364PathwayIntracellular Signalling Through FSH Receptor and Follicle Stimulating HormoneThe follicle-stimulating hormone receptor (FSHR) is a transmembrane G protein-coupled receptor located in the ovary, testis, and uterus. As its name suggests, the receptor is activated by follicle-stimulating hormone (FSH) and plays an important role in the regulation of reproduction. In the ovaries, FSHRs present on granulosa cells initiate follicular development. In the testis, FSHRs present on Sertoli cells are essential for spermatogenesis. The FSH receptor activates G(s) proteins which leads to the activation of adenylyl cyclase and produces the secondary messenger cAMP. cAMP then activates protein kinase A (PKA) which phosphorylates downstream effectors that lead to a specific cellular response.ProteinPW000448TopPathwayVisualizationContext46430004000#000099PathwayVisualization348364Intracellular Signalling Through FSH Receptor and Follicle Stimulating HormoneThe follicle-stimulating hormone receptor (FSHR) is a transmembrane G protein-coupled receptor located in the ovary, testis, and uterus. As its name suggests, the receptor is activated by follicle-stimulating hormone (FSH) and plays an important role in the regulation of reproduction. In the ovaries, FSHRs present on granulosa cells initiate follicular development. In the testis, FSHRs present on Sertoli cells are essential for spermatogenesis. The FSH receptor activates G(s) proteins which leads to the activation of adenylyl cyclase and produces the secondary messenger cAMP. cAMP then activates protein kinase A (PKA) which phosphorylates downstream effectors that lead to a specific cellular response.Protein1688Follicle-stimulating hormone activation of follicle-stimulating hormone receptor bound to G protein complexActivatingSubPathway11391000ProteinComplex151140997ProteinComplex14692Gonad DevelopmentSubPathway114441Compound8693Gamete Generation in FemalesSubPathway114541Compound8694Spermatogenesis in MalesSubPathway114641Compound810799408742Simoni M, Gromoll J, Nieschlag E: The follicle-stimulating hormone receptor: biochemistry, molecular biology, physiology, and pathophysiology. Endocr Rev. 1997 Dec;18(6):739-73. doi: 10.1210/edrv.18.6.0320.364Pathway1080Krauss, G. Biochemistry of signal transduction and regulation. (3rd ed.) (2003) Weinheim; New York : Wiley-VCH364Pathway108112059813Asatiani K, Gromoll J, Eckardstein SV, Zitzmann M, Nieschlag E, Simoni M: Distribution and function of FSH receptor genetic variants in normal men. Andrologia. 2002 Jun;34(3):172-6.364Pathway1CellCL:00000003NeuronCL:00005402Platelet CL:00002335HepatocyteCL:00001824Cardiomyocyte CL:00007468Beta cellCL:00006397Epithelial CellCL:00000661Homo sapiens9606EukaryoteHuman3Escherichia coli562Prokaryote24Solanum lycopersicum4081EukaryoteTomato18Saccharomyces cerevisiae4932EukaryoteYeast21Xenopus laevis8355EukaryoteAfrican clawed frog4Arabidopsis thaliana3702EukaryoteThale cress12Mus musculus10090EukaryoteMouse49Bathymodiolus platifrons220390EukaryoteDeep sea mussel60Nitzschia sp.0001EukaryoteNitzschia45Bos taurus9913EukaryoteCattle17Rattus norvegicus10116EukaryoteRat10Drosophila melanogaster7227EukaryoteFruit fly6Caenorhabditis elegans6239EukaryoteRoundworm2Bacteria2ProkaryoteBacteria19Schizosaccharomyces pombe4896Eukaryote25Escherichia coli (strain K12)83333Prokaryote23Pseudomonas aeruginosa287Prokaryote51Picea sitchensis3332EukaryoteSitka spruce29Saccharomyces cerevisiae (strain ATCC 204508 / S288c)559292EukaryoteBaker's yeast56Andro6666Eukaryote5CytoplasmGO:00057373Mitochondrial MatrixGO:00057591CytosolGO:000582910Cell MembraneGO:000588635ChloroplastGO:00095074PeroxisomeGO:000577713Endoplasmic ReticulumGO:000578314Mitochondrial Outer MembraneGO:00057412MitochondrionGO:00057397Endoplasmic Reticulum MembraneGO:000578925Golgi apparatusGO:000579427Peroxisome MembraneGO:000577815NucleusGO:000563426Golgi apparatus membraneGO:000013911Extracellular SpaceGO:000561512Mitochondrial Inner MembraneGO:000574331Periplasmic SpaceGO:000562032Inner MembraneGO:007025819sarcoplasmic reticulumGO:001652934Plant-Type VacuoleGO:000032524BrainBTO:000014289162Endothelium BTO:00003931LiverBTO:00007597297Nervous SystemBTO:00014845cardiocyteBTO:00015398Blood