Expression of developmentally regulated cytoskeleton and cell surface proteins in childhood spinal muscular atrophies

Journal of the Neurological Sciences
C SoubrouillardD Figarella-Branger

Abstract

Expression of some developmentally regulated cytoskeleton components (desmin, vimentin and myosin heavy chain isoforms) and cell surface proteins (including neural cell adhesion molecule (NCAM), its polysialylated (PSA) isoform and CD24) have been studied by immunohistochemical detection in a series of 23 infantile spinal muscular atrophies (SMA). According to the clinical classification established by Byers and Banker in 1961, 8 cases were type I SMA (Werdnig-Hoffmann's disease), 10 cases were type II (intermediate form), and 5 cases were type III (Kugelberg-Welander's disease). In 15 cases, the percentage of immunoreactive fibers with the various antibodies used has been quantified and the results correlated with clinical data. The aim of the study was to search for variations in the pattern of expression of the proteins to improve the accuracy of diagnosis and prognosis, and to gain an understanding of the pathological processes involved in SMA. The results showed that the pattern of expression of these cytoskeleton and cell surface proteins is abnormal in all types of SMA. However, it was strikingly different in type I and II SMA as opposed to type III. In type I and II SMA, strong NCAM and developmental myosin heavy chain ...Continue Reading

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Citations

Feb 7, 1998·Journal of the Neurological Sciences·S M Chou, H S Wang
Jun 19, 1998·Neuromuscular Disorders : NMD·T R HelliwellR E Appleton
Nov 17, 1999·Neuropathology and Applied Neurobiology·A Winter, A Bornemann
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Sep 1, 2007·Journal of Child Neurology·Charlotte J Sumner
May 9, 2014·Journal of Neuropathology and Experimental Neurology·Rebeca Martínez-HernándezEduardo F Tizzano

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