Affinage

CELSR1

Cadherin EGF LAG seven-pass G-type receptor 1 · UniProt Q9NYQ6

Length
3014 aa
Mass
329.5 kDa
Annotated
2026-06-09
52 papers in source corpus 23 papers cited in narrative 23 extracted findings
Cross-family judge vs UniProt: Affinage preferred faithfulness: 8/8 claims corpus-supported (100%)

Mechanistic narrative

Synthesis pass · prose summary of the discoveries below

CELSR1 is the core mammalian planar cell polarity (PCP) organizer, the first vertebrate member of the flamingo/starry night pathway, required to coordinate cell orientation across diverse epithelia and to initiate neural tube closure (PMID:12842012). It is a cleavage-deficient adhesion GPCR whose large cadherin ectodomain mediates homophilic adhesion: cryo-EM and crystallographic structures of the extracellular region show 14 domains in which an N-terminal CADH1-8 segment drives cell-cell adhesion while a C-terminal CADH9-GAIN compact module regulates adhesion and signaling, with Ca2+-dependent dimerization through the cadherin repeats (PMID:40295529, PMID:38307021). CELSR1 forms dense, highly stable punctate junctional assemblies through both trans- and cis-interactions; the PCP-mutant Celsr1Crsh selectively loses lateral cis-interactions, becomes mobile, and fails to organize Frizzled6 and Vangl2 into asymmetric junctional complexes, a defect rescuable by ectopic cis-dimerization (PMID:33529151). This stable junctional enrichment, rather than partner binding per se, distinguishes CELSR1 from CELSR2, since CELSR1 alone establishes epidermal PCP asymmetry despite both family members interacting equivalently with Vangl2 and Fz6 (PMID:36712970), and it recruits VANGL2 to cell-cell contacts (PMID:24632739). Through this activity CELSR1 directs collective polarity in skin, lung, oviduct, inner ear, and lymphatic valve tissues, acting upstream of Rho kinase in lung branching (PMID:20223754), coordinating intercellular basal body orientation in multiciliated cells (PMID:33468623), and limiting VE-cadherin/adherens junction maturation to permit dynamic endothelial movements during valve formation (PMID:23792146). CELSR1 also functions non-cell-autonomously in facial branchiomotor neuron migration, where neuroepithelial CELSR1 suppresses Wnt5a-mediated rostral chemoattraction to enforce caudal migration (PMID:27395006, PMID:36325991). Biochemically, CELSR1 couples to GαS through a cleavage- and tethered-agonist-independent mechanism, since it lacks GAIN autoproteolysis yet tethered-agonist point mutants retain GαS coupling (PMID:37224017). Human and mouse CELSR1 variants cause craniorachischisis and neural tube defects by disrupting plasma-membrane trafficking, junctional localization, VANGL2 recruitment, and PCP signaling (PMID:22095531, PMID:24632739, PMID:38272662).

Mechanistic history

Synthesis pass · year-by-year structured walk · 12 steps
  1. 2003 High

    Established CELSR1 as the founding mammalian PCP-pathway gene by linking its mutation to the two diagnostic readouts of disrupted planar polarity and failed neural tube closure.

    Evidence ENU mutagenesis and phenotypic analysis of spin cycle and crash mouse alleles

    PMID:12842012

    Open questions at the time
    • Molecular basis of how the missense alleles disrupt protein function not defined
    • Direct biochemical activity unaddressed
  2. 2010 Medium

    Extended CELSR1 PCP function to branching morphogenesis and to non-cell-autonomous control of directional neuron migration, broadening its role beyond epithelial polarity.

    Evidence Crash-allele and conditional mouse analysis, Rho kinase inhibition and FGF10 ex vivo branching assays (lung); conditional inactivation and genetic epistasis with Celsr2 (FBM neurons); immunofluorescence in neural tube

    PMID:20223754 PMID:20353824 PMID:20631168

    Open questions at the time
    • Molecular link between CELSR1 and Rho kinase not biochemically resolved
    • Identity of the suppressed migratory signal not yet known
    • Functional consequence of basal neuroepithelial enrichment not established
  3. 2011 Medium

    Identified loss of plasma-membrane trafficking as the cellular defect underlying human craniorachischisis-associated CELSR1 variants, separating localization from protein-interaction capacity.

