Peripheral myelin protein-22 is expressed in CNS myelin
نویسندگان
چکیده
منابع مشابه
Peripheral myelin protein 22 and protein zero: a novel association in peripheral nervous system myelin.
Mutations found in the two major glycosylated transmembrane proteins of the PNS myelin, the peripheral myelin protein zero (P0) and peripheral myelin protein 22 (PMP22), have been independently associated with the most common hereditary demyelinating peripheral neuropathies. Genotype-phenotype correlations in humans and transgenic animals have provided functional evidence that P0 and PMP22 are ...
متن کاملPeripheral myelin protein 22 alters membrane architecture
Peripheral myelin protein 22 (PMP22) is highly expressed in myelinating Schwann cells of the peripheral nervous system. PMP22 genetic alterations cause the most common forms of Charcot-Marie-Tooth disease (CMTD), which is characterized by severe dysmyelination in the peripheral nerves. However, the functions of PMP22 in Schwann cell membranes remain unclear. We demonstrate that reconstitution o...
متن کاملUncoupling of myelin assembly and schwann cell differentiation by transgenic overexpression of peripheral myelin protein 22.
We have generated previously transgenic rats that overexpress peripheral myelin protein 22 (PMP22) in Schwann cells. In the nerves of these animals, Schwann cells have segregated with axons to the normal 1:1 ratio but remain arrested at the promyelinating stage, apparently unable to elaborate myelin sheaths. We have examined gene expression of these dysmyelinating Schwann cells using semiquanti...
متن کاملNeurons promote the translocation of peripheral myelin protein 22 into myelin.
Schwann cells express low levels of myelin proteins in the absence of neurons. When Schwann cells and neurons are cultured together the production of myelin proteins is elevated, and myelin is formed. For peripheral myelin protein 22 (PMP22), the exact amount of protein produced is critical, because peripheral neuropathies result from its underexpression or overexpression. In this study we exam...
متن کاملPeripheral myelin protein 22 is a constituent of intercellular junctions in epithelia.
Alterations in peripheral myelin protein 22 (PMP22) gene expression are associated with a host of heritable demyelinating peripheral neuropathies, yet the function of the protein remains unknown. PMP22 expression is highest in myelinating Schwann cells of peripheral nerves; however, significant levels of PMP22 mRNAs can be detected in a variety of non-neural tissue, including epithelia. To date...
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ژورنال
عنوان ژورنال: Translational Neuroscience
سال: 2010
ISSN: 2081-6936,2081-3856
DOI: 10.2478/v10134-010-0038-3