PMID- 10441321
OWN - NLM
STAT- MEDLINE
DCOM- 20000127
LR  - 20190513
IS  - 0964-6906 (Print)
IS  - 0964-6906 (Linking)
VI  - 8
IP  - 9
DP  - 1999 Sep
TI  - Loss of the sarcoglycan complex and sarcospan leads to muscular dystrophy in
      beta-sarcoglycan-deficient mice.
PG  - 1589-98
AB  - beta-Sarcoglycan, one of the subunits of the sarcoglycan complex, is a
      transmembranous glycoprotein which associates with dystrophin and is the molecule
      responsible for beta-sarcoglycanopathy, a Duchenne-like autosomal recessive
      muscular dystrophy. To develop an animal model of beta-sarcoglycanopathy and to
      clarify the role of beta-sarcoglycan in the pathogenesis of the muscle
      degeneration in vivo, we developed beta-sarcoglycan-deficient mice using a gene
      targeting technique. beta-Sarcoglycan-deficient mice (BSG(-)(/-)mice) exhibited
      progressive muscular dystrophy with extensive degeneration and regeneration. The 
      BSG(-)(/-)mice also exhibited muscular hypertrophy characteristic of
      beta-sarcoglycanopathy. Immunohistochemical and immunoblot analyses of
      BSG(-)(/-)mice demonstrated that deficiency of beta-sarcoglycan also caused loss 
      of all of the other sarcoglycans as well as of sarcospan in the sarcolemma. On
      the other hand, laminin-alpha2, alpha- and beta-dystroglycan and dystrophin were 
      still present in the sarcolemma. However, the dystrophin-dystroglycan complex in 
      BSG(-)(/-)mice was unstable compared with that in the wild-type mice. Our data
      suggest that loss of the sarcoglycan complex and sarcospan alone is sufficient to
      cause muscular dystrophy, that beta-sarcoglycan is an important protein for
      formation of the sarcoglycan complex associated with sarcospan and that the role 
      of the sarcoglycan complex and sarcospan may be to strengthen the dystrophin axis
      connecting the basement membrane with the cytoskeleton.
FAU - Araishi, K
AU  - Araishi K
AD  - Division of Cell Biology, National Institute of Neuroscience, National Center of 
      Neurology and Psychiatry, Kodaira, Tokyo, 187-8502, Japan.
FAU - Sasaoka, T
AU  - Sasaoka T
FAU - Imamura, M
AU  - Imamura M
FAU - Noguchi, S
AU  - Noguchi S
FAU - Hama, H
AU  - Hama H
FAU - Wakabayashi, E
AU  - Wakabayashi E
FAU - Yoshida, M
AU  - Yoshida M
FAU - Hori, T
AU  - Hori T
FAU - Ozawa, E
AU  - Ozawa E
LA  - eng
SI  - GENBANK/AB024920
SI  - GENBANK/AB024921
SI  - GENBANK/AB024922
SI  - GENBANK/AB024923
PT  - Journal Article
PT  - Research Support, Non-U.S. Gov't
PL  - England
TA  - Hum Mol Genet
JT  - Human molecular genetics
JID - 9208958
RN  - 0 (Carrier Proteins)
RN  - 0 (Cytoskeletal Proteins)
RN  - 0 (DAG1 protein, human)
RN  - 0 (Dystrophin)
RN  - 0 (Membrane Glycoproteins)
RN  - 0 (Membrane Proteins)
RN  - 0 (Muscle Proteins)
RN  - 0 (Neoplasm Proteins)
RN  - 0 (RNA, Messenger)
RN  - 0 (SSPN protein, human)
RN  - 0 (Sspn protein, mouse)
RN  - 146888-27-9 (Dystroglycans)
SB  - IM
MH  - Animals
MH  - Carrier Proteins/genetics/metabolism
MH  - Cytoskeletal Proteins/*deficiency/genetics
MH  - Disease Models, Animal
MH  - Dystroglycans
MH  - Dystrophin/metabolism
MH  - Fluorescent Antibody Technique
MH  - Humans
MH  - Membrane Glycoproteins/*deficiency/genetics
MH  - Membrane Proteins/genetics/metabolism
MH  - Mice
MH  - Mice, Knockout
MH  - Molecular Sequence Data
MH  - Muscle Proteins/deficiency/genetics
MH  - Muscle, Skeletal/metabolism
MH  - Muscular Dystrophies/*genetics
MH  - Myocardium/cytology
MH  - *Neoplasm Proteins
MH  - Phenotype
MH  - RNA, Messenger/metabolism
EDAT- 1999/08/11 00:00
MHDA- 1999/08/11 00:01
CRDT- 1999/08/11 00:00
PHST- 1999/08/11 00:00 [pubmed]
PHST- 1999/08/11 00:01 [medline]
PHST- 1999/08/11 00:00 [entrez]
AID - ddc203 [pii]
AID - 10.1093/hmg/8.9.1589 [doi]
PST - ppublish
SO  - Hum Mol Genet. 1999 Sep;8(9):1589-98. doi: 10.1093/hmg/8.9.1589.