    Evidence Subcellular localization and protein-interaction assays of human CRN patient variants, compared to crash/spin cycle mutants

    PMID:22095531

    Open questions at the time
    • Single lab
    • How trafficking failure translates to PCP loss in vivo not shown
  4. 2013 High

    Revealed a junctional-dynamics function: CELSR1 restrains VE-cadherin/adherens junction maturation to enable the endothelial cell rearrangements of lymphatic valve formation.

    Evidence Celsr1-deficient mouse analysis, live imaging, and localization of Celsr1 and VE-cadherin

    PMID:23792146

    Open questions at the time
    • Direct biochemical link between CELSR1 and VE-cadherin not defined
    • Signaling effector unknown
  5. 2014 Medium

    Tied CELSR1 asymmetric boundary localization to recruitment of VANGL2 and to organ-scale tissue polarity, and showed patient variants impair the CELSR1-VANGL2 interaction.

    Evidence Celsr1-deficient mouse with mosaic analysis (oviduct); Co-IP and localization assays of spina bifida TG-repeat variants with VANGL2

    PMID:24632739 PMID:25406397

    Open questions at the time
    • Co-IP without reciprocal validation
    • Whether VANGL2 recruitment is direct not resolved
  6. 2016 High

    Localized the non-cell-autonomous migratory control to CELSR1 in the rhombomere neuroepithelium and identified Dvl3 as a downstream effector of CELSR1-driven endothelial migration.

    Evidence Cell-type-specific conditional inactivation with dye-fill tracing (FBM neurons); CELSR1 gain/loss-of-function with migration and tube-formation assays in endothelial cells

    PMID:27301287 PMID:27395006

    Open questions at the time
    • CELSR1-Dvl3 interaction not confirmed by Co-IP
    • Identity of the suppressed neuroepithelial signal still unknown at this stage
  7. 2017 Medium

    Connected CELSR1 to progenitor-fate control and sensory-bundle orientation, linking its polarity activity to retinoic-acid-dependent neurogenesis and stereocilia alignment.

    Evidence Celsr1 loss-of-function mouse analysis with RA transcriptional assays and progenitor quantification (cortex); KO analysis with immunofluorescence and behavior (vestibular epithelia)

    PMID:28159525 PMID:29257130

    Open questions at the time
    • Single lab for each context
    • Mechanism coupling endfoot branching to RA signaling not defined
  8. 2021 High

    Defined the molecular mechanism of CELSR1 PCP organization: stable cis/trans junctional assemblies are required to cluster Frizzled6 and Vangl2 asymmetrically, with the Crash mutation acting through loss of cis-interaction.

    Evidence Biochemistry, super-resolution microscopy, FRAP, Co-IP with Fzd6/Vangl2, and ectopic cis-dimerization rescue (epidermis/junctions); double-mutant epistasis with Camsap3 in oviduct ciliated cells

    PMID:33468623 PMID:33529151

    Open questions at the time
    • Structural basis of cis vs trans contacts not resolved at this point
    • How junctional clustering couples to downstream signaling unaddressed
  9. 2022 High

    Identified the suppressed migratory signal as Wnt5a, establishing that CELSR1 enforces caudal neuron migration by blocking Wnt5a-mediated rostral chemoattraction.

    Evidence Celsr1;Wnt5a double mutants, Wnt5a bead implantation in hindbrain explants, and Wnt5a overexpression in Celsr1 mutant background

    PMID:36325991

    Open questions at the time
    • Molecular mechanism by which CELSR1 suppresses Wnt5a signaling not defined
    • Receptor mediating Wnt5a chemoattraction not identified
  10. 2023 High

    Resolved the signaling mode and family specialization of CELSR1: it is a cleavage-deficient adhesion GPCR coupling to GαS independent of the tethered-agonist paradigm, and is the dominant Celsr driving epidermal PCP via stable junctional enrichment.

    Evidence G protein coupling, autoproteolysis, and tethered-agonist mutant assays; reciprocal Celsr1/Celsr2 CRISPR KO with FRAP, junctional enrichment, and Co-IP

    PMID:36712970 PMID:37224017

    Open questions at the time
    • Physiological ligand/activation trigger of GαS coupling unknown
    • How GαS signaling integrates with PCP organization not established
  11. 2024 High

    Provided atomic-resolution structures of the cadherin ectodomain, assigning adhesion to N-terminal repeats and regulatory function to the CADH9-GAIN module, and confirmed pathogenicity of a de novo missense variant through PCP and junction assays.