VesselBTO:0001102741118PancreasBTO:000098825IntestineBTO:000064832Adrenal CortexBTO:00000454Adrenal MedullaBTO:00000497188511PW_BS0000084311PW_BS0000042111PW_BS00000214101PW_BS000014731013PW_BS00007385241011PW_BS000085103331PW_BS0001031553241PW_BS0001551613181PW_BS0001611783211PW_BS000178222341PW_BS0000241151012PW_BS0001151985181PW_BS000024151141PW_BS0001512905491PW_BS00002411PW_BS0000012253541PW_BS0000241333121PW_BS0001331115121PW_BS0001111321121PW_BS0001323683601PW_BS0000283081011PW_BS0000241644PW_BS000164253541PW_BS000024406351PW_BS000115122551PW_BS000122124151PW_BS0001241203171PW_BS0001201355171PW_BS0001351181171PW_BS0001184793101PW_BS0001152975101PW_BS0000242991101PW_BS000024501361PW_BS000115205561PW_BS000024388161PW_BS0001122111018PW_BS000024405105PW_BS0001153761017PW_BS00005316212PW_BS000016261115PW_BS0000265411PW_BS000005181311PW_BS000018221411PW_BS000022397113PW_BS0000393211PW_BS000003215114PW_BS00002146114PW_BS00004649711PW_BS000049432511PW_BS000043101711PW_BS000010592711PW_BS000059231511PW_BS00002329111PW_BS000029711PW_BS000007311511PW_BS000031918511PW_BS000091541315PW_BS000054562611PW_BS0000561041431PW_BS0001041122121PW_BS0001121471241PW_BS00014716611PW_BS0001661601181PW_BS00016019914181PW_BS000024206261PW_BS0000242137181PW_BS00002421425181PW_BS0000242164181PW_BS00002421013181PW_BS0000242491341PW_BS0000242881441PW_BS000024226441PW_BS0000242851041PW_BS00002432914121PW_BS0000283361121PW_BS0000283344121PW_BS00002833527121PW_BS0000283317121PW_BS00002835625121PW_BS00002813013121PW_BS000130184121PW_BS000024372102PW_BS00002815111PW_BS0000155181PW_BS000051383751PW_BS00010027151PW_BS0000272231241PW_BS000024390761PW_BS0001123987171PW_BS0001133821451PW_BS000100407251PW_BS000115408451PW_BS000115429151PW_BS0001154192551PW_BS0001151251351PW_BS0001254222751PW_BS00011539914171PW_BS0001131192171PW_BS0001193744171PW_BS0000534641171PW_BS00011545525171PW_BS00011513613171PW_BS00013637527171PW_BS00005348414101PW_BS0001154812101PW_BS0001154824101PW_BS0001154957101PW_BS00011549025101PW_BS0001154781010PW_BS00011549127101PW_BS00011530013101PW_BS0000243891461PW_BS000112502461PW_BS0001155072561PW_BS000115209106PW_BS0000245082761PW_BS0001153951361PW_BS00011313121PW_BS0000133211515PW_BS0000325811411PW_BS0000586618518PW_BS00006672513PW_BS000072612517PW_BS000061892PW_BS000089971521PW_BS000097100521PW_BS000100101531PW_BS000101117131PW_BS00011712915121PW_BS00012910813PW_BS00010814315191PW_BS0001431465191PW_BS000146107313PW_BS000107188118PW_BS0000242171518PW_BS00002421815181PW_BS0000241632181PW_BS0001631901118PW_BS0000242771218PW_BS00002417018PW_BS0001702811251PW_BS000024315123PW_BS0000243221231PW_BS0000243183123PW_BS00002413412121PW_BS0001343331212PW_BS00002833217121PW_BS000028350114121PW_BS00002812815121PW_BS0001283511512PW_BS00002835325127PW_BS000028943PW_BS000094109323PW_BS000109412125PW_BS0001151231751PW_BS00012343311451PW_BS0001154101551PW_BS000115435155PW_BS0001154461217PW_BS00011544717171PW_BS000115468114171PW_BS00011544415171PW_BS00011547225177PW_BS0001154701517PW_BS00011548515101PW_BS0001154991510PW_BS0001155161561PW_BS000115517156PW_BS00011530635511PW_BS000024171211PW_BS0000173841251PW_BS0001003911261PW_BS00011212112171PW_BS000121471914PW_BS0000476131PW_BS00000621217181PW_BS0000242273441PW_BS000024241529PW_BS00002425715291PW_BS00002430412PW_BS00002429341PW_BS00002429817101PW_BS0000245131761PW_BS000115599856PW_BS000501608321PW_BS0005016651818PW_BS0005087413PW_BS0000742441011PW_BS000024204111PW_BS000020986Guanosine triphosphateHMDB0001273Guanosine triphosphate (GTP) is a guanine nucleotide containing three phosphate groups esterified to the sugar moiety. GTP functions as a carrier of phosphates and pyrophosphates involved in channeling chemical energy into specific biosynthetic pathways. GTP activates the signal transducing G proteins which are involved in various cellular processes including proliferation, differentiation, and activation of several intracellular kinase cascades. Proliferation and apoptosis are regulated in part by the hydrolysis of GTP by small GTPases Ras and Rho. Another type of small GTPase, Rab, plays a role in the docking and fusion of vesicles and may also be involved in vesicle formation. In addition to its role in signal transduction, GTP