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Prepublished online as a Blood First Edition Paper on May 15, 2003; DOI 10.1182/blood-2002-06-1705. This Article Full Text (PDF) Supplemental Table All Versions of this Article: 2002-06-1705v1 102/6/2268    most recent Alert me when this article is cited Alert me if a correction is posted Services Email this article to a friend Similar articles in this journal Similar articles in PubMed Alert me to new issues of the journal Download to citation manager Rights and Permissions Citing Articles Citing Articles via HighWire Citing Articles via CrossRef Citing Articles via Google Scholar Google Scholar Articles by Fricke, B. Articles by Stewart, G. W Search for Related Content PubMed PubMed Citation Articles by Fricke, B. Articles by Stewart, G. W Social Bookmarking                   What's this? Submitted July 1, 2002 Accepted April 26, 2003 The 'stomatin' gene and protein in overhydrated hereditary stomatocytosis Britta Fricke, A C Argent, Margaret C Chetty, Arnold R Pizzey, E J Turner, Mei M Ho, Achille M Iolascon, Monika von Duering, and Gordon W Stewart* Department of Medicine, University College London, London, United Kingdom Abteilung fur Neuroanatomie, Institut fur Anatomie MA 6/152, Ruhr-Universitat, Bochum, Germany Department of Bacteriology, National Institute for Biological Standards and Control, Hertfordshire, United Kingdom Department of Biochemistry and Molecular Biology, Universita Federico II, Naples, Italy Department of Hematology, University College London, London, United Kingdom * Corresponding author; email: g.stewart{at}ucl.ac.uk. In 'overhydrated hereditary stomatocytosis,' Coomassie and silver-stained polyacrylamide gels show an apparently complete deficit of the 32kD membrane protein, 'stomatin.' We have used an anti-stomatin antibody to examine peripheral blood films, bone marrow, splenic tissue and hepatic tissue from these patients by immunocytochemistry. This technique revealed that, in fact, some red cells did show positive stomatin immunoreactivity; and consistent with this result, western blot analysis of the red cell membranes confirmed that about 1/20-1/50 of the normal amount of stomatin was in fact present. Flow cytometry, combining immunoreactive quantitation of stomatin expression with thiazole orange staining for reticulocytes, showed that in OHSt, the young cells had more stomatin. Magnetic-activated cell separation studies, using beads to which an anti-transferrin receptor antibody was conjugated, confirmed that in OHSt there was a correspondence between expression of stomatin and the transferrin receptor. Immunocytochemistry and western blotting revealed that in OHSt patients, the protein was present in spleen, liver, neutrophils, platelets, monocytes and about 50% of the peripheral lymphocytes, with the same distribution as in normal controls. Neither southern blots, nor direct sequencing of multiple subclones of the cDNA, nor sequencing of amplicons from genomic DNA revealed any significant abnormality. The deficiency of stomatin from red cells appears to be due to a loss of stomatin from these red cells on maturation in the bone marrow and in the circulation. CiteULike    Connotea    Del.icio.us    Digg    Reddit    Technorati    What's this? This article has been cited by other articles: M. Mairhofer, M. Steiner, U. Salzer, and R. Prohaska Stomatin-like Protein-1 Interacts with Stomatin and Is Targeted to Late Endosomes J. Biol. Chem., October 16, 2009; 284(42): 29218 - 29229. [Abstract] [Full Text] [PDF] J. F. Flatt and L. J. Bruce The hereditary stomatocytoses Haematologica, August 1, 2009; 94(8): 1039 - 1041. [Full Text] [PDF] M. Fujiwara, S. Hamada, M. Hiratsuka, Y. Fukao, T. Kawasaki, and K. Shimamoto Proteome Analysis of Detergent-Resistant Membranes (DRMs) Associated with OsRac1-Mediated Innate Immunity in Rice Plant Cell Physiol., July 1, 2009; 50(7): 1191 - 1200. [Abstract] [Full Text] [PDF] L. J. Bruce, H. Guizouarn, N. M. Burton, N. Gabillat, J. Poole, J. F. Flatt, R. L. Brady, F. Borgese, J. Delaunay, and G. W. Stewart The monovalent cation leak in overhydrated stomatocytic red blood cells results from amino acid substitutions in the Rh-associated glycoprotein Blood, February 5, 2009; 113(6): 1350 - 1357. [Abstract] [Full Text] [PDF] B. T. Carroll, G. R. Dubyak, M. M. Sedensky, and P. G. Morgan Sulfated Signal from ASJ Sensory Neurons Modulates Stomatin-dependent Coordination in Caenorhabditis elegans J. Biol. Chem., November 24, 2006; 281(47): 35989 - 35996. [Abstract] [Full Text] [PDF] P. G. Gallagher Red Cell Membrane Disorders Hematology, January 1, 2005; 2005(1): 13 - 18. [Abstract] [Full Text] [PDF] M. P. Price, R. J. Thompson, J. O. Eshcol, J. A. Wemmie, and C. J. Benson Stomatin Modulates Gating of Acid-sensing Ion Channels J. Biol. Chem., December 17, 2004; 279(51): 53886 - 53891. [Abstract] [Full Text] [PDF]

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