    Evidence X-ray crystallography of EC1-4 and EC4-7 with bead aggregation and MD simulations; in vitro localization, junction, PCP, and proliferation assays of p.(Cys1318Tyr)

    PMID:38272662 PMID:38307021

    Open questions at the time
    • Crystallography indicates only weak homophilic adhesion by isolated EC repeats
    • Full-length receptor architecture not captured by fragment structures
  12. 2025 High

    Delivered a near-complete cryo-EM model of the CELSR1 extracellular region, showing a compact 14-domain module formed by CADH9-GAIN contacts and Ca2+-dependent cadherin-mediated dimerization, unifying adhesion and signaling roles structurally.

    Evidence 3.8 Å cryo-EM reconstruction with cell-based adhesion and domain-deletion assays

    PMID:40295529

    Open questions at the time
    • Transmembrane/intracellular regions and GAIN-GPCR coupling not structurally resolved
    • Trans-dimer interface geometry remains putative

Open questions

Synthesis pass · forward-looking unresolved questions
  • How GAIN/GPCR-mediated GαS coupling is physiologically activated and mechanistically integrated with cadherin-based junctional clustering to drive asymmetric PCP complex assembly remains unresolved.
  • No identified activating ligand or trigger for GαS coupling
  • No structure of the full-length receptor or its intracellular signaling output
  • Mechanistic bridge between adhesion, G protein signaling, and Vangl2/Fzd6 clustering not established

Mechanism profile

Synthesis pass · controlled-vocabulary classification · explore literature graph →
Molecular activity
GO:0060090 molecular adaptor activity 2 GO:0098631 cell adhesion mediator activity 2 GO:0060089 molecular transducer activity 1
Localization
GO:0005886 plasma membrane 3
Pathway
R-HSA-1266738 Developmental Biology 3 R-HSA-1500931 Cell-Cell communication 3 R-HSA-162582 Signal Transduction 3
Partners
CDH5DVL3FZD6VANGL2WNT5A
Complex memberships
CELSR1-Frizzled6-Vangl2 asymmetric junctional PCP complex