also serves as an energy-rich precursor of mononucleotide units in the enzymatic biosynthesis of DNA and RNA.86-01-1C00044683015996GTP6569NC1=NC2=C(N=CN2[C@@H]2O[C@H](COP(O)(=O)OP(O)(=O)OP(O)(O)=O)[C@@H](O)[C@H]2O)C(=O)N1C10H16N5O14P3InChI=1S/C10H16N5O14P3/c11-10-13-7-4(8(18)14-10)12-2-15(7)9-6(17)5(16)3(27-9)1-26-31(22,23)29-32(24,25)28-30(19,20)21/h2-3,5-6,9,16-17H,1H2,(H,22,23)(H,24,25)(H2,19,20,21)(H3,11,13,14,18)/t3-,5-,6-,9-/m1/s1XKMLYUALXHKNFT-UUOKFMHZSA-N({[({[(2R,3S,4R,5R)-5-(2-amino-6-oxo-6,9-dihydro-1H-purin-9-yl)-3,4-dihydroxyoxolan-2-yl]methoxy}(hydroxy)phosphoryl)oxy](hydroxy)phosphoryl}oxy)phosphonic acid523.1804522.990659781-1.708triphosphate, guanosine0-3FDB0225275'-gtp;Gtg;Gtp;Guanosine 5'-(tetrahydrogen triphosphate);Guanosine 5'-triphosphate;Guanosine 5'-triphosphorate;Guanosine 5'-triphosphoric acid;Guanosine triphosphate;Guanosine mono(tetrahydrogen triphosphate) (ester);H4gtp;Guanosine-5'-triphosphatePW_C000986GTP81824041939240911441537350068553841036043155610316164901787478222117531151176919811981151127252906932717696222577144133775441117795113280025368800883088012116489113253119985406120066122121204124122768120122818135123774118125365479125443297126480299126907501126982205128051388936Guanosine diphosphateHMDB0001201Guanosine 5'-(trihydrogen diphosphate). A guanine nucleotide containing two phosphate groups esterified to the sugar moiety. It is an ester of pyrophosphoric acid with the nucleoside guanosine. GDP consists of the pyrophosphate group, the pentose sugar ribose, and the nucleobase guanine. GDP is the product of GTP dephosphorylation by GTPases, e.g. the G-proteins that are involved in signal transduction.146-91-8C00035897717552GDP-4-DEHYDRO-6-DEOXY-D-MANNOSE8630NC1=NC2=C(N=CN2[C@@H]2O[C@H](COP(O)(=O)OP(O)(O)=O)[C@@H](O)[C@H]2O)C(=O)N1C10H15N5O11P2InChI=1S/C10H15N5O11P2/c11-10-13-7-4(8(18)14-10)12-2-15(7)9-6(17)5(16)3(25-9)1-24-28(22,23)26-27(19,20)21/h2-3,5-6,9,16-17H,1H2,(H,22,23)(H2,19,20,21)(H3,11,13,14,18)/t3-,5-,6-,9-/m1/s1QGWNDRXFNXRZMB-UUOKFMHZSA-N[({[(2R,3S,4R,5R)-5-(2-amino-6-oxo-6,9-dihydro-3H-purin-9-yl)-3,4-dihydroxyoxolan-2-yl]methoxy}(hydroxy)phosphoryl)oxy]phosphonic acid443.2005443.024329371-2.007{[(2R,3S,4R,5R)-5-(2-amino-6-oxo-3H-purin-9-yl)-3,4-dihydroxyoxolan-2-yl]methoxy(hydroxy)phosphoryl}oxyphosphonic acid0-3FDB0224875'-gdp;Gdp;Guanosine 5'-(trihydrogen pyrophosphate);Guanosine 5'-diphosphate;Guanosine 5'-pyrophosphate;Guanosine mono(trihydrogen diphosphate);Guanosine pyrophosphate;Guanosine-5'-diphosphate;Guanosine-diphosphate;Ppg;Guanosine diphosphate;Guanosine 5'-diphosphoric acidPW_C000936GDP83823841762142391241547350078553821036041155610116164881787476222117541151177121111823198127272901339515169322177142133775461117795213280023368800803088012216489115253119983406120068122121205124121847405122766120122820135123775118124400376125363479125445297126905501126984205423MagnesiumHMDB0000547Magnesium salts are essential in nutrition, being required for the activity of many enzymes, especially those concerned with oxidative phosphorylation. Physiologically, it exists as an ion in the body. It is a component of both intra- and extracellular fluids and is excreted in the urine and feces. Deficiency causes irritability of the nervous system with tetany, vasodilatation, convulsions, tremors, depression, and psychotic behavior. Magnesium ion in large amounts is an ionic laxative, and magnesium sulfate (Epsom salts) is sometimes used for this purpose. So-called "milk of magnesia" is a water suspension of one of the few insoluble magnesium compounds, magnesium hydroxide; the undissolved particles give rise to its appearance and name. Milk of magnesia is a mild base, and is commonly used as an antacid.22537-22-0C003058881842013-HYDROXY-MAGNESIUM-PROTOPORP865DB01378[Mg++]MgInChI=1S/Mg/q+2JLVVSXFLKOJNIY-UHFFFAOYSA-Nmagnesium(2+) ion24.30523.9850418980magnesium(2+) ion22FDB003518Magnesium;Magnesium ions;Magnesium ion;Magnesium, doubly charged positive ion;Magnesium, ion (mg(2+));Mg(2+);Mg2+PW_C000423Mg2+86822742681647627272681158191888322936399833992211167461483491529431764142124102411592942233126293373745403147749148695449745652531045329111535611253761035906147593415160381556094161625016664841786594164688116069791997170205719420672272137233211725021473102167313198747322211763132118432101231222512324249125132881258122612729290152752851533730877137133772363297793733678393334784173357848911578522331785363567857413080020368800451848004837280623118806541358086515809652538184151938323839490027108596223110559390115687398119974406120070122120247382120702407120981408121181124121265429121319419121924125122086405122408422122759120122921399123307119123546374123835464123889455124477136124637376124978375125447297125598484125669479125777481125921482125947299125973495126000490126243478126553491126753300127125389127164501127380502127407388127451507127804209128125508128347395414Adenosine triphosphateHMDB0000538Adenosine triphosphate (ATP) is a nucleotide consisting of a purine base (adenine) attached to the first carbon atom of ribose (a pentose sugar). Three phosphate groups are esterified at the fifth carbon atom of the ribose. ATP is incorporated into nucleic acids by polymerases in the processes of DNA replication and transcription. ATP contributes to cellular energy charge and participates in overall energy balance, maintaining cellular homeostasis. ATP can act as an extracellular signaling molecule via interactions with specific purinergic receptors to mediate a wide variety of processes as diverse as neurotransmission, inflammation, apoptosis, and bone remodelling. Extracellular ATP and its metabolite adenosine have also been shown to exert a variety of effects on nearly every cell type in human skin, and ATP seems to play a direct role in triggering skin inflammatory, regenerative, and fibrotic responses to mechanical injury, an indirect role in melanocyte proliferation and apoptosis, and a complex role in Langerhans cell-directed adaptive immunity. During exercise, intracellular homeostasis depends on the matching of adenosine triphosphate (ATP) supply and ATP demand. Metabolites play a useful role in communicating the extent of ATP demand to the metabolic supply pathways. Effects as different as proliferation or differentiation, chemotaxis, release of cytokines or lysosomal constituents, and generation of reactive oxygen or nitrogen species are elicited upon stimulation of blood cells with extracellular ATP. The increased concentration of adenosine triphosphate (ATP) in erythrocytes from patients with chronic renal failure (CRF) has been observed in many studies but the mechanism leading to these abnormalities still is controversial. (PMID: 15490415, 15129319, 14707763, 14696970, 11157473).56-65-5C00002595715422ATP5742DB00171NC1=NC=NC2=C1N=CN2[C@@H]1O[C@H](COP(O)(=O)OP(O)(=O)OP(O)(O)=O)[C@@H](O)[C@H]1OC10H16N5O13P3InChI=1S/C10H16N5O13P3/c11-8-5-9(13-2-12-8)15(3-14-5)10-7(17)6(16)4(26-10)1-25-30(21,22)28-31(23,24)27-29(18,19)20/h2-4,6-7,10,16-17H,1H2,(H,21,22)(H,23,24)(H2,11,12,13)(H2,18,19,20)/t4-,6-,7-,10-/m1/s1ZKHQWZAMYRWXGA-KQYNXXCUSA-N({[({[(2R,3S,4R,5R)-5-(6-amino-9H-purin-9-yl)-3,4-dihydroxyoxolan-2-yl]methoxy}(hydroxy)phosphoryl)oxy](hydroxy)phosphoryl}oxy)phosphonic acid507.181506.995745159-2.057adenosine triphosphate0-3FDB0218135'-(tetrahydrogen triphosphate) adenosine;5'-atp;Atp;Adenosine 5'-triphosphate;Adenosine 5'-triphosphorate;Adenosine 5'-triphosphoric acid;Adenosine triphosphate;Adenylpyrophosphorate;Adenylpyrophosphoric acid;Adephos;Adetol;Adynol;Atipi;Atriphos;Cardenosine;Fosfobion;Glucobasin;Myotriphos;Phosphobion;Striadyne;Triadenyl;Triphosphaden;Triphosphoric acid adenosine ester;Adenosine-5'-triphosphate;H4atp;Adenosine triphosphoric acid;Adenosine-5'-triphosphoric acidPW_C000414ATP9221460826616414224781373332799593439976321051821121021464921561421605824055924342727264628122930296631637236166136175143992344743147689148645450328950352651557520597521510052501045291101531311153461125390103540611754301185443120554212955561325569133560313556211085846143585414658761075897147592415160481556109161623016664931786839188687016069761997157205718420672092107225213722921172981987302216739021774082187432163748122274991908186225118472771190317012010281120391641217828512578226126912901326422315327308423263154262132242694318770282537721813477233329774683337763233678037332780413507816812878214351782403537841133578494115788501307886533178919334800283688004618480674119856291948261241132349411328238811628010911991412211999240612015440712024538212036241212124642912139212312139743312147140812197441012206512512207938312208340512240242212244443512291939912300944612381646412395144712395646812402937412452744412461613612463039812463437612494347212497237512501147012530429712537147912539229912551548112559548412612348512622030012623449512624047812654749112659649912691350112712338912773151612778139512779639012780120912811950812816751741cAMPHMDB0000058cAMP