PMID- 10189375
OWN - NLM
STAT- MEDLINE
DCOM- 19990506
LR  - 20190508
IS  - 0021-9525 (Print)
IS  - 0021-9525 (Linking)
VI  - 145
IP  - 1
DP  - 1999 Apr 5
TI  - Membrane targeting and stabilization of sarcospan is mediated by the sarcoglycan 
      subcomplex.
PG  - 153-65
AB  - The dystrophin-glycoprotein complex (DGC) is a multisubunit complex that spans
      the muscle plasma membrane and forms a link between the F-actin cytoskeleton and 
      the extracellular matrix. The proteins of the DGC are structurally organized into
      distinct subcomplexes, and genetic mutations in many individual components are
      manifested as muscular dystrophy. We recently identified a unique tetraspan-like 
      dystrophin-associated protein, which we have named sarcospan (SPN) for its
      multiple sarcolemma spanning domains (Crosbie, R.H., J. Heighway, D.P. Venzke,
      J.C. Lee, and K.P. Campbell. 1997. J. Biol. Chem. 272:31221-31224). To probe
      molecular associations of SPN within the DGC, we investigated SPN expression in
      normal muscle as a baseline for comparison to SPN's expression in animal models
      of muscular dystrophy. We show that, in addition to its sarcolemma localization, 
      SPN is enriched at the myotendinous junction (MTJ) and neuromuscular junction
      (NMJ), where it is a component of both the dystrophin- and utrophin-glycoprotein 
      complexes. We demonstrate that SPN is preferentially associated with the
      sarcoglycan (SG) subcomplex, and this interaction is critical for stable
      localization of SPN to the sarcolemma, NMJ, and MTJ. Our experiments indicate
      that assembly of the SG subcomplex is a prerequisite for targeting SPN to the
      sarcolemma. In addition, the SG- SPN subcomplex functions to stabilize
      alpha-dystroglycan to the muscle plasma membrane. Taken together, our data
      provide important information about assembly and function of the SG-SPN
      subcomplex.
FAU - Crosbie, R H
AU  - Crosbie RH
AD  - Howard Hughes Medical Institute, Department of Physiology and Biophysics,
      University of Iowa College of Medicine, Iowa City, Iowa 52242, USA.
FAU - Lebakken, C S
AU  - Lebakken CS
FAU - Holt, K H
AU  - Holt KH
FAU - Venzke, D P
AU  - Venzke DP
FAU - Straub, V
AU  - Straub V
FAU - Lee, J C
AU  - Lee JC
FAU - Grady, R M
AU  - Grady RM
FAU - Chamberlain, J S
AU  - Chamberlain JS
FAU - Sanes, J R
AU  - Sanes JR
FAU - Campbell, K P
AU  - Campbell KP
LA  - eng
SI  - GENBANK/AF016028
SI  - GENBANK/AF120276
SI  - GENBANK/U02487
GR  - T32 HL007121/HL/NHLBI NIH HHS/United States
GR  - DK25295/DK/NIDDK NIH HHS/United States
GR  - HL07121/HL/NHLBI NIH HHS/United States
GR  - R01NS1915/NS/NINDS NIH HHS/United States
PT  - Comparative Study
PT  - Journal Article
PT  - Research Support, Non-U.S. Gov't
PT  - Research Support, U.S. Gov't, P.H.S.
PL  - United States
TA  - J Cell Biol
JT  - The Journal of cell biology
JID - 0375356
RN  - 0 (Carrier Proteins)
RN  - 0 (Cytoskeletal Proteins)
RN  - 0 (DAG1 protein, human)
RN  - 0 (Dystrophin)
RN  - 0 (Macromolecular Substances)
RN  - 0 (Membrane Glycoproteins)
RN  - 0 (Membrane Proteins)
RN  - 0 (Neoplasm Proteins)
RN  - 0 (SSPN protein, human)
RN  - 0 (Sarcoglycans)
RN  - 0 (Sspn protein, mouse)
RN  - 0 (Utrn protein, mouse)
RN  - 0 (Utrophin)
RN  - 146888-27-9 (Dystroglycans)
SB  - IM
MH  - Amino Acid Sequence
MH  - Animals
MH  - Biological Transport
MH  - CHO Cells
MH  - Cardiomyopathy, Hypertrophic/genetics/metabolism/pathology
MH  - Carrier Proteins/*metabolism
MH  - Cricetinae
MH  - Cytoskeletal Proteins/deficiency/genetics/*physiology
MH  - Dystroglycans
MH  - Dystrophin/deficiency/genetics/*physiology
MH  - Humans
MH  - Hydrogen-Ion Concentration
MH  - Macromolecular Substances
MH  - Male
MH  - Membrane Glycoproteins/deficiency/genetics/*physiology
MH  - Membrane Proteins/deficiency/genetics/*metabolism/physiology
MH  - Mice
MH  - Mice, Knockout
MH  - Mice, Transgenic
MH  - Models, Molecular
MH  - Molecular Sequence Data
MH  - Muscle, Skeletal/metabolism
MH  - Muscular Dystrophy, Animal/genetics/*metabolism
MH  - *Neoplasm Proteins
MH  - Neuromuscular Junction/metabolism
MH  - Rabbits
MH  - Sarcoglycans
MH  - Sarcolemma/metabolism/ultrastructure
MH  - Sequence Alignment
MH  - Species Specificity
MH  - Tendons/metabolism
MH  - Transfection
MH  - Utrophin
PMC - PMC2148225
EDAT- 1999/04/06 00:00
MHDA- 1999/04/06 00:01
CRDT- 1999/04/06 00:00
PHST- 1999/04/06 00:00 [pubmed]
PHST- 1999/04/06 00:01 [medline]
PHST- 1999/04/06 00:00 [entrez]
AID - 10.1083/jcb.145.1.153 [doi]
PST - ppublish
SO  - J Cell Biol. 1999 Apr 5;145(1):153-65. doi: 10.1083/jcb.145.1.153.