Entry - *601965 - SPHINGOSINE-1-PHOSPHATE RECEPTOR 3; S1PR3 - OMIM
 
* 601965

SPHINGOSINE-1-PHOSPHATE RECEPTOR 3; S1PR3


Alternative titles; symbols

ENDOTHELIAL DIFFERENTIATION GENE 3; EDG3
S1P RECEPTOR 3; S1P3


HGNC Approved Gene Symbol: S1PR3

Cytogenetic location: 9q22.1     Genomic coordinates (GRCh38): 9:88,990,863-89,005,155 (from NCBI)


TEXT

Description

The lysosphingolipid sphingosine 1-phosphate (S1P) regulates cell proliferation, apoptosis, motility, and neurite retraction. Its actions may be both intracellular as a second messenger and extracellular as a receptor ligand. S1P and the structurally related lysolipid mediator lysophosphatidic acid (LPA) signal cells through a set of G protein-coupled receptors (GPRs) known as EDG receptors. Some EDG receptors (e.g., EDG1, 601974) are S1P receptors; others (e.g., EDG2, 602282) are LPA receptors (summary by Chun et al., 2002).


Nomenclature

Chun et al. (2002) proposed a nomenclature scheme for the lysophosphatic acid (LPA) and sphingosine 1-phosphatase (S1P) receptors that is consistent with the International Union of Pharmacology (IUP) guidelines. According to these guidelines, a receptor is to be named with the abbreviation of the natural agonist with the highest potency, followed by a subscripted arabic number. Thus they suggested that the designation EDG3 should be changed to S1P3.


Cloning and Expression

GPRs contain 7 hydrophobic transmembrane domains connected by hydrophilic intra- and extracellular loops. They transduce a variety of hormone and neurotransmitter signals into intracellular effects via G proteins (see 600239, 601047, 601908). Yamaguchi et al. (1996) isolated a human GPR gene that they designated EDG3 because of its sequence similarity (51.9%) to EDG1, an endothelial differentiation gene. Northern blotting showed that a 2.8-kb transcript of EDG3 is expressed in all tissues, but most abundantly in heart, placenta, kidney, and liver.


Gene Function

An et al. (1997) found that overexpression of EDG3 in mammalian cells activated a serum response element-driven transcriptional reporter gene in response to S1P, suggesting that EDG3 is a functional receptor for S1P.

By microinjection of EDG mRNA into Xenopus oocytes, Ancellin and Hla (1999) determined that human EDG3 and rat Edg5 (605111), but not human EDG1, conferred S1P-responsive intracellular calcium transients. All 3 EDGs were also activated by sphingosylphosphorylcholine (SPC), albeit at higher concentrations. Ancellin and Hla (1999) also found evidence that the 3 receptors signal differentially by coupling to different G proteins.

Himmel et al. (2000) investigated the sphingolipid-induced activation of inward rectifier K+ currents, or I(K.ACh), in freshly isolated guinea pig, mouse, and human atrial myocytes. S1P activated I(K.ACh) in atrial myocytes from all 3 species, and activation of human myocytes by S1P was blocked by the EDG3-selective antagonist suramin. SPC also activated I(K.ACh) currents in guinea pig myocytes, but was almost ineffective in mouse and human myocytes. PCR analysis identified EDG1, EDG3, and EDG5 transcripts in human atrial cells. The authors concluded that myocyte activation by S1P and SPC exhibits large species differences and that the S1P-induced I(K.ACh) activation in human atrial myocytes is mediated by EDG3.

Nofer et al. (2004) studied the vasoactive effects of 3 lysophospholipids present in high density lipoprotein (HDL): SPC, S1P, and lysosulfatide (LSF). All 3 elevated intracellular Ca(2+) concentration and activated AKT1 (164730) and nitric oxide synthase-3 (NOS3; 163729), which resulted in NO release and vasodilation. Deficiency of EDG3 abolished the lysophospholipid vasodilatory effects and reduced the effect of HDL by approximately 60%. The authors concluded that HDL is a carrier of bioactive lysophospholipids that regulate vascular tone via EDG3-mediated NO release.