or cyclic AMP is an adenine nucleotide containing one phosphate group which is esterified to both the 3'- and 5'-positions of the sugar moiety. It is a second messenger and a key intracellular regulator, functioning as a mediator of activity for a number of hormones, including epinephrine, glucagon, and ACTH. cAMP is synthesized from ATP by adenylate cyclase. Adenylate cyclase is located at the cell membranes. Adenylate cyclase is activated by the hormones glucagon and adrenaline and by G protein. Liver adenylate cyclase responds more strongly to glucagon, and muscle adenylate cyclase responds more strongly to adrenaline. cAMP decomposition into AMP is catalyzed by the enzyme phosphodiesterase.60-92-4C00575607617489CAMP5851DB02527NC1=NC=NC2=C1N=CN2[C@@H]1O[C@@H]2COP(O)(=O)O[C@H]2[C@H]1OC10H12N5O6PInChI=1S/C10H12N5O6P/c11-8-5-9(13-2-12-8)15(3-14-5)10-6(16)7-4(20-10)1-19-22(17,18)21-7/h2-4,6-7,10,16H,1H2,(H,17,18)(H2,11,12,13)/t4-,6-,7-,10-/m1/s1IVOMOUWHDPKRLL-KQYNXXCUSA-N(4aR,6R,7R,7aS)-6-(6-amino-9H-purin-9-yl)-2,7-dihydroxy-hexahydro-2lambda5-furo[3,2-d][1,3,2]dioxaphosphinin-2-one329.2059329.052519653-1.963(4aR,6R,7R,7aS)-6-(6-aminopurin-9-yl)-2,7-dihydroxy-tetrahydro-4H-2lambda5-furo[3,2-d][1,3,2]dioxaphosphinin-2-one0-1FDB001497Cyclic AMP;3'5'-cyclic amp;6-(6-amino-9h-purin-9-yl)tetrahydro-4h-furo[3,2-d][1,3,2]dioxaphosphinine-2,7-diol 2-oxide;Acrasin;Adenosine 3',5'-cyclic monophosphate;Adenosine 3',5'-cyclic phosphate;Adenosine 3',5'-cyclophosphate;Adenosine 3',5'-monophosphate;Adenosine 3,5'-cyclic monophosphorate;Adenosine 3,5'-cyclic monophosphoric acid;Adenosine cyclic monophosphate;Adenosine cyclic-monophosphate;Adenosine-cyclic-phosphate;Adenosine-cyclic-phosphoric-acid;Cyclic 3',5'-amp;Cyclic 3',5'-adenylate;Cyclic 3',5'-adenylic acid;Cyclic amp;Cyclic adenosine 3',5'-phosphate;Camp;Adenosine 3',5'-phosphate;Adenosine-3',5'-cyclic-monophosphate;Cyclic adenylic acid;Adenosine 3',5'-cyclic monophosphoric acid;Adenosine 3',5'-cyclic phosphoric acid;Adenosine 3',5'-phosphoric acid;Adenosine-3',5'-cyclic-monophosphoric acid;Cyclic adenylatePW_C000041cAMP2671647529353910002119168417414118301987870911180044184121904122124457135170PyrophosphateHMDB0000250The anion, the salts, and the esters of pyrophosphoric acid are called pyrophosphates. The pyrophosphate anion is abbreviated PPi and is formed by the hydrolysis of ATP into AMP in cells. This hydrolysis is called pyrophosphorolysis. The pyrophosphate anion has the structure P2O74-, and is an acid anhydride of phosphate. It is unstable in aqueous solution and rapidly hydrolyzes into inorganic phosphate. Pyrophosphate is an osteotoxin (arrests bone development) and an arthritogen (promotes arthritis). It is also a metabotoxin (an endogenously produced metabolite that causes adverse health affects at chronically high levels). Chronically high levels of pyrophosphate are associated with hypophosphatasia. Hypophosphatasia (also called deficiency of alkaline phosphatase or phosphoethanolaminuria) is a rare, and sometimes fatal, metabolic bone disease. Hypophosphatasia is associated with a molecular defect in the gene encoding tissue non-specific alkaline phosphatase (TNSALP). TNSALP is an enzyme that is tethered to the outer surface of osteoblasts and chondrocytes. TNSALP hydrolyzes several substances, including inorganic pyrophosphate (PPi) and pyridoxal 5'-phosphate (PLP), a major form of vitamin B6. When TSNALP is low, inorganic pyrophosphate (PPi) accumulates outside of cells and inhibits the formation of hydroxyapatite, one of the main components of bone, causing rickets in infants and children and osteomalacia (soft bones) in adults. Vitamin B6 must be dephosphorylated by TNSALP before it can cross the cell membrane. Vitamin