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- W2107201375 abstract "Purpose of review This review discusses the mechanisms involved in the generation of thorny red blood cells (RBCs), known as acanthocytes, in patients with neuroacanthocytosis, a heterogenous group of neurodegenerative hereditary disorders that include chorea-acanthocytosis (ChAc) and McLeod syndrome (MLS). Recent findings Although molecular defects associated with neuroacanthocytosis have been identified recently, their pathophysiology and the related RBC abnormalities are largely unknown. Studies in ChAc RBCs have shown an altered association between the cytoskeleton and the integral membrane protein compartment in the absence of major changes in RBC membrane composition. In ChAc RBCs, abnormal Lyn kinase activation in a Syk-independent fashion has been reported recently, resulting in increased band 3 tyrosine phosphorylation and perturbation of the stability of the multiprotein band 3-based complexes bridging the membrane to the spectrin-based membrane skeleton. Similarly, in MLS, the absence of XK-protein, which is associated with the spectrin–actin–4.1 junctional complex, is associated with an abnormal membrane protein phosphorylation state, with destabilization of the membrane skeletal network resulting in generation of acanthocytes. Summary A novel mechanism in generation of acanthocytes involving abnormal Lyn activation, identified in ChAc, expands the acanthocytosis phenomenon toward protein–protein interactions, controlled by phosphorylation-related abnormal signaling." @default.
- W2107201375 created "2016-06-24" @default.
- W2107201375 creator A5017035150 @default.
- W2107201375 creator A5034746599 @default.
- W2107201375 creator A5043729712 @default.
- W2107201375 date "2014-05-01" @default.
- W2107201375 modified "2023-09-26" @default.
- W2107201375 title "Abnormal red cell features associated with hereditary neurodegenerative disorders" @default.
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- W2107201375 doi "https://doi.org/10.1097/moh.0000000000000035" @default.
- W2107201375 hasPubMedId "https://pubmed.ncbi.nlm.nih.gov/24626044" @default.
- W2107201375 hasPublicationYear "2014" @default.
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