Niessen et al. (2008) demonstrated that protease-activated receptor-1 (PAR1; 187930) signaling sustains a lethal inflammatory response that can be interrupted by inhibition of either thrombin (176930) or PAR1 signaling. The S1P axis is a downstream component of PAR1 signaling, and by combining chemical and genetic probes for S1P3, Niessen et al. (2008) showed a critical role for dendritic cell PAR1-S1P3 crosstalk in regulating amplification of inflammation in sepsis syndrome. Conversely, dendritic cells sustain escalated systemic coagulation and are the primary hub at which coagulation and inflammation intersect within the lymphatic compartment. Loss of dendritic cell PAR1-S1P3 signaling sequestered dendritic cells and inflammation into draining lymph nodes, and attenuated dissemination of interleukin-1-beta (147720) to the lungs. Thus, Niessen et al. (2008) concluded that activation of dendritic cells by coagulation in the lymphatics emerged as a theretofore unknown mechanism that promotes systemic inflammation and lethality in decompensated innate immune responses.


Mapping

Yamaguchi et al. (1996) mapped the S1PR3 gene to chromosome 9q22.1-q22.2 by fluorescence in situ hybridization.


Animal Model

Ishii et al. (2001) disrupted the Edg3 gene in mice, resulting in complete absence of the gene, transcript, and protein. Edg3-null mice were viable and fertile and developed normally with no obvious phenotypic abnormality. Wildtype mouse embryonic fibroblasts expressed Edg1, Edg5, and Edg3 and were highly responsive to S1P in phospholipase C (PLC; see 600810) activation, adenylyl cyclase inhibition, and Rho (see 602732) activation. Edg3-null fibroblasts showed significant decreases in PLC activation, slight decreases in adenylyl cyclase inhibition, and no change in Rho activation. Ishii et al. (2001) concluded that Edg3 has a nonessential role in normal mouse development but shows nonredundant cellular signaling in response to S1P.

Ishii et al. (2002) developed mice null for both Edg3 and Edg5. Mice deficient in Edg5 alone were viable and fertile and developed normally. The litter sizes from Edg5-Edg3 double-null crosses were remarkably reduced, and these pups often did not survive through infancy, although double-null survivors showed no obvious phenotype. Ishii et al. (2002) concluded that either receptor subtype supports embryonic development, but deletion of both produces marked perinatal lethality. They examined mouse embryonic fibroblasts for the effects of receptor deletions on S1P signaling. Edg5-null fibroblasts showed a significant decrease in Rho activation with exposure to S1P, and double-null fibroblasts displayed a complete loss of Rho activation and a significant decrease in PLC activation and calcium mobilization, with no effect on adenylyl cyclase inhibition. Ishii et al. (2002) concluded that there is preferential coupling of Edg5 and Edg3 to Rho and PLC/Ca(2+) pathways, respectively, in the mouse.


REFERENCES

  1. An, S., Bleu, T., Huang, W., Hallmark, O. G., Coughlin, S. R., Goetzl, E. J. Identification of cDNAs encoding two G protein-coupled receptors for lysosphingolipids. FEBS Lett. 417: 279-282, 1997. [PubMed: 9409733, related citations] [Full Text]

  2. Ancellin, N., Hla, T. Differential pharmacological properties and signal transduction of the sphingosine 1-phosphate receptors EDG-1, EDG-3, and EDG-5. J. Biol. Chem. 274: 18997-19002, 1999. [PubMed: 10383399, related citations] [Full Text]

  3. Chun, J., Goetzl, E. J., Hla, T., Igarashi, Y., Lynch, K. R., Moolenaar, W., Pyne, S., Tigyi, G. International Union of Pharmacology. XXXIV. Lysophospholipid receptor nomenclature. Pharm. Rev. 54: 265-269, 2002. [PubMed: 12037142, related citations] [Full Text]

