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In the PNS, myelin protein zero (MPZ or P0) has a similar role to that of PLP in the CNS in that it is involved in holding together the multiple concentric layers of glial cell membrane that constitute the myelin sheath. The primary lipid of myelin is a glycolipid called galactocerebroside. The intertwining hydrocarbon chains of sphingomyelin ...
Myelinated neuron. HNPP is typically caused by autosomal dominant deletion of PMP22 (peripheral myelin protein 22 at locus 17p11.2). [8] PMP22 displays haploinsufficiency, such that the normal copy of the gene's activity is insufficient to compensate for loss of function of the affected copy. [9]
Inflammatory demyelinating diseases (IDDs), sometimes called Idiopathic (IIDDs) due to the unknown etiology of some of them, are a heterogenous group of demyelinating diseases - conditions that cause damage to myelin, the protective sheath of nerve fibers - that occur against the background of an acute or chronic inflammatory process.
The myelin sheath that surrounds and protects nerve cells is made by cells called oligodendrocytes. In a person with MS, these cells are lost, so damaged myelin sheaths cannot be repaired.
A demyelinating disease refers to any disease affecting the nervous system where the myelin sheath surrounding neurons is damaged. [1] This damage disrupts the transmission of signals through the affected nerves, resulting in a decrease in their conduction ability.
Myelin is formed by oligodendrocytes in the central nervous system and Schwann cells in the peripheral nervous system.Therefore, the first stage of myelinogenesis is often defined as the differentiation of oligodendrocyte progenitor cells (OPCs) or Schwann cell progenitors into their mature counterparts, [4] followed by myelin formation around axons.
The T cells recognize myelin as foreign and attack it, explaining why these cells are also called "autoreactive lymphocytes". Demyelination, further inflammation and axonal transection are the result. The attack of myelin starts inflammatory processes, which triggers other immune cells and the release of soluble factors like cytokines and ...
EMG test is often performed together with another test called nerve conduction study, which measures the conducting function of nerves. NCV study shows loss of nerve conduction in the distal segment (3 to 4 days after injury). According to NCV study, in axonotmesis there is an absence of distal sensory-motor responses.