DYSTROPHIN-RELATED PROTEIN IS FOUND IN THE CENTRAL-NERVOUS-SYSTEM OF MICE AT VARIOUS DEVELOPMENTAL STAGES, ESPECIALLY AT THE POSTSYNAPTIC MEMBRANE

被引:28
作者
KAMAKURA, K
TADANO, Y
KAWAI, M
ISHIURA, S
NAKAMURA, R
MIYAMOTO, K
NAGATA, N
SUGITA, H
机构
[1] NATL SHIMOSHIZU HOSP,DEPT NEUROL,CHIBA,JAPAN
[2] UNIV TOKYO,INST APPL MICROBIOL,TOKYO 113,JAPAN
[3] NATL INST NEUROSCI,DIV NEUROMUSCULAR RES,TOKYO,JAPAN
关键词
DYSTROPHIN-RELATED PROTEIN; MDX MOUSE; B10; MOUSE; CENTRAL NERVOUS SYSTEM; POSTSYNAPTIC MEMBRANE;
D O I
10.1002/jnr.490370607
中图分类号
Q189 [神经科学];
学科分类号
071006 ;
摘要
Dystrophin deficiency is known to be the cause of X-linked Duchenne muscular dystrophy (DMD). A recently cloned B3-cDNA shares 80% homology with the C-terminus and actin-binding portion of dystrophin. This autosome-derived gene product is called dystrophin-related protein (DRP). DRP is known to exist in fetal muscles even in mdx mice, an animal model for X-linked DMD, but not in mature mouse muscles. We raised a polyclonal antibody against a B3-unique amino acid sequence (Ab-LDP) and investigated the existence and distribution of DRP in the central nervous system (CNS) tissues of mdx and normal control B10 mice at various stages of development using immunoblotting and immunohistochemical methods. The former shows that DRP exists in the CNS of both B10 and mdx mice, regardless of the developmental stage, with the exception that the 420 kDa DRP band of the 15-day fetus is faint. In immunohistochemical studies, the choroid plexus, some neurons, glial cells, pia mater, and blood vessels were stained with Ab-LDP. Staining intensity did not differ between B10 and mdx mice or between developmental stages except that the 15-day fetus stained only faintly. This is in contrast to the results obtained for muscles in which DRP localized to muscle membrane in embryo decreases and is assembled at the neuromuscular junction in adults. In addition, an electron microscopic study on the cerebral cortex from adult B10 mice was also performed and revealed Ab-LDP staining of the postsynaptic membrane of dendrite and the rough endoplasmic reticulum of neurons. This is a new finding and is of interest because DRP is expressed at the neuromuscular junction and its localization is similar to that of the acetylcholine receptor. (C) 1994 Wiley-Liss, Inc.
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页码:728 / 734
页数:7
相关论文
共 24 条
  • [1] MOSAIC EXPRESSION OF DYSTROPHIN IN SYMPTOMATIC CARRIERS OF DUCHENNES MUSCULAR-DYSTROPHY
    ARAHATA, K
    ISHIHARA, T
    KAMAKURA, K
    TSUKAHARA, T
    ISHIURA, S
    BABA, C
    MATSUMOTO, T
    NONAKA, I
    SUGITA, H
    [J]. NEW ENGLAND JOURNAL OF MEDICINE, 1989, 320 (03) : 138 - 142
  • [2] IMMUNOSTAINING OF SKELETAL AND CARDIAC-MUSCLE SURFACE-MEMBRANE WITH ANTIBODY AGAINST DUCHENNE MUSCULAR-DYSTROPHY PEPTIDE
    ARAHATA, K
    ISHIURA, S
    ISHIGURO, T
    TSUKAHARA, T
    SUHARA, Y
    EGUCHI, C
    ISHIHARA, T
    NONAKA, I
    OZAWA, E
    SUGITA, H
    [J]. NATURE, 1988, 333 (6176) : 861 - 863
  • [3] ACTIVATION AND BLOCKING OF NEURONAL NICOTINIC ACETYLCHOLINE-RECEPTOR RECONSTITUTED IN XENOPUS OOCYTES
    BERTRAND, D
    BALLIVET, M
    RUNGGER, D
    [J]. PROCEEDINGS OF THE NATIONAL ACADEMY OF SCIENCES OF THE UNITED STATES OF AMERICA, 1990, 87 (05) : 1993 - 1997
  • [4] EXPRESSION OF THE MURINE DUCHENNE MUSCULAR-DYSTROPHY GENE IN MUSCLE AND BRAIN
    CHAMBERLAIN, JS
    PEARLMAN, JA
    MUZNY, DM
    GIBBS, RA
    RANIER, JE
    REEVES, AA
    CASKEY, CT
    [J]. SCIENCE, 1988, 239 (4846) : 1416 - 1418
  • [5] TRANSCRIPTION OF THE DYSTROPHIN GENE IN HUMAN-MUSCLE AND NON-MUSCLE TISSUES
    CHELLY, J
    KAPLAN, JC
    MAIRE, P
    GAUTRON, S
    KAHN, A
    [J]. NATURE, 1988, 333 (6176) : 858 - 860
  • [6] HATTORI T, 1989, BIOMED RES-TOKYO, V10, P355
  • [7] DYSTROPHIN - THE PROTEIN PRODUCT OF THE DUCHENNE MUSCULAR-DYSTROPHY LOCUS
    HOFFMAN, EP
    BROWN, RH
    KUNKEL, LM
    [J]. CELL, 1987, 51 (06) : 919 - 928
  • [8] DISTAL TRANSCRIPT OF THE DYSTROPHIN GENE INITIATED FROM AN ALTERNATIVE 1ST EXON AND ENCODING A 75-KDA PROTEIN WIDELY DISTRIBUTED IN NONMUSCLE TISSUES
    HUGNOT, JP
    GILGENKRANTZ, H
    VINCENT, N
    CHAFEY, P
    MORRIS, GE
    MONACO, AP
    BERWALDNETTER, Y
    KOULAKOFF, A
    KAPLAN, JC
    KAHN, A
    CHELLY, J
    [J]. PROCEEDINGS OF THE NATIONAL ACADEMY OF SCIENCES OF THE UNITED STATES OF AMERICA, 1992, 89 (16) : 7506 - 7510
  • [9] ANTIBODY AGAINST THE C-TERMINAL PORTION OF DYSTROPHIN CROSS-REACTS WITH THE 400 KDA PROTEIN IN THE PIA MATER OF DYSTROPHIN-DEFICIENT MDX MOUSE-BRAIN
    ISHIURA, S
    ARAHATA, K
    TSUKAHARA, T
    KOGA, R
    ANRAKU, H
    YAMAGUCHI, M
    KIKUCHI, T
    NONAKA, I
    SUGITA, H
    [J]. JOURNAL OF BIOCHEMISTRY, 1990, 107 (04) : 510 - 513
  • [10] THE SUBCELLULAR-DISTRIBUTION OF CHROMOSOME 6-ENCODED DYSTROPHIN-RELATED PROTEIN IN THE BRAIN
    KHURANA, TS
    WATKINS, SC
    KUNKEL, LM
    [J]. JOURNAL OF CELL BIOLOGY, 1992, 119 (02) : 357 - 366