  4. Himmel, H. M., Meyer Zu Heringdorf, D., Graf, E., Dobrev, D., Kortner, A., Schuler, S., Jakobs, K. H., Ravens, U. Evidence for Edg-3 receptor-mediated activation of I(K.ACh) by sphingosine-1-phosphate in human atrial cardiomyocytes. Molec. Pharm. 58: 449-454, 2000. [PubMed: 10908314, related citations] [Full Text]

  5. Ishii, I., Friedman, B., Ye, X., Kawamura, S., McGiffert, C., Contos, J. J. A., Kingsbury, M. A., Zhang, G., Brown, J. H., Chun, J. Selective loss of sphingosine 1-phosphate signaling with no obvious phenotypic abnormality in mice lacking its G protein-coupled receptor, LP-B3/EDG-3. J. Biol. Chem. 276: 33697-33704, 2001. [PubMed: 11443127, related citations] [Full Text]

  6. Ishii, I., Ye, X., Friedman, B., Kawamura, S., Contos, J. J. A., Kingsbury, M. A., Yang, A. H., Zhang, G., Brown, J. H., Chun, J. Marked perinatal lethality and cellular signaling deficits in mice null for the two sphingosine 1-phosphate (S1P) receptors, S1P-2/LP-B2/EDG-5 and S1P-3/LP-B3/EDG-3. J. Biol. Chem. 277: 25152-25159, 2002. [PubMed: 12006579, related citations] [Full Text]

  7. Niessen, F., Schaffner, F., Furlan-Freguia, C., Pawlinski, R., Bhattacharjee, G., Chun, J., Derian, C. K., Andrade-Gordon, P., Rosen, H., Ruf, W. Dendritic cell PAR1-S1P3 signalling couples coagulation and inflammation. Nature 452: 654-658, 2008. [PubMed: 18305483, related citations] [Full Text]

  8. Nofer, J.-R., van der Giet, M., Tolle, M., Wolinska, I., von Wnuck Lipinski, K., Baba, H. A., Tietge, U. J., Godecke, A., Ishii, I., Kleuser, B., Schafers, M., Fobker, M., Zidek, W., Assmann, G., Chun, J., Levkau, B. HDL induces NO-dependent vasorelaxation via the lysophospholipid receptor S1P-3. J. Clin. Invest. 113: 569-581, 2004. [PubMed: 14966566, images, related citations] [Full Text]

  9. Yamaguchi, F., Tokuda, M., Hatase, O., Brenner, S. Molecular cloning of the novel human G protein-coupled receptor (GPCR) gene mapped on chromosome 9. Biochem. Biophys. Res. Commun. 227: 608-614, 1996. [PubMed: 8878560, related citations] [Full Text]


Ada Hamosh - updated : 4/16/2008
Marla J. F. O'Neill - updated : 3/4/2004
Patricia A. Hartz - updated : 8/19/2002
Paul J. Converse - updated : 7/10/2000
Rebekah S. Rasooly - updated : 4/24/1998
Creation Date:
Lori M. Kelman : 9/2/1997
carol : 05/24/2022
alopez : 11/30/2015
carol : 5/7/2014
wwang : 9/25/2008
alopez : 5/14/2008
terry : 4/16/2008
carol : 2/29/2008
carol : 2/29/2008
terry : 4/5/2005
tkritzer : 3/5/2004
terry : 3/4/2004
alopez : 1/21/2004
terry : 11/22/2002
mgross : 8/19/2002
mgross : 8/19/2002
mgross : 7/10/2000
mgross : 7/6/2000
alopez : 5/26/1998
psherman : 4/29/1998
psherman : 4/24/1998
dholmes : 9/15/1997
dholmes : 9/5/1997

