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Not curated in GtoImmuPdb
Target id: 180
Nomenclature: CELSR3
Systematic Nomenclature: ADGRC3
Family: Adhesion Class GPCRs
Annotation status:
Annotated and reviewed, awaiting update
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Gene and Protein Information  |
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| Adhesion G protein-coupled receptor | ||||||
| Species | TM | AA | Chromosomal Location | Gene Symbol | Gene Name | Reference |
| Human | 7 | 3312 | 3p21.31 | CELSR3 | cadherin EGF LAG seven-pass G-type receptor 3 | 20 |
| Mouse | 7 | 3301 | 9 F2 | Celsr3 | cadherin, EGF LAG seven-pass G-type receptor 3 | 17 |
| Rat | 7 | 3313 | 8q32 | Celsr3 | cadherin, EGF LAG seven-pass G-type receptor 3 | 10 |
| Previous and Unofficial Names |
| ADGRC3 (adhesion G protein-coupled receptor C3) | MEGF2 | multiple epidermal growth factor-like domains 2 |
| Database Links |
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| Specialist databases | |
| GPCRdb | celr3_human (Hs), celr3_mouse (Mm), celr3_rat (Rn) |
| Other databases | |
| Alphafold | Q9NYQ7 (Hs), Q91ZI0 (Mm), O88278 (Rn) |
| Ensembl Gene | ENSG00000008300 (Hs), ENSMUSG00000023473 (Mm), ENSRNOG00000053889 (Rn) |
| Entrez Gene | 1951 (Hs), 107934 (Mm), 83466 (Rn) |
| Human Protein Atlas | ENSG00000008300 (Hs) |
| KEGG Gene | hsa:1951 (Hs), mmu:107934 (Mm), rno:83466 (Rn) |
| OMIM | 604264 (Hs) |
| Pharos | Q9NYQ7 (Hs) |
| RefSeq Nucleotide | NM_001407 (Hs), NM_080437 (Mm), NM_031320 (Rn) |
| RefSeq Protein | NP_001398 (Hs), NP_536685 (Mm), NP_112610 (Rn) |
| UniProtKB | Q9NYQ7 (Hs), Q91ZI0 (Mm), O88278 (Rn) |
| Wikipedia | CELSR3 (Hs) |
| Associated Protein Comments | ||
| Extracellular and transmembrane interactors: ADGRC3 [13], Frizzled-3 [15], dystroglycan [9]. |
| Agonist Comments | ||
| No ligands identified: orphan receptor. |
| Primary Transduction Mechanisms
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| Transducer | Effector/Response |
| Other - See Comments | |
| Comments: Principal transduction is potentially through Gαq/11 by calcium imaging [13]. | |
| References: | |
| Tissue Distribution
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| Expression Datasets |
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| Functional Assays
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| Physiological Functions
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| Physiological Consequences of Altering Gene Expression
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| Phenotypes, Alleles and Disease Models
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Mouse data from MGI | ||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||
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| Biologically Significant Variant Comments |
| Well characterised amino acid changing SNPs include rs12107252 (R1758Q), rs3821875 (T805S), and rs3733085 (P157A). |
| General Comments |
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CELSR3 (adherin, EGF LAG seven-pass G-type receptor 3) is a receptor that belongs to Family IV Adhesion-GPCRs along with CELSR1 and CELSR2 [8]. The gene is localized on human chromosome 3 and mouse chromosome 9. Family IV Adhesion-GPCRs have orthologs in vertebrate and invertebrate species. A recent publication reveals an increase in Celsr3 expression in pancreatic tumour stellate cells [4]. Activation of these cells or their trans-differentiation is regulated by paracrine and autocrine loops of growth factors which are associated with pathological conditions such as liver injury, cirrhosis as well as cancer. |
1. Beall SA, Boekelheide K, Johnson KJ. (2005) Hybrid GPCR/cadherin (Celsr) proteins in rat testis are expressed with cell type specificity and exhibit differential Sertoli cell-germ cell adhesion activity. J Androl, 26 (4): 529-38. [PMID:15955893]
2. Bjarnadóttir TK, Geirardsdóttir K, Ingemansson M, Mirza MA, Fredriksson R, Schiöth HB. (2007) Identification of novel splice variants of Adhesion G protein-coupled receptors. Gene, 387 (1-2): 38-48. [PMID:17056209]
3. Cortijo C, Gouzi M, Tissir F, Grapin-Botton A. (2012) Planar cell polarity controls pancreatic Beta cell differentiation and glucose homeostasis. Cell Rep, 2 (6): 1593-606. [PMID:23177622]
