Molecular characterization of sialophorin (CD43), the lymphocyte surface sialoglycoprotein defective in Wiskott-Aldrich syndrome

Journal Article · Sat Apr 01 00:00:00 EST 1989 · Proceedings of the National Academy of Sciences of the United States of America; (USA)

DOI:https://doi.org/10.1073/pnas.86.8.2819· OSTI ID:5190413

Shelley, C S; Remold-O'Donnell, E; Davis, III, A E; Bruns, G A.P.; Rosen, F S; Carroll, M C; Whitehead, A S ^[1]

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1. Harvard Medical School, Boston, MA (USA)

Sialophorin (CD43) of leukocytes and platelets is a surface sialoglycoprotein that is phenotypically defective on lymphocytes of patients with the X chromosome-linked immunodeficiency Wiskott-Aldrich syndrome. Previous studies with monoclonal antibodies indicate that sialophorin is a component of a T-lymphocyte activation pathway. Here the authors describe the cDNA cloning and derived amino acid sequence of human sialophorin. The sequence predicts an integral membrane polypeptide with an N-terminal hydrophobic signal region followed by a mucin-like 235-residue extracellular region with a uniform distribution of 46 serine, 47 threonine, and 24 proline residues. This is followed by a 23-residue transmembrane region and a 123-residue C-terminal intracellular region. These latter regions have been highly conserved during evolution; the intracellular region contains a number of potential phosphorylation sites that might mediate transduction of activation signals. The chromosomal location of the sialophorin gene was determined and the implications of this assignment for the pathogenesis of the Wiskott-Aldrich syndrome are discussed.

• OSTI ID: 5190413
• Journal Information: Proceedings of the National Academy of Sciences of the United States of America; (USA), Vol. 86:8; ISSN 0027-8424
• Country of Publication: United States
• Language: English

Related Subjects

59 BASIC BIOLOGICAL SCIENCES
HEREDITARY DISEASES
ETIOLOGY
LYMPHOCYTES
CHEMICAL ACTIVATION
MEMBRANE PROTEINS
AMINO ACID SEQUENCE
BLOOD PLATELETS
DNA SEQUENCING
GENETIC MAPPING
GLYCOPROTEINS
MAN
MESSENGER-RNA
MONOCLONAL ANTIBODIES
PHOSPHORUS 32
X CHROMOSOME
ANIMAL CELLS
ANIMALS
ANTIBODIES
BETA DECAY RADIOISOTOPES
BETA-MINUS DECAY RADIOISOTOPES
BIOLOGICAL MATERIALS
BLOOD
BLOOD CELLS
BODY FLUIDS
CHROMOSOMES
CONNECTIVE TISSUE CELLS
DAYS LIVING RADIOISOTOPES
DISEASES
HETEROCHROMOSOMES
ISOTOPES
LEUKOCYTES
LIGHT NUCLEI
MAMMALS
MAPPING
MATERIALS
MOLECULAR STRUCTURE
NUCLEI
NUCLEIC ACIDS
ODD-ODD NUCLEI
ORGANIC COMPOUNDS
PHOSPHORUS ISOTOPES
PRIMATES
PROTEINS
RADIOISOTOPES
RNA
SOMATIC CELLS
STRUCTURAL CHEMICAL ANALYSIS
VERTEBRATES
550200* - Biochemistry