Evidence

Reading pass · 23 per-paper findings extracted from the source corpus
Year Finding Method Journal Conf PMIDs
2003 Missense mutations in Celsr1 (spin cycle and crash alleles) disrupt planar cell polarity of inner ear hair cells and cause severe neural tube defects due to failure to initiate neural tube closure in mice, establishing Celsr1 as the first mammalian member of the flamingo/starry night PCP pathway. ENU mutagenesis, missense mutation identification, phenotypic analysis of heterozygous and homozygous mutants Current biology : CB High 12842012
2011 Missense variants in human CELSR1 associated with craniorachischisis abolish or diminish trafficking of CELSR1 protein to the plasma membrane, without affecting protein-protein interactions; comparable membrane trafficking defects were seen in crash and spin cycle mouse Celsr1 mutants. Subcellular protein localization assays, protein-protein interaction assays, sequencing of human CRN patients Human mutation Medium 22095531
2010 Celsr1 (Crash allele) is required for normal lung branching morphogenesis; mutant lungs show fewer branches, thickened mesenchyme, disrupted epithelial integrity, perturbed cytoskeletal remodelling, and failure of mutant endoderm to branch in response to FGF10; this phenotype is recapitulated by Rho kinase inhibition, placing Celsr1 upstream of Rho kinase in lung PCP signaling. Mouse mutant analysis (Celsr1Crsh), Rho kinase inhibitor treatment, ex vivo branching assay with FGF10 Human molecular genetics Medium 20223754
2013 Celsr1 is recruited from endothelial filopodia to discrete membrane domains at cell-cell contacts during lymphatic valve morphogenesis and regulates dynamic endothelial cell movements by inhibiting stabilization of VE-cadherin and maturation of adherens junctions. Celsr1-deficient mouse analysis, live imaging of endothelial cell behaviors, localization studies of Celsr1 and VE-cadherin Developmental cell High 23792146
2010 Celsr1 functions non-cell-autonomously in facial branchiomotor (FBM) neuron migration: Celsr1 is expressed in FBM neuron precursors and the floor plate (not in migrating FBM neurons themselves), and its loss causes neurons to migrate rostrally instead of caudally. Celsr1 is epistatic to Celsr2 in this context. Knock-out and Crash allele mouse analysis, conditional inactivation, genetic epistasis (Celsr2 epistatic to Celsr1) The Journal of neuroscience : the official journal of the Society for Neuroscience High 20631168
2014 Celsr1 is required for multilevel polarity in the mouse oviduct: it is concentrated at specific cellular boundaries perpendicular to the ovary-uterus axis; its loss randomizes ciliary beat orientation, cell elongation/orientation, and epithelial fold directionality. Mosaic analysis shows that Celsr1 primarily regulates epithelial cell geometry, which secondarily aligns epithelial folds. Celsr1-deficient mouse analysis, mosaic analysis, immunofluorescence localization, ciliary motion assays Development (Cambridge, England) High 25406397
2014 Two TG dinucleotide repeat variants in CELSR1 found in spina bifida patients change its subcellular localization and impair its physical association with VANGL2, diminishing the ability of CELSR1 to recruit VANGL2 to cell-cell contacts. In vitro subcellular localization assay, co-immunoprecipitation (physical interaction assay between CELSR1 and VANGL2) PloS one Medium 24632739
2016 Celsr1 functions non-cell-autonomously in FBM neuron migration: conditional inactivation specifically in the ventricular zone of rhombomeres r3-r5 (not in the floor plate) causes rostral migration of FBM neurons, indicating that Celsr1 in the adjacent neuroepithelium suppresses signals that could otherwise attract FBM neurons rostrally. Conditional (Cre-lox) inactivation of Celsr1 in specific cell types, dye fill experiments to trace neuron origins Developmental biology High 27395006
2019 The CELSR1 missense mutation P870L (c.2609G>A) is a gain-of-function mutation that upregulates both the PCP pathway and canonical WNT signaling in cells, and induces both neural tube defects and congenital heart defects in zebrafish embryos. In vitro cell-based assay for PCP and WNT pathway activity, zebrafish in vivo injection assay Clinical science (London, England : 1979) Medium 27756857
2021 Mouse Celsr1 engages in both trans- and cis-interactions, forming dense and highly stable punctate assemblies at junctions. The PCP-mutant variant Celsr1Crsh selectively impairs lateral cis-interactions, displays increased mobility, fails to engage in homophilic adhesion with wild-type protein, and consequently fails to organize Frizzled6 and Vangl2 into asymmetric junctional complexes, a defect rescuable by ectopic cis-dimerization. Biochemical assays, super-resolution microscopy, FRAP, co-immunoprecipitation with Fzd6 and Vangl2, ectopic cis-dimerization rescue experiment eLife High 33529151
2021 In mouse oviduct multi-ciliated cells, CELSR1 is required for intercellular coordination of basal body (BB) orientation, while CAMSAP3 (a microtubule minus-end regulator) controls intracellular BB orientation; CELSR1 loss disrupts intercellular but not intracellular polarity coordination, and does not affect CAMSAP3 localization. Celsr1-deficient and Camsap3-mutant mouse analysis, immunofluorescence, genetic epistasis (double mutant analysis) Journal of cell science High 33468623
2017 Celsr1 controls branching of apical neural progenitor cell (aNPC) basal processes abutting the meninges and thereby regulates retinoic acid (RA)-dependent neurogenesis. Loss of Celsr1 decreases endfeet number, modifies RA-dependent transcriptional activity, and biases aNPC commitment toward self-renewal at the expense of basal progenitor and neuron production, resulting in cortical hypoplasia. Celsr1 loss-of-function mouse analysis, RA transcriptional activity assay, progenitor fate quantification Molecular psychiatry Medium 29257130