* 601965

SPHINGOSINE-1-PHOSPHATE RECEPTOR 3; S1PR3


Alternative titles; symbols

ENDOTHELIAL DIFFERENTIATION GENE 3; EDG3
S1P RECEPTOR 3; S1P3


HGNC Approved Gene Symbol: S1PR3

Cytogenetic location: 9q22.1     Genomic coordinates (GRCh38): 9:88,990,863-89,005,155 (from NCBI)


TEXT

Description

The lysosphingolipid sphingosine 1-phosphate (S1P) regulates cell proliferation, apoptosis, motility, and neurite retraction. Its actions may be both intracellular as a second messenger and extracellular as a receptor ligand. S1P and the structurally related lysolipid mediator lysophosphatidic acid (LPA) signal cells through a set of G protein-coupled receptors (GPRs) known as EDG receptors. Some EDG receptors (e.g., EDG1, 601974) are S1P receptors; others (e.g., EDG2, 602282) are LPA receptors (summary by Chun et al., 2002).


Nomenclature

Chun et al. (2002) proposed a nomenclature scheme for the lysophosphatic acid (LPA) and sphingosine 1-phosphatase (S1P) receptors that is consistent with the International Union of Pharmacology (IUP) guidelines. According to these guidelines, a receptor is to be named with the abbreviation of the natural agonist with the highest potency, followed by a subscripted arabic number. Thus they suggested that the designation EDG3 should be changed to S1P3.


Cloning and Expression

GPRs contain 7 hydrophobic transmembrane domains connected by hydrophilic intra- and extracellular loops. They transduce a variety of hormone and neurotransmitter signals into intracellular effects via G proteins (see 600239, 601047, 601908). Yamaguchi et al. (1996) isolated a human GPR gene that they designated EDG3 because of its sequence similarity (51.9%) to EDG1, an endothelial differentiation gene. Northern blotting showed that a 2.8-kb transcript of EDG3 is expressed in all tissues, but most abundantly in heart, placenta, kidney, and liver.


Gene Function

An et al. (1997) found that overexpression of EDG3 in mammalian cells activated a serum response element-driven transcriptional reporter gene in response to S1P, suggesting that EDG3 is a functional receptor for S1P.

By microinjection of EDG mRNA into Xenopus oocytes, Ancellin and Hla (1999) determined that human EDG3 and rat Edg5 (605111), but not human EDG1, conferred S1P-responsive intracellular calcium transients. All 3 EDGs were also activated by sphingosylphosphorylcholine (SPC), albeit at higher concentrations. Ancellin and Hla (1999) also found evidence that the 3 receptors signal differentially by coupling to different G proteins.

Himmel et al. (2000) investigated the sphingolipid-induced activation of inward rectifier K+ currents, or I(K.ACh), in freshly isolated guinea pig, mouse, and human atrial myocytes. S1P activated I(K.ACh) in atrial myocytes from all 3 species, and activation of human myocytes by S1P was blocked by the EDG3-selective antagonist suramin. SPC also activated I(K.ACh) currents in guinea pig myocytes, but was almost ineffective in mouse and human myocytes. PCR analysis identified EDG1, EDG3, and EDG5 transcripts in human atrial cells. The authors concluded that myocyte activation by S1P and SPC exhibits large species differences and that the S1P-induced I(K.ACh) activation in human atrial myocytes is mediated by EDG3.

Nofer et al. (2004) studied the vasoactive effects of 3 lysophospholipids present in high density lipoprotein (HDL): SPC, S1P, and lysosulfatide (LSF). All 3 elevated intracellular Ca(2+) concentration and activated AKT1 (164730) and nitric oxide synthase-3 (NOS3; 163729), which resulted in NO release and vasodilation. Deficiency of EDG3 abolished the lysophospholipid vasodilatory effects and reduced the effect of HDL by approximately 60%. The authors concluded that HDL is a carrier of bioactive lysophospholipids that regulate vascular tone via EDG3-mediated NO release.

