lipophilin) [1] is the major myelin protein from the central nervous system (CNS). It plays an important role in the formation or maintenance of the multilamellar structure of myelin.
Proteolipid protein 1 (PLP1) is a form of myelin proteolipid protein (PLP). Mutations in PLP1 are associated with Pelizaeus–Merzbacher disease.It is a 4 transmembrane domain protein which is proposed to bind other copies of itself on the extracellular side of the membrane.
Protein content includes myelin basic protein (MBP), [21] which is abundant in the CNS where it plays a critical, non-redundant role in formation of compact myelin; myelin oligodendrocyte glycoprotein (MOG), [22] which is specific to the CNS; and proteolipid protein (PLP), [23] which is the most abundant protein in CNS myelin, but only a minor ...
Myelin basic protein (MBP) is a protein believed [weasel words] to be important in the process of myelination of nerves in the nervous system. The myelin sheath is a multi-layered membrane, unique to the nervous system, that functions as an insulator to greatly increase the velocity of axonal impulse conduction . [ 5 ]
In addition, this proteolipid is localized in compact myelin of cells in the nervous system and has been implicated in myelin biogenesis and/or function. The protein plays a role in the formation, stabilization and maintenance of glycosphingolipid -enriched membrane microdomains .
Pelizaeus–Merzbacher disease is caused by X-linked recessive mutations in the major myelin protein proteolipid protein 1 (PLP1). This causes hypomyelination in the central nervous system and severe neurological disease. The majority of mutations result in duplications of the entire PLP1 gene.
A proteolipid is a protein covalently linked to lipid molecules, which can be fatty acids, isoprenoids or sterols. The process of such a linkage is known as protein lipidation , and falls into the wider category of acylation and post-translational modification .
Myelinogenesis is the formation and development of myelin sheaths in the nervous system, typically initiated in late prenatal neurodevelopment and continuing throughout postnatal development. [1] Myelinogenesis continues throughout the lifespan to support learning and memory via neural circuit plasticity as well as remyelination following ...