Targeting of the ETS factor Gabp alpha disrupts neuromuscular junction synaptic function

O'Leary, Debra A., Noakes, Peter G., Lavidis, Nick A., Kola, Ismail, Hertzog, Paul J. and Ristevski, Sika (2007) Targeting of the ETS factor Gabp alpha disrupts neuromuscular junction synaptic function. Molecular and Cellular Biology, 27 9: 3470-3480. doi:10.1128/MCB.00659-06

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Author O'Leary, Debra A.
Noakes, Peter G.
Lavidis, Nick A.
Kola, Ismail
Hertzog, Paul J.
Ristevski, Sika
Title Targeting of the ETS factor Gabp alpha disrupts neuromuscular junction synaptic function
Journal name Molecular and Cellular Biology   Check publisher's open access policy
ISSN 0270-7306
Publication date 2007-01-01
Sub-type Article (original research)
DOI 10.1128/MCB.00659-06
Open Access Status File (Publisher version)
Volume 27
Issue 9
Start page 3470
End page 3480
Total pages 11
Editor D. Bar-Sagi
Place of publication Washington, DC, United States
Publisher American Society for Microbiology
Language eng
Abstract The GA-binding protein (GABP) transcription factor has been shown in vitro to regulate the expression of the neuromuscular proteins utrophin, acetylcholine esterase, and acetylcholine receptor subunits delta and E through the N-box promoter motif (5'-CCGGAA-3'), but its in vivo function remains unknown. A single point mutation within the N-box of the gene encoding the acetylcholine receptor F subunit has been identified in several patients suffering from postsynaptic congenital myasthenic syndrome, implicating the GA-binding protein in neuromuscular function and disease. Since conventional gene targeting results in an embryonic-lethal phenotype, we used conditional targeting to investigate the role of GABP alpha in neuromuscular junction and skeletal muscle development. The diaphragm and soleus muscles from mutant mice display alterations in morphology and distribution (of acetylcholine receptor clusters at the neuromuscular junction and neurotransmission properties consistent with reduced receptor function. Furthermore, we confirmed decreased expression of the acetylcholine receptor epsilon subunit and increased expression of the gamma subunit in skeletal muscle tissues. Therefore, the GABP transcription factor aids in the structural formation and function of neuromuscular junctions by regulating the expression of postsynaptic genes.
Keyword Biochemistry & Molecular Biology
Cell Biology
Congenital Myasthenic Syndromes
Epsilon-subunit Gene
Acetylcholine-receptor Deficiency
Transcription Factor Gabp
Transmitter Release
Adult Mice
Q-Index Code C1
Q-Index Status Confirmed Code

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Created: Tue, 19 Feb 2008, 03:05:37 EST