PMID- 10545507
OWN - NLM
STAT- MEDLINE
DCOM- 19991124
LR  - 20191023
IS  - 0021-9525 (Print)
IS  - 0021-9525 (Linking)
VI  - 147
IP  - 3
DP  - 1999 Nov 1
TI  - Different dystrophin-like complexes are expressed in neurons and glia.
PG  - 645-58
AB  - Duchenne muscular dystrophy is a fatal muscle disease that is often associated
      with cognitive impairment. Accordingly, dystrophin is found at the muscle
      sarcolemma and at postsynaptic sites in neurons. In muscle, dystrophin forms part
      of a membrane-spanning complex, the dystrophin-associated protein complex (DPC). 
      Whereas the composition of the DPC in muscle is well documented, the existence of
      a similar complex in brain remains largely unknown. To determine the composition 
      of DPC-like complexes in brain, we have examined the molecular associations and
      distribution of the dystrobrevins, a widely expressed family of
      dystrophin-associated proteins, some of which are components of the muscle DPC.
      beta-Dystrobrevin is found in neurons and is highly enriched in postsynaptic
      densities (PSDs). Furthermore, beta-dystrobrevin forms a specific complex with
      dystrophin and syntrophin. By contrast, alpha-dystrobrevin-1 is found in
      perivascular astrocytes and Bergmann glia, and is not PSD-enriched.
      alpha-Dystrobrevin-1 is associated with Dp71, utrophin, and syntrophin. In the
      brains of mice that lack dystrophin and Dp71, the dystrobrevin-syntrophin
      complexes are still formed, whereas in dystrophin-deficient muscle, the assembly 
      of the DPC is disrupted. Thus, despite the similarity in primary sequence, alpha-
      and beta-dystrobrevin are differentially distributed in the brain where they form
      separate DPC-like complexes.
FAU - Blake, D J
AU  - Blake DJ
AD  - Department of Human Anatomy and Genetics, University of Oxford, Oxford OX1 3QX,
      United Kingdom. dblake@enterprise.molbiol.ox.ac.uk
FAU - Hawkes, R
AU  - Hawkes R
FAU - Benson, M A
AU  - Benson MA
FAU - Beesley, P W
AU  - Beesley PW
LA  - eng
PT  - Journal Article
PT  - Research Support, Non-U.S. Gov't
PL  - United States
TA  - J Cell Biol
JT  - The Journal of cell biology
JID - 0375356
RN  - 0 (Cytoskeletal Proteins)
RN  - 0 (Dystrophin)
RN  - 0 (Dystrophin-Associated Proteins)
RN  - 0 (Immune Sera)
RN  - 0 (Membrane Proteins)
RN  - 0 (Muscle Proteins)
RN  - 0 (Nerve Tissue Proteins)
RN  - 0 (Protein Isoforms)
RN  - 0 (Utrn protein, mouse)
RN  - 0 (Utrophin)
RN  - 0 (apo-dystrophin 1)
RN  - 0 (dystrobrevin)
RN  - 0 (postsynaptic density proteins)
RN  - 0 (syntrophin)
SB  - IM
MH  - Amino Acid Sequence
MH  - Animals
MH  - Brain/cytology/metabolism
MH  - Cytoskeletal Proteins/metabolism
MH  - Dystrophin/analogs & derivatives/deficiency/genetics/*metabolism
MH  - *Dystrophin-Associated Proteins
MH  - Gene Deletion
MH  - Immune Sera/immunology
MH  - Membrane Proteins/deficiency/genetics/immunology/*metabolism
MH  - Mice
MH  - Mice, Transgenic
MH  - Molecular Sequence Data
MH  - Muscle Proteins/metabolism
MH  - Muscular Dystrophy, Duchenne/metabolism
MH  - Nerve Tissue Proteins/metabolism
MH  - Neuroglia/*metabolism
MH  - Neurons/cytology/*metabolism
MH  - Protein Binding
MH  - Protein Isoforms/deficiency/genetics/immunology/metabolism
MH  - Rats
MH  - Solubility
MH  - Synaptosomes/metabolism
MH  - Utrophin
PMC - PMC2151186
EDAT- 1999/11/05 00:00
MHDA- 1999/11/05 00:01
CRDT- 1999/11/05 00:00
PHST- 1999/11/05 00:00 [pubmed]
PHST- 1999/11/05 00:01 [medline]
PHST- 1999/11/05 00:00 [entrez]
AID - 10.1083/jcb.147.3.645 [doi]
PST - ppublish
SO  - J Cell Biol. 1999 Nov 1;147(3):645-58. doi: 10.1083/jcb.147.3.645.