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FLASH GENE
Symbol ADGRG6 contributors: mct/npt/pgu - updated : 14-11-2023
HGNC name adhesion G protein-coupled receptor G6
HGNC id 13841
EXPRESSION
Type widely
   expressed in (based on citations)
organ(s)
SystemOrgan level 1Organ level 2Organ level 3Organ level 4LevelPubmedSpeciesStageRna symbol
Cardiovascularheartventricle    Mus musculusFetal
Digestiveintestinesmall intestine  highly
Reproductivefemale systemplacenta  highly
Respiratorylung     Mus musculusAdult
Urinarykidneytubule    Homo sapiensFetal
 kidneytubulecollecting duct highly Homo sapiensAdult
tissue
SystemTissueTissue level 1Tissue level 2LevelPubmedSpeciesStageRna symbol
Epithelialbarrier/liningretinal pigment epithelium (RPE)  
Muscularstriatumcardiac   Mus musculusFetal
cells
SystemCellPubmedSpeciesStageRna symbol
Cardiovascularendothelial cell
Respiratoryalveolar cell type 2 Homo sapiens
Respiratoryepithelial cell Homo sapiens
Urinaryepithelial cell Homo sapiens
cell lineage
cell lines
fluid/secretion
at STAGE
PROTEIN
PHYSICAL PROPERTIES
STRUCTURE
motifs/domains
  • a long extracellular N-terminal region and
  • C1r-C1s, Uegf and Bmp1 (CUB) and pentraxin (PTX)-like modules
  • a mucin-like spacer
  • a seven transmembrane domain (7tm_2)
  • a conserved GAIN domain
  • a GPCR proteolytic site (GPS) domains and heptahelical transmembrane domain shared by all GPCRs
    • HOMOLOGY
    interspecies homolog to rattus Gpr126 (84,68 pc)
    homolog to murine Gpr126 (84.19 pc)
    Homologene
    FAMILY
  • G-protein coupled receptor 2 family
  • LN-TM7 subfamily
  • adhesion GPCR (aGPCR) family of proteins
    • CATEGORY receptor membrane G
    SUBCELLULAR LOCALIZATION     plasma membrane
    basic FUNCTION
  • may be involved in neuropeptide signaling pathway
  • playing an important role in cell-adhesion and potentially in inflammation and coagulation
  • regulates the proliferation of pulmonary arterial smooth muscle cells and airway smooth muscle cells
  • required autonomously in Schwann cells for myelination
  • drives the differentiation of promyelinating Schwann cells by elevating cAMP levels, thereby triggering SLC22a16 expression and myelination
  • potentially equired for embryonic viability and cardiovascular development
  • essential for myelination and other aspects of peripheral nerve development in mammals
  • new roles in axonal sorting, formation of mature non-myelinating Schwann cells and organization of the perineurium
  • is required for proper trabeculation, contraction, and energy metabolism of the heart
  • acts as a receptor to drive myelin gene expression in PNS Schwann cells, whereas in the heart, the N-terminus of GPR126 acts as a ligand or coreceptor activating an unknown receptor
  • directly increases cAMP by coupling to heterotrimeric G-proteins
  • functions potentially in Schwann cells for proper development and myelination and these functions are mediated via G-protein-signaling pathways
  • modulates both physiological and pathological angiogenesis through VEGFA signaling, providing a potential target for the treatment of angiogenesis-related diseases
  • is critical for myelination of peripheral nerves
  • has also been implicated in bone mineralization
  • ADGRG6 is essential for Schwann cells (SCs) development and myelination
  • adhesion G protein-coupled receptor essential for Schwann cell (SC) myelination with important contributions to repair after nerve crush injury
  • ADGRG6 contributes to the terminal Schwann cell response at the neuromuscular junction following peripheral nerve injury
  • ADGRG6 extracellular regions (ECRs) utilizes a multi-faceted dynamic approach to regulate receptor function and provide relevant insights for ECR-targeted drug design
  • ADGRG6 is essential for placental development
  • possible role of ADGRG6 in kidney physiology
  • critical developmental roles in Schwann cell maturation and its mediated myelination in the peripheral nervous system
  • is likely involved in mounting an epithelial inflammatory response to smoke exposure
    • CELLULAR PROCESS
    PHYSIOLOGICAL PROCESS
    PATHWAY
    metabolism
    signaling signal transduction
    a component
    INTERACTION
    DNA
    RNA
    small molecule
    protein
    cell & other
  • ADGRG6-expressing cells are known to be exposed to mechanical stimuli
    • REGULATION
    ASSOCIATED DISORDERS
    corresponding disease(s) AMCN4
    Other morbid association(s)
    TypeGene ModificationChromosome rearrangementProtein expressionProtein Function
    constitutional     --low  
    in type 2 alveolar epithelial cells is decreased in chronic obstructive pulmonary disease (COPD)
    Susceptibility
    Variant & Polymorphism
    Candidate gene
    Marker
    Therapy target
    SystemTypeDisorderPubmed
    respiratorylungchr.Bronchopathy obstructive
    as likely a novel therapeutic target for COPD
    neurologyacquired 
    represents an attractive potential target to stimulate repair in myelin disease or following nerve injury
    ANIMAL & CELL MODELS
  • Gpr126 deletion causes heart phenotypes in mice causing embryonic lethality
  • a mutation in Gpr126 causes a severe congenital hypomyelinating peripheral neuropathy in mice
  • Gpr126-/- mice have a lethal arthrogryposis phenotype
  • Gpr126-null mice with a heterozygous placenta survive but exhibit GPR126-defective peripheral nervous system (PNS) phenotype