2017 Celsr1 is asymmetrically distributed at cell boundaries between hair cells and neighboring supporting cells in developing vestibular and auditory sensory epithelia; loss of Celsr1 results in misoriented stereociliary bundles of vestibular hair cells, particularly in the cristae of the semicircular canals. Celsr1 knockout mouse analysis, immunofluorescence localization, behavioral analysis (circling) Developmental biology Medium 28159525
2023 CELSR1 and CELSR3 are cleavage-deficient (lack autoproteolytic cleavage at the GAIN domain), while CELSR2 is efficiently cleaved. Despite this, CELSR1-3 all engage GαS. CELSR1 tethered agonist (TA) point mutants retain GαS coupling activity, indicating CELSR1 signals via a cleavage-independent mechanism distinct from the canonical tethered-agonist paradigm. G protein coupling assays, autoproteolysis assays, tethered agonist point mutant analysis Cell reports High 37224017
2024 CELSR1 tethered agonist (TA) point mutants retain GαS coupling activity; CELSR1 and CELSR3 are cleavage-deficient, while CELSR2 autoproteolysis enhances GαS coupling, yet acute TA exposure alone is insufficient for full activation. G protein coupling assays, autoproteolysis assays, tethered agonist point mutant analysis bioRxiv : the preprint server for biologypreprint Medium 37066404
2025 Cryo-EM reconstruction of the mouse CELSR1 extracellular region (ECR) at 3.8 Å reveals 14 domains forming a compact module via conserved interactions between the CADH9 and C-terminal GAIN domains. In the presence of Ca2+, the CELSR1 ECR forms a dimer species via cadherin repeats putatively in antiparallel fashion. Cell-based assays show the N-terminal CADH1-8 repeat is required for cell-cell adhesion, while the C-terminal CADH9-GAIN compact module regulates cellular adhesion/signaling. Cryo-EM structure determination (3.8 Å), cell-based adhesion assays, domain deletion analysis Nature communications High 40295529
2024 Crystal structures of human CELSR1 EC1-4 and EC4-7 reveal typical cadherin folds with a non-canonical linker between EC5 and EC6. EC1-4 only dimerizes at high concentration in solution; EC7-MAD10 mediates dimerization; simulations and experiments indicate flexibility at EC5-6. Cell-based bead aggregation assays do not support strong adhesion by EC repeats alone, suggesting weak homophilic adhesion by CELSR1 cadherin repeats. X-ray crystallography (EC1-4 and EC4-7 crystal structures), bead aggregation assays, solution dimerization assays, molecular dynamics simulations Structure (London, England : 1993) High 38307021
2023 Celsr1 is the major Celsr family member driving epidermal planar cell polarity: removal of Celsr1 alone abolishes PCP protein asymmetry and hair follicle polarization, whereas loss of Celsr2 alone has no effect on epidermal PCP. FRAP assays show Celsr1 stably enriches at junctional interfaces while Celsr2 is much less efficiently recruited; both interact equivalently with Vangl2 and Fz6. CRISPR/Cas9 knockout of Celsr1 and Celsr2, FRAP, junctional enrichment assay, co-immunoprecipitation with Vangl2 and Fz6 Frontiers in cell and developmental biology High 36712970
2022 Celsr1 suppresses Wnt5a-mediated chemoattraction to direct caudal facial branchiomotor (FBM) neuron migration: in Celsr1;Wnt5a double mutants, FBM neurons never migrate rostrally; Wnt5a-coated beads attract FBM neurons rostrally in wild-type explants; and overexpression of Wnt5a in r3 of Celsr1 mutants greatly enhances rostral migration, establishing a genetic epistasis mechanism. Genetic epistasis (double mutant Celsr1;Wnt5a), bead implantation assay in hindbrain explants, Wnt5a overexpression in Celsr1 mutant background Development (Cambridge, England) High 36325991
2010 Celsr1 protein is enriched at the basal surface of neuroepithelial cells within the early neural tube and in ventricular zone cells at the spinal cord midline, and this basal enrichment is lost in Celsr1 homozygous mutant embryos; this basal localization is spatiotemporally associated with dorsal sensory tract morphogenesis. Immunofluorescence localization in wild-type and Celsr1 mutant embryos, comparative analysis of protein distribution Molecular and cellular neurosciences Medium 20353824
2020 Knockdown of Celsr1 in the subventricular zone (SVZ) after cerebral ischemia reduces neuroblast proliferation, CD31-positive cell number, and motor function, and increases apoptosis and infarct volume; these effects are associated with downregulation of p-PKC, placing Celsr1 upstream of Wnt/PKC signaling in post-ischemic neurogenesis and angiogenesis. Lentiviral Celsr1 knockdown in MCAO rat model, immunohistochemistry, functional behavioral assays International journal of molecular sciences Medium 32070035
2016 CELSR1 promotes endothelial cell migration and tube formation through Dishevelled segment polarity protein 3 (Dvl3); gain- and loss-of-function of CELSR1 in human aortic endothelial cells bidirectionally regulate migration and angiogenic tube formation. TALE-VP64-mediated CELSR1 overexpression, shRNA knockdown, scratch/transwell migration assay, tube formation assay Biochemistry. Biokhimiia Medium 27301287
2024 De novo heterozygous CELSR1 missense variant p.(Cys1318Tyr) disrupts subcellular localization of CELSR1, affects cell-cell junctions, impairs planar cell polarity signaling, and lowers proliferation rate in vitro. In vitro subcellular localization assays, cell-cell junction assay, PCP signaling assay, proliferation assay Journal of medical genetics Medium 38272662

