AChRδ Antibody (166) is a rat monoclonal IgG2a antibody that detects AChRδ in Torpedo, Rana, and Xenopus species through applications such as western blotting (WB), immunofluorescence (IF), and immunohistochemistry. AChRδ, also known as CMS2A, FCCMS, SCCMS, or CHRND, is a 517 amino acid multi-pass membrane protein that plays a crucial role in neuromuscular transmission by forming part of the nicotinic acetylcholine receptor complex. This receptor is essential for muscle contraction, as AChRδ mediates fast synaptic transmission at the neuromuscular junction by allowing cation influx upon acetylcholine binding. Proper functioning of AChRδ is vital, as mutations or dysfunctions are associated with severe neuromuscular disorders, including lethal type multiple pterygium syndrome and congenital myasthenic syndromes, both slow-channel and fast-channel types. Localization of AChRδ at the postsynaptic membrane ensures effective response to neurotransmitter release, facilitating rapid communication between nerve and muscle cells. Anti-AChRδ antibody (166) serves as an invaluable tool for researchers studying these critical pathways and the underlying mechanisms of neuromuscular diseases.
For Research Use Only. Not Intended for Diagnostic or Therapeutic Use.
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Nicotinic Acetylcholine Receptor delta/CHRND Antibody (166) References:
- Association of the gene encoding the delta-subunit of the muscle acetylcholine receptor (CHRND) with acquired autoimmune myasthenia gravis. | Giraud, M., et al. 2004. Genes Immun. 5: 80-3. PMID: 14735155
- CHRND mutation causes a congenital myasthenic syndrome by impairing co-clustering of the acetylcholine receptor with rapsyn. | Müller, JS., et al. 2006. Brain. 129: 2784-93. PMID: 16916845
- Mutation analysis of CHRNA1, CHRNB1, CHRND, and RAPSN genes in multiple pterygium syndrome/fetal akinesia patients. | Vogt, J., et al. 2008. Am J Hum Genet. 82: 222-7. PMID: 18179903
- Clinical phenotype and the lack of mutations in the CHRNG, CHRND, and CHRNA1 genes in two Indian families with Escobar syndrome. | Kodaganur, SG., et al. 2013. Clin Dysmorphol. 22: 54-58. PMID: 23448903
- Prenatal detection of uniparental disomy of chromosome 2 carrying a CHRND pathogenic variant that causes lethal multiple pterygium syndrome. | Shen, W., et al. 2018. Clin Genet. 93: 1248-1249. PMID: 29399782
- Severe congenital myasthenic syndrome associated with novel biallelic mutation of the CHRND gene. | Bonanno, C., et al. 2020. Neuromuscul Disord. 30: 336-339. PMID: 32360402
- Efficacy of salbutamol monotherapy in slow-channel congenital myasthenic syndrome caused by a novel mutation in CHRND. | Tawara, N., et al. 2021. Muscle Nerve. 63: E30-E32. PMID: 33428214
- Mutual stimulatory signaling between human myogenic cells and rat cerebellar neurons. | Tamáš, M., et al. 2021. Physiol Rep. 9: e15077. PMID: 34713978
- Case Report: Early diagnosis of lethal multiple pterygium syndrome with micrognathia: Two novel mutations in the CHRND gene. | Chen, C., et al. 2023. Front Genet. 14: 1005624. PMID: 36733345
- Transcriptome profile of subsynaptic myonuclei at the neuromuscular junction in embryogenesis. | Ohkawara, B., et al. 2024. J Neurochem. 168: 342-354. PMID: 37994470