The Very Large G Protein Coupled Receptor (Vlgr1) in Hair Cells

The very large G protein coupled receptor (Vlgr1) is a member of adhesion receptors or large N-terminal family B-7 transmembrane helixes (LNB7TM) receptors within the seven trans-membrane receptor superfamily. Vlgr1 is the largest GPCR identified to date; its mRNA spans 19 kb and encodes 6,300 amino...

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Bibliographic Details
Published in:Journal of molecular neuroscience 2013-05, Vol.50 (1), p.204-214
Main Authors: Sun, Jin-Peng, Li, Rong, Ren, Hong-Ze, Xu, An-Ting, Yu, Xiao, Xu, Zhi-Gang
Format: Article
Language:English
Subjects:
Adenosine
Amino Acid Sequence
Amino acids
Animals
Biomedical and Life Sciences
Biomedicine
Cell Biology
Cloning
Hair Cells, Auditory - metabolism
Hearing loss
Hearing protection
Humans
Ligands
Mice
Molecular Sequence Data
Mutation
Neurochemistry
Neurology
Neurosciences
Odors
Proteins
Proteomics
Receptors, G-Protein-Coupled - chemistry
Receptors, G-Protein-Coupled - genetics
Receptors, G-Protein-Coupled - metabolism
Signal transduction
Usher Syndromes - genetics
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Summary:The very large G protein coupled receptor (Vlgr1) is a member of adhesion receptors or large N-terminal family B-7 transmembrane helixes (LNB7TM) receptors within the seven trans-membrane receptor superfamily. Vlgr1 is the largest GPCR identified to date; its mRNA spans 19 kb and encodes 6,300 amino acids. Vlgr1 is a core component of ankle-link complex in inner ear hair cells. Knock-out and mutation mouse models show that loss of Vlgr1 function leads to abnormal stereociliary development and hearing loss, indicating crucial roles of Vlgr1 in hearing transduction or auditory system development. Over the past 10 or so years, human genetics data suggested that Vlgr1 mutations cause Usher syndromes and seizures. Although significant progresses have been made, the details of Vlgr1’s function in hair cells, its signaling cascade, and the mechanisms underlying causative effects of Vlgr1 mutations in human diseases remain elusive and ask for further investigation.
ISSN:0895-8696
1559-1166
DOI:10.1007/s12031-012-9911-5