B6 deficiency in the brain impairs synthesis of neurotransmitters which can cause seizures. In some cases, a build-up of calcium pyrophosphate dihydrate crystals in the joints can cause pseudogout.14000-31-8C0001364410218361PPI559142DB04160[O-]P([O-])(=O)OP([O-])([O-])=OO7P2InChI=1S/H4O7P2/c1-8(2,3)7-9(4,5)6/h(H2,1,2,3)(H2,4,5,6)/p-4XPPKVPWEQAFLFU-UHFFFAOYSA-J(phosphonooxy)phosphonic acid173.9433173.9119253784pyrophosphoric acid0-3FDB021918(4-)diphosphoric acid ion;(p2o74-)diphosphate;Diphosphate;Diphosphoric acid;Ppi;Pyrometaphosphate;Pyrophosphate;Pyrophosphate tetraanion;Pyrophosphate(4-) ion;[o3popo3](4-);Diphosphat;P2o7(4-);Pyrophosphat;Pyrophosphate ion;Phosphonato phosphoric acid;Pyrophosphoric acid;Pyrophosphoric acid ionPW_C000170Ppi1223546384292373532882221217316204924105928152941751448685450348952521045294101540911754241035433118545812055481115559132558413356061355655108587910762391666978199707318871341637272160731219873182138275151828321011869161120022221204116412315225123232491251228812579226126952901521930615375183476017425613154269731877235329773171287763533678416335789283317915311279950134799581308004737280417170856301947863849481412594819382986782231106343911132703951132753891155271361155323991199341221200171241200324061203304101209364071212614291213411211214863831224074221229854441235021191238314641240443981249773751253242971253952991254104791255974841256564851258764811265524911268692051269353881269505011273372061281245081104PhosphateHMDB0001429Phosphate is a salt of phosphoric acid. In organic chemistry, a phosphate, or organophosphate, is an ester of phosphoric acid. Organic phosphates are important in biochemistry, biogeochemistry and ecology. Phosphate (Pi) is an essential component of life. In biological systems, phosphorus is found as a free phosphate ion in solution and is called inorganic phosphate, to distinguish it from phosphates bound in various phosphate esters. Inorganic phosphate is generally denoted Pi and at physiological (neutral) pH primarily consists of a mixture of HPO<sup>2-</sup><sub>4</sub> and H<sub>2</sub>PO<sup>-</sup><sub>4</sub> ions. phosphates are most commonly found in the form of adenosine phosphates, (AMP, ADP and ATP) and in DNA and RNA and can be released by the hydrolysis of ATP or ADP. Similar reactions exist for the other nucleoside diphosphates and triphosphates. Phosphoanhydride bonds in ADP and ATP, or other nucleoside diphosphates and triphosphates, contain high amounts of energy which give them their vital role in all living organisms. Phosphate must be actively transported into cells against its electrochemical gradient. In vertebrates, two unrelated families of Na+-dependent Pi transporters carry out this task. Remarkably, the two families transport different Pi species: whereas type II Na+/Pi cotransporters (SCL34) prefer divalent HPO4(2), type III Na+/Pi cotransporters (SLC20) transport monovalent H2PO4. The SCL34 family comprises both electrogenic and electroneutral members that are expressed in various epithelia and other polarized cells. Through regulated activity in apical membranes of the gut and kidney, they maintain body Pi homeostasis, and in salivary and mammary glands, liver, and testes they play a role in modulating the Pi content of luminal fluids. Phosphate levels in the blood play an important role in hormone signaling and in bone homeostasis. In classical endocrine regulation, low serum phosphate induces the renal production of the seco-steroid hormone 1,25-dihydroxyvitamin D3 (1,25(OH)2D3).This active metabolite of vitamin D acts to restore circulating mineral (i.e. phosphate and calcium) levels by increasing absorption in the intestine, reabsorption in the kidney, and mobilization of calcium and phosphate from bone. Thus, chronic renal failure is associated with hyperparathyroidism, which in turn contributes to osteomalacia (softening of the bones). Another complication of chronic renal failure is hyperphosphatemia (low levels of phosphate in the blood). Hyperphosphatemia (excess levels of phosphate in the blood) is a prevalent condition in kidney dialysis patients and is associated with increased risk of mortality. Hypophosphatemia (hungry bone syndrome) has been