Niessen et al. (2008) demonstrated that protease-activated receptor-1 (PAR1; 187930) signaling sustains a lethal inflammatory response that can be interrupted by inhibition of either thrombin (176930) or PAR1 signaling. The S1P axis is a downstream component of PAR1 signaling, and by combining chemical and genetic probes for S1P3, Niessen et al. (2008) showed a critical role for dendritic cell PAR1-S1P3 crosstalk in regulating amplification of inflammation in sepsis syndrome. Conversely, dendritic cells sustain escalated systemic coagulation and are the primary hub at which coagulation and inflammation intersect within the lymphatic compartment. Loss of dendritic cell PAR1-S1P3 signaling sequestered dendritic cells and inflammation into draining lymph nodes, and attenuated dissemination of interleukin-1-beta (147720) to the lungs. Thus, Niessen et al. (2008) concluded that activation of dendritic cells by coagulation in the lymphatics emerged as a theretofore unknown mechanism that promotes systemic inflammation and lethality in decompensated innate immune responses.


Mapping

Yamaguchi et al. (1996) mapped the S1PR3 gene to chromosome 9q22.1-q22.2 by fluorescence in situ hybridization.


Animal Model

Ishii et al. (2001) disrupted the Edg3 gene in mice, resulting in complete absence of the gene, transcript, and protein. Edg3-null mice were viable and fertile and developed normally with no obvious phenotypic abnormality. Wildtype mouse embryonic fibroblasts expressed Edg1, Edg5, and Edg3 and were highly responsive to S1P in phospholipase C (PLC; see 600810) activation, adenylyl cyclase inhibition, and Rho (see 602732) activation. Edg3-null fibroblasts showed significant decreases in PLC activation, slight decreases in adenylyl cyclase inhibition, and no change in Rho activation. Ishii et al. (2001) concluded that Edg3 has a nonessential role in normal mouse development but shows nonredundant cellular signaling in response to S1P.

Ishii et al. (2002) developed mice null for both Edg3 and Edg5. Mice deficient in Edg5 alone were viable and fertile and developed normally. The litter sizes from Edg5-Edg3 double-null crosses were remarkably reduced, and these pups often did not survive through infancy, although double-null survivors showed no obvious phenotype. Ishii et al. (2002) concluded that either receptor subtype supports embryonic development, but deletion of both produces marked perinatal lethality. They examined mouse embryonic fibroblasts for the effects of receptor deletions on S1P signaling. Edg5-null fibroblasts showed a significant decrease in Rho activation with exposure to S1P, and double-null fibroblasts displayed a complete loss of Rho activation and a significant decrease in PLC activation and calcium mobilization, with no effect on adenylyl cyclase inhibition. Ishii et al. (2002) concluded that there is preferential coupling of Edg5 and Edg3 to Rho and PLC/Ca(2+) pathways, respectively, in the mouse.


REFERENCES

  1. An, S., Bleu, T., Huang, W., Hallmark, O. G., Coughlin, S. R., Goetzl, E. J. Identification of cDNAs encoding two G protein-coupled receptors for lysosphingolipids. FEBS Lett. 417: 279-282, 1997. [PubMed: 9409733] [Full Text: https://doi.org/10.1016/s0014-5793(97)01301-x]

  2. Ancellin, N., Hla, T. Differential pharmacological properties and signal transduction of the sphingosine 1-phosphate receptors EDG-1, EDG-3, and EDG-5. J. Biol. Chem. 274: 18997-19002, 1999. [PubMed: 10383399] [Full Text: https://doi.org/10.1074/jbc.274.27.18997]

  3. Chun, J., Goetzl, E. J., Hla, T., Igarashi, Y., Lynch, K. R., Moolenaar, W., Pyne, S., Tigyi, G. International Union of Pharmacology. XXXIV. Lysophospholipid receptor nomenclature. Pharm. Rev. 54: 265-269, 2002. [PubMed: 12037142] [Full Text: https://doi.org/10.1124/pr.54.2.265]