Source papers

Stage 0 corpus · 52 papers · ranked by NIH iCite citations
Year Title Journal Citations PMID
2003 Mutation of Celsr1 disrupts planar polarity of inner ear hair cells and causes severe neural tube defects in the mouse. Current biology : CB 500 12842012
2011 Mutations in the planar cell polarity genes CELSR1 and SCRIB are associated with the severe neural tube defect craniorachischisis. Human mutation 157 22095531
2010 The PCP genes Celsr1 and Vangl2 are required for normal lung branching morphogenesis. Human molecular genetics 129 20223754
2013 Planar cell polarity protein Celsr1 regulates endothelial adherens junctions and directed cell rearrangements during valve morphogenesis. Developmental cell 118 23792146
2010 Atypical cadherins Celsr1-3 differentially regulate migration of facial branchiomotor neurons in mice. The Journal of neuroscience : the official journal of the Society for Neuroscience 97 20631168
2014 Celsr1 is required for the generation of polarity at multiple levels of the mouse oviduct. Development (Cambridge, England) 83 25406397
2012 Role of the planar cell polarity gene CELSR1 in neural tube defects and caudal agenesis. Birth defects research. Part A, Clinical and molecular teratology 82 22371354
2002 Differential expression of the seven-pass transmembrane cadherin genes Celsr1-3 and distribution of the Celsr2 protein during mouse development. Developmental dynamics : an official publication of the American Association of Anatomists 79 11891983
2009 Identification of CELSR1 as a susceptibility gene for ischemic stroke in Japanese individuals by a genome-wide association study. Atherosclerosis 77 19403135
2014 Identification of novel CELSR1 mutations in spina bifida. PloS one 72 24632739
2001 The flamingo-related mouse Celsr family (Celsr1-3) genes exhibit distinct patterns of expression during embryonic development. Mechanisms of development 70 11677057
1997 Celsr1, a neural-specific gene encoding an unusual seven-pass transmembrane receptor, maps to mouse chromosome 15 and human chromosome 22qter. Genomics 61 9339365
2017 Seven pass Cadherins CELSR1-3. Seminars in cell & developmental biology 53 28716607
2016 A novel mutation in CELSR1 is associated with hereditary lymphedema. Vascular cell 48 26855770
2012 Celsr1-3 cadherins in PCP and brain development. Current topics in developmental biology 45 23140629
2017 Celsr1 coordinates the planar polarity of vestibular hair cells during inner ear development. Developmental biology 42 28159525
2016 Planar cell polarity genes Celsr1 and Vangl2 are necessary for kidney growth, differentiation, and rostrocaudal patterning. Kidney international 41 27597235
2021 Celsr1 adhesive interactions mediate the asymmetric organization of planar polarity complexes. eLife 40 33529151
2016 Genetic analysis of rare coding mutations of CELSR1-3 in congenital heart and neural tube defects in Chinese people. Clinical science (London, England : 1979) 38 27756857
2010 Basal enrichment within neuroepithelia suggests novel function(s) for Celsr1 protein. Molecular and cellular neurosciences 37 20353824
2019 Increasing evidence of hereditary lymphedema caused by CELSR1 loss-of-function variants. American journal of medical genetics. Part A 33 31215153
2021 Overexpression of circ_CELSR1 facilitates paclitaxel resistance of ovarian cancer by regulating miR-149-5p/SIK2 axis. Anti-cancer drugs 31 33735118
2023 The adhesion GPCRs CELSR1-3 and LPHN3 engage G proteins via distinct activation mechanisms. Cell reports 29 37224017
2012 Planar cell polarity genes, Celsr1-3, in neural development. Neuroscience bulletin 29 22622831
2017 Neural progenitor fate decision defects, cortical hypoplasia and behavioral impairment in Celsr1-deficient mice. Molecular psychiatry 28 29257130
2017 Sex-Based Genetic Association Study Identifies CELSR1 as a Possible Chronic Obstructive Pulmonary Disease Risk Locus among Women. American journal of respiratory cell and molecular biology 24 27854507