associated to postoperative electrolyte aberrations and after parathyroidectomy. (PMID: 17581921, 11169009, 11039261, 9159312, 17625581)Fibroblast growth factor 23 (FGF-23) has recently been recognized as a key mediator of phosphate homeostasis, its most notable effect being promotion of phosphate excretion. FGF-23 was discovered to be involved in diseases such as autosomal dominant hypophosphatemic rickets, X-linked hypophosphatemia, and tumor-induced osteomalacia in which phosphate wasting was coupled to inappropriately low levels of 1,25(OH)2D3. FGF-23 is regulated by dietary phosphate in humans. In particular it was found that phosphate restriction decreased FGF-23, and phosphate loading increased FGF-23.14265-44-2C00009106118367CPD-85871032[O-]P([O-])([O-])=OO4PInChI=1S/H3O4P/c1-5(2,3)4/h(H3,1,2,3,4)/p-3NBIIXXVUZAFLBC-UHFFFAOYSA-Kphosphoric acid94.971494.953423phosphoric acid0-2DBMET00532FDB022617Nfb orthophosphate;O-phosphoric acid;Ortho-phosphate;Orthophosphate (po43-);Orthophosphate(3-);Phosphate;Phosphate (po43-);Phosphate anion(3-);Phosphate ion (po43-);Phosphate ion(3-);Phosphate trianion;Phosphate(3-);Phosphoric acid ion(3-);Pi;[po4](3-);Orthophosphate;Phosphate ion;Po4(3-);Phosphoric acid;Orthophosphoric acid;Phosphoric acid ionPW_C001104Pi24484881458181883129803176314176749250010272947273746312929316672363661385123424922447531503127515875207975216100531711153511125381103544712055431295573133560513556251085693658481435855146591114759411516040155610016162941076487178669110167141176842188688916071612057189206721221173061987389210740221274361637475222819622582582271011824110134257117481321176111511773213119041701192716412014281127282901326322334819174225530442350315424353184369232277018253771942937721713477940336779661307804833278057329782453537866933180022368892793089383138394796384110558390110640391113235941158453981162061091199824061200691221206994071210571241212161251212684291213521211214091231214233821218524051233041191236211181237861361238384641239684471239813991244053761249484721253624791254462971257744811259542991262214781265943001266042981267234841269045011274133881277832091281663951281775131283153892055Follicle-stimulating hormone receptorP23945Receptor for follicle-stimulating hormone. The activity of this receptor is mediated by G proteins which activate adenylate cyclaseHMDBP02692FSHR2p21-p16AK29256214189141601Guanine nucleotide-binding protein G(s) subunit alpha isoforms shortP63092Guanine nucleotide-binding proteins (G proteins) are involved as modulators or transducers in various transmembrane signaling systems. The G(s) protein is involved in hormonal regulation of adenylate cyclase:it activates the cyclase in response to beta-adrenergic stimuliHMDBP01781GNAS20q13.3M14631138102413914415873501185516281355885993618GβP62873Guanine nucleotide-binding protein G(I)/G(S)/G(T) subunit beta-1 is a guanine nucleotide-binding protein (G protein) are involved as a modulator or transducer in various transmembrane signaling systems. The beta and gamma chains are required for the GTPase activity, for replacement of GDP by GTP, and for G protein- effector interactionHMDBP08398GNB11p36.33BC0058881381224138144157735010855161869324170006308135590599136006608136181661361876653625Guanine nucleotide-binding protein G(I)/G(S)/G(O) subunit gamma-12Q9UBI6Guanine nucleotide-binding proteins (G proteins) are involved as a modulator or transducer in various transmembrane signaling systems. The beta and gamma chains are required for the GTPase activity, for replacement of GDP by GTP, and for G protein- effector interactionHMDBP08405GNG121p31.3AF1881811381124137144156735009855776Glycoprotein hormones alpha chainP0121514190154192280766235777Follitropin subunit betaP012251419115419328076723864Adenylate cyclase type 2Q08462This is a membrane-bound, calmodulin-insensitive adenylyl cyclase.