  4. Himmel, H. M., Meyer Zu Heringdorf, D., Graf, E., Dobrev, D., Kortner, A., Schuler, S., Jakobs, K. H., Ravens, U. Evidence for Edg-3 receptor-mediated activation of I(K.ACh) by sphingosine-1-phosphate in human atrial cardiomyocytes. Molec. Pharm. 58: 449-454, 2000. [PubMed: 10908314] [Full Text: https://doi.org/10.1124/mol.58.2.449]

  5. Ishii, I., Friedman, B., Ye, X., Kawamura, S., McGiffert, C., Contos, J. J. A., Kingsbury, M. A., Zhang, G., Brown, J. H., Chun, J. Selective loss of sphingosine 1-phosphate signaling with no obvious phenotypic abnormality in mice lacking its G protein-coupled receptor, LP-B3/EDG-3. J. Biol. Chem. 276: 33697-33704, 2001. [PubMed: 11443127] [Full Text: https://doi.org/10.1074/jbc.M104441200]

  6. Ishii, I., Ye, X., Friedman, B., Kawamura, S., Contos, J. J. A., Kingsbury, M. A., Yang, A. H., Zhang, G., Brown, J. H., Chun, J. Marked perinatal lethality and cellular signaling deficits in mice null for the two sphingosine 1-phosphate (S1P) receptors, S1P-2/LP-B2/EDG-5 and S1P-3/LP-B3/EDG-3. J. Biol. Chem. 277: 25152-25159, 2002. [PubMed: 12006579] [Full Text: https://doi.org/10.1074/jbc.M200137200]

  7. Niessen, F., Schaffner, F., Furlan-Freguia, C., Pawlinski, R., Bhattacharjee, G., Chun, J., Derian, C. K., Andrade-Gordon, P., Rosen, H., Ruf, W. Dendritic cell PAR1-S1P3 signalling couples coagulation and inflammation. Nature 452: 654-658, 2008. [PubMed: 18305483] [Full Text: https://doi.org/10.1038/nature06663]

  8. Nofer, J.-R., van der Giet, M., Tolle, M., Wolinska, I., von Wnuck Lipinski, K., Baba, H. A., Tietge, U. J., Godecke, A., Ishii, I., Kleuser, B., Schafers, M., Fobker, M., Zidek, W., Assmann, G., Chun, J., Levkau, B. HDL induces NO-dependent vasorelaxation via the lysophospholipid receptor S1P-3. J. Clin. Invest. 113: 569-581, 2004. [PubMed: 14966566] [Full Text: https://doi.org/10.1172/JCI18004]

  9. Yamaguchi, F., Tokuda, M., Hatase, O., Brenner, S. Molecular cloning of the novel human G protein-coupled receptor (GPCR) gene mapped on chromosome 9. Biochem. Biophys. Res. Commun. 227: 608-614, 1996. [PubMed: 8878560] [Full Text: https://doi.org/10.1006/bbrc.1996.1553]


Contributors:
Ada Hamosh - updated : 4/16/2008
Marla J. F. O'Neill - updated : 3/4/2004
Patricia A. Hartz - updated : 8/19/2002
Paul J. Converse - updated : 7/10/2000
Rebekah S. Rasooly - updated : 4/24/1998

Creation Date:
Lori M. Kelman : 9/2/1997

Edit History:
carol : 05/24/2022
alopez : 11/30/2015
carol : 5/7/2014
wwang : 9/25/2008
alopez : 5/14/2008
terry : 4/16/2008
carol : 2/29/2008
carol : 2/29/2008
terry : 4/5/2005
tkritzer : 3/5/2004
terry : 3/4/2004
alopez : 1/21/2004
terry : 11/22/2002
mgross : 8/19/2002
mgross : 8/19/2002
mgross : 7/10/2000
mgross : 7/6/2000
alopez : 5/26/1998
psherman : 4/29/1998
psherman : 4/24/1998
dholmes : 9/15/1997
dholmes : 9/5/1997