4. Erkan M, Weis N, Pan Z, Schwager C, Samkharadze T, Jiang X, Wirkner U, Giese NA, Ansorge W, Debus J, Huber PE, Friess H, Abdollahi A, Kleeff J. (2010) Organ-, inflammation- and cancer specific transcriptional fingerprints of pancreatic and hepatic stellate cells. Mol Cancer, 9: 88. [PMID:20416094]
5. Feng J, Xu Y, Wang M, Ruan Y, So KF, Tissir F, Goffinet A, Zhou L. (2012) A role for atypical cadherin Celsr3 in hippocampal maturation and connectivity. J Neurosci, 32 (40): 13729-43. [PMID:23035085]
6. Fenstermaker AG, Prasad AA, Bechara A, Adolfs Y, Tissir F, Goffinet A, Zou Y, Pasterkamp RJ. (2010) Wnt/planar cell polarity signaling controls the anterior-posterior organization of monoaminergic axons in the brainstem. J Neurosci, 30 (47): 16053-64. [PMID:21106844]
7. Formstone CJ, Little PF. (2001) The flamingo-related mouse Celsr family (Celsr1-3) genes exhibit distinct patterns of expression during embryonic development. Mech Dev, 109: 91-94. [PMID:11677057]
8. Fredriksson R, Gloriam DE, Höglund PJ, Lagerström MC, Schiöth HB. (2003) There exist at least 30 human G-protein-coupled receptors with long Ser/Thr-rich N-termini. Biochem Biophys Res Commun, 301 (3): 725-34. [PMID:12565841]
9. Lindenmaier LB, Parmentier N, Guo C, Tissir F, Wright KM. (2019) Dystroglycan is a scaffold for extracellular axon guidance decisions. Elife, 8. [PMID:30758284]
10. Nakayama M, Nakajima D, Nagase T, Nomura N, Seki N, Ohara O. (1998) Identification of high-molecular-weight proteins with multiple EGF-like motifs by motif-trap screening. Genomics, 51: 27-34. [PMID:9693030]
11. Qu Y, Glasco DM, Zhou L, Sawant A, Ravni A, Fritzsch B, Damrau C, Murdoch JN, Evans S, Pfaff SL, Formstone C, Goffinet AM, Chandrasekhar A, Tissir F. (2010) Atypical cadherins Celsr1-3 differentially regulate migration of facial branchiomotor neurons in mice. J Neurosci, 30 (28): 9392-401. [PMID:20631168]
12. Shima Y, Copeland NG, Gilbert DJ, Jenkins NA, Chisaka O, Takeichi M, Uemura T. (2002) Differential expression of the seven-pass transmembrane cadherin genes Celsr1-3 and distribution of the Celsr2 protein during mouse development. Dev Dyn, 223 (3): 321-32. [PMID:11891983]
13. Shima Y, Kawaguchi SY, Kosaka K, Nakayama M, Hoshino M, Nabeshima Y, Hirano T, Uemura T. (2007) Opposing roles in neurite growth control by two seven-pass transmembrane cadherins. Nat Neurosci, 10 (8): 963-9. [PMID:17618280]
14. Shima Y, Kengaku M, Hirano T, Takeichi M, Uemura T. (2004) Regulation of dendritic maintenance and growth by a mammalian 7-pass transmembrane cadherin. Dev Cell, 7 (2): 205-16. [PMID:15296717]
15. Thakar S, Wang L, Yu T, Ye M, Onishi K, Scott J, Qi J, Fernandes C, Han X, Yates 3rd JR et al.. (2017) Evidence for opposing roles of Celsr3 and Vangl2 in glutamatergic synapse formation. Proc Natl Acad Sci USA, 114 (4): E610-E618. [PMID:28057866]
16. Tissir F, Bar I, Jossin Y, De Backer O, Goffinet AM. (2005) Protocadherin Celsr3 is crucial in axonal tract development. Nat Neurosci, 8 (4): 451-7. [PMID:15778712]
17. Tissir F, De-Backer O, Goffinet AM, Lambert de Rouvroit C. (2002) Developmental expression profiles of Celsr (Flamingo) genes in the mouse. Mech Dev, 112 (1-2): 157-60. [PMID:11850187]
18. Tissir F, Goffinet AM. (2006) Expression of planar cell polarity genes during development of the mouse CNS. Eur J Neurosci, 23 (3): 597-607. [PMID:16487141]
19. Tissir F, Qu Y, Montcouquiol M, Zhou L, Komatsu K, Shi D, Fujimori T, Labeau J, Tyteca D, Courtoy P, Poumay Y, Uemura T, Goffinet AM. (2010) Lack of cadherins Celsr2 and Celsr3 impairs ependymal ciliogenesis, leading to fatal hydrocephalus. Nat Neurosci, 13 (6): 700-7. [PMID:20473291]
20. Wu Q, Maniatis T. (2000) Large exons encoding multiple ectodomains are a characteristic feature of protocadherin genes. Proc Natl Acad Sci USA, 97 (7): 3124-9. [PMID:10716726]
21. Ying G, Wu S, Hou R, Huang W, Capecchi MR, Wu Q. (2009) The protocadherin gene Celsr3 is required for interneuron migration in the mouse forebrain. Mol Cell Biol, 29 (11): 3045-61. [PMID:19332558]
22. Zhou L, Bar I, Achouri Y, Campbell K, De Backer O, Hebert JM, Jones K, Kessaris N, de Rouvroit CL, O'Leary D, Richardson WD, Goffinet AM, Tissir F. (2008) Early forebrain wiring: genetic dissection using conditional Celsr3 mutant mice. Science, 320 (5878): 946-9. [PMID:18487195]
23. Zhou L, Qu Y, Tissir F, Goffinet AM. (2009) Role of the atypical cadherin Celsr3 during development of the internal capsule. Cereb Cortex, 19 Suppl 1: i114-9. [PMID:19349379]
