ArticleMolecular and cellular biology2007
Targeting of the ETS factor GABPalpha disrupts neuromuscular junction synaptic function.
Article in Molecular and cellular biology, 2007. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 15 papers.
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Who cites it
15 citing papers in PubMed, 31 citations in OpenAlex.
- NMJ-related diseases beyond the congenital myasthenic syndromes.Frontiers in cell and developmental biology · 2023Review
- MiR-142a-3p: A novel ACh receptor transcriptional regulator in association with peripheral nerve injury.Molecular therapy. Nucleic acids · 2022Article
- STAT3 Is an Upstream Regulator ofInternational journal of molecular sciences · 2021Article
- Adrenergic-Independent Signaling via CHRNA2 Regulates Beige Fat Activation.Developmental cell · 2020Article
- GA binding protein augments autophagy via transcriptional activation of BECN1-PIK3C3 complex genes.Autophagy · 2014Article
- Regulatory elements associated with paternally-expressed genes in the imprinted murine Angelman/Prader-Willi syndrome domain.PloS one · 2013Article
- Understanding the role of ETS-mediated gene regulation in complex biological processes.Advances in cancer research · 2013Review
- Molecular mechanisms for age-associated mitochondrial deficiency in skeletal muscle.Journal of aging research · 2012Article
- Critical requirement of GABPalpha for normal T cell development.The Journal of biological chemistry · 2010Article
- Ets transcription factors control epithelial maturation and transit and crypt-villus morphogenesis in the mammalian intestine.The American journal of pathology · 2009Article
- Ets transcription factors in intestinal morphogenesis, homeostasis and disease.Histology and histopathology · 2008Review
- GABPbeta2 is dispensible for normal lymphocyte development but moderately affects B cell responses.The Journal of biological chemistry · 2008Article
- Targeting the GA binding protein beta1L isoform does not perturb lymphocyte development and function.Molecular and cellular biology · 2008Article
- Identification and structural characterization of a CBP/p300-binding domain from the ETS family transcription factor GABP alpha.Journal of molecular biology · 2008Article
- GA-binding protein is dispensable for neuromuscular synapse formation and synapse-specific gene expression.Molecular and cellular biology · 2007Article
Corrections and comments
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Authors and funding
6 authors at 3 institutions in 1 country.
Funding
No grant is acknowledged in the PubMed record.
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 epsilon 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 epsilon 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 GABPalpha 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.
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