2021 Intercellular and intracellular cilia orientation is coordinated by CELSR1 and CAMSAP3 in oviduct multi-ciliated cells. Journal of cell science 23 33468623
2019 Sex-limited penetrance of lymphedema to females with CELSR1 haploinsufficiency: A second family. Clinical genetics 23 31403174
2013 Atypical cadherins Celsr1-3 and planar cell polarity in vertebrates. Progress in molecular biology and translational science 22 23481196
2022 CELSR1 variants are associated with partial epilepsy of childhood. American journal of medical genetics. Part B, Neuropsychiatric genetics : the official publication of the International Society of Psychiatric Genetics 21 36453712
2022 CELSR1 Risk Alleles in Familial Bicuspid Aortic Valve and Hypoplastic Left Heart Syndrome. Circulation. Genomic and precision medicine 19 35133174
2023 Celsr1 and Celsr2 exhibit distinct adhesive interactions and contributions to planar cell polarity. Frontiers in cell and developmental biology 18 36712970
2016 The atypical cadherin Celsr1 functions non-cell autonomously to block rostral migration of facial branchiomotor neurons in mice. Developmental biology 17 27395006
2020 CELSR1 Promotes Neuroprotection in Cerebral Ischemic Injury Mainly Through the Wnt/PKC Signaling Pathway. International journal of molecular sciences 16 32070035
2016 CELSR1 Is a Positive Regulator of Endothelial Cell Migration and Angiogenesis. Biochemistry. Biokhimiia 13 27301287
2021 Implication of Vestibular Hair Cell Loss of Planar Polarity for the Canal and Otolith-Dependent Vestibulo-Ocular Reflexes in Celsr1 Mice. Frontiers in neuroscience 10 34790090
2025 Structural basis for regulation of CELSR1 by a compact module in its extracellular region. Nature communications 9 40295529
2020 CELSR1 Acts as an Oncogene Regulated by miR-199a-5p in Glioma. Cancer management and research 8 33061581
2024 Structure of the extracellular region of the adhesion GPCR CELSR1 reveals a compact module which regulates G protein-coupling. bioRxiv : the preprint server for biology 7 38328199
2023 Lymphedema is associated with CELSR1 in Phelan-McDermid syndrome. Clinical genetics 6 37232218
2023 Upregulation of CELSR1 expression promotes ovarian cancer cell proliferation, migration, and invasion. Medical oncology (Northwood, London, England) 6 38070011
2014 The CELSR1 polymorphisms rs6007897 and rs4044210 are associated with ischaemic stroke in Chinese Han population. Annals of human biology 6 25117632
2001 Mutational analysis of the neuronal cadherin gene CELSR1 and exclusion as a candidate for catatonic schizophrenia in a large family. Psychiatric genetics 6 11807409
2024 CELSR1, a core planar cell polarity protein, features a weakly adhesive and flexible cadherin ectodomain. Structure (London, England : 1993) 5 38307021
2021 Lymphedema complicated by protein-losing enteropathy with a 22q13.3 deletion and the potential role of CELSR1: A case report. Medicine 5 34128868
2013 Association of genetic variants of CELSR1 and 3q28 with hypertension in community-dwelling individuals. Biomedical reports 5 24649039
2003 Genetic variation in the seven-pass transmembrane cadherin CELSR1: lack of association with schizophrenia. Psychiatric genetics 5 12782967
2023 The adhesion GPCRs CELSR1-3 and LPHN3 engage G proteins via distinct activation mechanisms. bioRxiv : the preprint server for biology 4 37066404
2023 Ureteropelvic junction obstruction with primary lymphoedema associated with CELSR1 variants. Journal of medical genetics 4 37225411
2024 De novo heterozygous missense variants in CELSR1 as cause of fetal pleural effusions and progressive fetal hydrops. Journal of medical genetics 3 38272662
2026 Biallelic variants in CELSR1 cause brain malformations, neurodevelopmental disorders and epilepsy in humans. Nature communications 2 41530147
2022 Celsr1 suppresses Wnt5a-mediated chemoattraction to prevent incorrect rostral migration of facial branchiomotor neurons. Development (Cambridge, England) 1 36325991

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