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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 ...
Central pontine myelinolysis is a neurological condition involving severe damage to the myelin sheath of nerve cells in the pons (an area of the brainstem). It is predominately iatrogenic (treatment-induced), and is characterized by acute paralysis, dysphagia (difficulty swallowing), dysarthria (difficulty speaking), and other neurological symptoms.
In the first group, a healthy and normal myelin is destroyed by toxic substances, chemicals, or autoimmune reactions. In the second group, the myelin is inherently abnormal and undergoes degeneration. [6] The Poser criteria named this second group dysmyelinating diseases. [7]
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.
The myelin sheaths separate from the axons at the Schmidt-Lanterman incisures first and then rapidly deteriorate and shorten to form bead-like structures. Schwann cells continue to clear up the myelin debris by degrading their own myelin, phagocytose extracellular myelin and attract macrophages to myelin debris for further phagocytosis. [11]
Astrocytes can heal partially the lesion leaving a scar. These scars (sclerae) are the known plaques or lesions usually reported in MS. A repair process, called remyelination, takes place in early phases of the disease, but the oligodendrocytes are unable to completely rebuild the cell's myelin sheath.
In myelinated axons, Schwann cells form the myelin sheath. The sheath is not continuous. Individual myelinating Schwann cells cover about 1 mm of an axon [3] – equating to about 1000 Schwann cells along a 1-m length of the axon. The gaps between adjacent Schwann cells are called nodes of Ranvier.
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.