title: Characteristic expression of cell adhesion molecules in adhalin deficiency.
abstract: We have reported the reduction of the B1 subunit of laminin and that of heparan sulfate proteoglycan (HSPG) in two Japanese patients with adhalin deficiency. We here investigated immunohistochemically the expression of cell adhesion molecules, including intercellular adhesion molecule-1 (ICAM-1), neural cell adhesion molecule (NCAM), and CD44 (HCAM), in four Japanese patients with adhalin deficiency, compared to other types of muscular dystrophy. We found that NCAM was upregulated in a fair number of muscle fibers, regardless of the type of muscular dystrophy. ICAM-1 was detected on the rare muscle cell membrane in all patients. CD44 was barely detected on the muscle cell membrane in adhalin deficiency, in contrast to the strong expression of CD44 which was observed in other types of muscular dystrophy. These findings suggest that a different degenerative or regenerative process is involved in adhalin deficiency compared to other types of muscular dystrophy.
authors.affiliation: Third Department of Internal Medicine, Faculty of Medicine, Kagoshima University, Japan.
authors.forename: I
authors.initials: I
authors.lastname: Higuchi
authors.forename: H
authors.initials: H
authors.lastname: Fukunaga
authors.forename: F
authors.initials: F
authors.lastname: Motokura
authors.forename: T
authors.initials: T
authors.lastname: Goto
authors.forename: N
authors.initials: N
authors.lastname: Niiyama
authors.forename: M
authors.initials: M
authors.lastname: Inose
authors.forename: K
authors.initials: K
authors.lastname: Izumi
authors.forename: R
authors.initials: R
authors.lastname: Ohkubo
authors.forename: M
authors.initials: M
authors.lastname: Nakagawa
authors.forename: K
authors.initials: K
authors.lastname: Arimura
authors.forename: M
authors.initials: M
authors.lastname: Osame
mesh_terms: D000328:Adult; D001706:Biopsy; D015816:Cell Adhesion Molecules, Neuronal / Q000032:analysis / Q000096:biosynthesis*; D003598:Cytoskeletal Proteins / Q000172:deficiency*; D016326:Extracellular Matrix Proteins / Q000032:analysis / Q000096:biosynthesis; D005260:Female; D006801:Humans; D018960:Hyaluronan Receptors / Q000032:analysis / Q000096:biosynthesis; D007150:Immunohistochemistry; D018799:Intercellular Adhesion Molecule-1 / Q000032:analysis / Q000096:biosynthesis*; D008297:Male; D008562:Membrane Glycoproteins / Q000172:deficiency*; D008875:Middle Aged; D018485:Muscle Fibers, Skeletal / Q000737:chemistry / Q000378:metabolism / Q000473:pathology; D018482:Muscle, Skeletal / Q000737:chemistry / Q000378:metabolism / Q000473:pathology; D009136:Muscular Dystrophies / Q000378:metabolism / Q000473:pathology; D049031:Sarcoglycans
chemical_list: D015816:Cell Adhesion Molecules, Neuronal; D003598:Cytoskeletal Proteins; D016326:Extracellular Matrix Proteins; D018960:Hyaluronan Receptors; D008562:Membrane Glycoproteins; D049031:Sarcoglycans; D018799:Intercellular Adhesion Molecule-1