Introduction of CELSR3
CELSR3, encoded by CELSR3 gene, is a member of the flamingo subfamily, one of the cadherin superfamily. The flamingo cadherins belong to nonclassic-type cadherins that do not interact with catenins. The ectodomain of flamingo cadherin contains seven epidermal growth factor-like repeats, nine cadherin domains and two laminin A G-type repeats. Besides, it also has seven transmembrane domains which are characteristic feature of their subfamily.
|Basic Information of CELSR3|
|Protein Name||Cadherin EGF LAG seven-pass G-type receptor 3|
|Aliases||FMI1, EGFL1, HFMI1, MEGF2, ADGRC3, CDHF11, RESDA1|
|Organism||Homo sapiens (Human)|
Function of CELSR3 Membrane Protein
CELSR3 may function as a regulator of nervous system development. It can regulate the contact-dependent neurite growth and ependymal ciliogenesis through the planar cell polarity (PCP) signaling. The deficiency of CELSR3 and CELSR1 may significantly suppress the ependyma ciliogenesis and lead to lethal hydrocephalus. Moreover, CELSR3 together with Fzd3 participate in the regulation of the development of most longitudinal tracts in the central nervous system. And they also mediate axon guidance in the peripheral, sympathetic and enteric nervous systems. The study showed that Celsr3 and Fzd3 regulate the formation of a scaffold of pioneer neurons and their axons which control growing thalamocortical axons. In addition, CELSR3 may involve in tumor formation. The studies have been shown that CELSR3 moter hypermethylation is associated with the progress of oral squamous cell carcinoma. And CELSR3 has selectively up-regulated in tumor stellate cells.
Fig.1 G protein-coupled receptors.
Application of CELSR3 Membrane Protein in Literature
1. Chen Z., et al. Genetic analysis of Wnt/PCP genes in neural tube defects. Bmc Medical Genomics. 2018, 11(1): 38. PMID: 29618362
The study is designed to analyze Wnt/PCP genes in Chinese and Caucasian neural tube defects (NTD). The result shows that CELSR1, CELSR2, and CELSR3 genes were significantly clustered along with rare driver coding mutations. And compound heterozygote variants of CELSR2 p.Thr2026Met and DVL3 p.Asp403Asn could down-regulate PCP signals.
2. Wang F., et al. The role of Celsr3 in the development of central somatosensory projections from dorsal root ganglia. Neuroscience. 2017, 359: 267-76. PMID: 28754314
The study is to explore the function of CELSR3 in dorsal root ganglion (DRG) neurons using conditional gene inactivation in crosses with Wnt1-Cre mice. The result shows that CELSR3 is not a necessary factor for the patterning of central DRG projections, but it plays an important role in the mediation of central somatosensory projections development from dorsal root ganglia.
3. Khor G H., et al. Involvement of CELSR3 Hypermethylation in Primary Oral Squamous Cell Carcinoma. Asian Pacific Journal of Cancer Prevention Apjcp. 2016, 17(1): 219-23. PMID: 26838213
The study shows that a high methylation level of CELSR3 is detected in 86% of oral squamous cell carcinomas patients. And CELSR3 quantitative methylation levels are markedly associated with patient ethnicity.
4. Su L., et al. Deregulation of the planar cell polarity genes CELSR3 and FZD3 in Hirschsprung disease. Experimental & Molecular Pathology. 2016, 101(2): 241-8. PMID: 27619161
Su’s study reveals that the down-regulated expression of CELSR3 and FZD3, the planar cell polarity genes may obstruct the enteric innervation pattern, consequently resulting in the risk of Hirschsprung disease (HSCR).
5. Karpathakis A., et al. Prognostic Impact of Novel Molecular Subtypes of Small Intestinal Neuroendocrine Tumor. Clinical Cancer Research. 2016, 22(1): 250-8. PMID: 26169971
The study is the first comprehensive integrated molecular analysis of small intestinal neuroendocrine tumors (SINETs). Results show that SINETs are highly epigenetically dysregulated. And 21 epigenetically dysregulated genes were identified, including CDX1 (86%), CELSR3 (84%), FBP1 (84%), and GIPR (74%).
CELSR3 Preparation Options
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