PRX
Description
The PRX (periaxin) is a protein-coding gene located on chromosome 19.
PRX may refer to: Periaxin Scaffolding protein that functions as part of a dystroglycan complex in Schwann cells, and as part of EZR and AHNAK-containing complexes in eye lens fiber cells. Required for the maintenance of the peripheral myelin sheath that is essential for normal transmission of nerve impulses and normal perception of sensory stimuli. Required for normal transport of MBP mRNA from the perinuclear to the paranodal regions. Required for normal remyelination after nerve injury. Required for normal elongation of Schwann cells and normal length of the internodes between the nodes of Ranvier. The demyelinated nodes of Ranvier permit saltatory transmission of nerve impulses; shorter internodes cause slower transmission of nerve impulses. Required for the formation of appositions between the abaxonal surface of the myelin sheath and the Schwann cell plasma membrane; the Schwann cell cytoplasm is restricted to regions between these appositions. Required for the formation of Cajal bands and of Schmidt-Lanterman incisures that correspond to short, cytoplasm-filled regions on myelinated nerves. Recruits DRP2 to the Schwann cell plasma membrane. Required for normal protein composition of the eye lens fiber cell plasma membrane and normal eye lens fiber cell morphology. Homodimer (via PDZ domain) (PubMed:24675079). Interacts with SCN10A. Found in a complex with SCN10A (By similarity). Interacts with DRP2. Identified in a dystroglycan complex that contains at least PRX, DRP2, UTRN, DMD and DAG1 (By similarity). Detected in a complex composed of at least EZR, AHNAK, PPL and PRX (By similarity). Identified in a complex with EZR, AHNAK, BFSP1, BFSP2, ANK2, PLEC, VIM and spectrin (By similarity).
PRX, also known as periaxin, plays a critical role in maintaining the structure and function of the peripheral myelin sheath, which is essential for efficient nerve impulse transmission. It acts as a scaffolding protein, forming complexes with other proteins in Schwann cells, the cells responsible for myelin production. These complexes contribute to the integrity of the myelin sheath and the proper arrangement of its components. PRX is involved in transporting myelin basic protein (MBP) mRNA from the nucleus to the paranodal regions, ensuring the correct localization of this essential myelin protein. Furthermore, PRX is required for the process of remyelination, the repair of damaged myelin sheaths after nerve injury. Additionally, PRX plays a role in the elongation of Schwann cells and the formation of the proper length of internodes, the spaces between the nodes of Ranvier, which are essential for the efficient saltatory conduction of nerve impulses. PRX is also found in complexes in eye lens fiber cells, where it contributes to the correct protein composition and morphology of the cell membrane.
PRX is also known as CMT4F.