HMDBP00921ADCY25p15.3AB02898314.6.1.138292417314136381741315cAMP-dependent protein kinase catalytic subunit betaP22694Mediates cAMP-dependent signaling triggered by receptor binding to GPCRs. PKA activation regulates diverse cellular processes such as cell proliferation, the cell cycle, differentiation and regulation of microtubule dynamics, chromatin condensation and decondensation, nuclear envelope disassembly and reassembly, as well as regulation of intracellular transport mechanisms and ion fluxHMDBP01415PRKACB1p36.1AY92736612.7.11.1138372385323419985766Ethylene-responsive transcription factor ESR1Q9SAD4ESR11385223419823900Serine/threonine-protein phosphatase PP1-alpha catalytic subunitP62136Protein phosphatase that associates with over 200 regulatory proteins to form highly specific holoenzymes which dephosphorylate hundreds of biological targets. Protein phosphatase 1 (PP1) is essential for cell division, and participates in the regulation of glycogen metabolism, muscle contractility and protein synthesis. Involved in regulation of ionic conductances and long-term synaptic plasticity. May play an important role in dephosphorylating substrates such as the postsynaptic density-associated Ca(2+)/calmodulin dependent protein kinase II. Component of the PTW/PP1 phosphatase complex, which plays a role in the control of chromatin structure and cell cycle progression during the transition from mitosis into interphase. Regulates NEK2 function in terms of kinase activity and centrosome number and splitting, both in the presence and absence of radiation-induced DNA damage. Regulator of neural tube and optic fissure closure, and enteric neural crest cell (ENCCs) migration during development.
HMDBP08684PPP1CA11q13J0475913.1.3.16385423385828026781802Cyclic AMP-responsive element-binding protein 1P16220This protein binds the cAMP response element (CRE), a sequence present in many viral and cellular promoters. CREB stimulates transcription on binding to the CRE. Transcription activation is enhanced by the TORC coactivators which act independently of Ser-133 phosphorylation. Implicated in synchronization of circadian rhythmicityHMDBP02133CREB12q34X55545138572398023115974273627Guanine nucleotide-binding protein G(T) subunit gamma-T1P63211Guanine nucleotide-binding proteins (G proteins) are involved as a modulator or transducer in various transmembrane signaling systems. The beta and gamma chains are required for the GTPase activity, for replacement of GDP by GTP, and for G protein- effector interactionHMDBP08407GNGT17q21.3BC02936715160813558959913622214997Follicle-stimulating hormone receptor bound to G protein complex1PW_P0009971134205511135160111136361811137362514169361120Guanine nucleotide-binding protein gamma-beta1PW_P000120137362711383618146324121Guanine nucleotide-binding protein alpha1PW_P0001211391601146420998Follicle-stimulating hormone receptor1PW_P0009981138205511000Glycoprotein hormones alpha chain1PW_P001000114057761122Adenylate cyclase type 21PW_P000122140864157423246524992Adenylate cyclase bound to G(s) subunit alpha1PW_P0009921121864111221601141498614154231922cAMP-dependent protein kinase catalytic subunit beta1PW_P0009221047131513744231923Ethylene-responsive transcription factor ESR11PW_P000923104857661924Serine/threonine-protein phosphatase PP1-alpha catalytic subunit1PW_P000924104939001925Cyclic AMP-responsive element-binding protein 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215 735 240 735 210 5true1813136M1415 577.5 L1415 627.5 L1465 577.5 z10true1813137M1935 397 C1931 470 2020.5 475.5 2057.5 475.5 149false1813138M1740 585 C1837 585 1956.5 472.5 2051.5 478.5 149false1813139M1720 497.5 L1720 547.5 L1770 497.5 z10true1813140M1720 497.5 L1720 547.5 L1770 497.5 z10true1813141M2230 402 C2179 479 2114.5 474.5 2045.5 476.5 149false18trueM 1914.838507077443 416.1981988034234 L 1910 402 L 1900.123252686975 413.2893694471681false13143M2155 595 C2155 527 2208 403 2350 402 5false1813144M2595 595 C2597 523 2496 399 2350 402 5false18trueM 25.946855044164835 13.26155629629604 L 11 12 L 17.380887721185843 25.575134323078345false13145M2461.5 540 C2460.5 480 2461 402 2350 402 5false18trueM 25.946855044164835 13.26155629629604 L 11 12 L 17.380887721185843 25.575134323078345false13212M2595 785 C2595 815 2600 890 2600 920 149false18trueM 2287.5 877.0096189432334 L 2295 890 L 2302.5 877.0096189432334false13213M2695 1165 C2610 1166 2600 1030 2600 1000 149false18trueM 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1663.7224853612868 1412.7967934901462 L 1655 1425 L 1669.9295295258044 1426.4522906520851false13816M1949 1456.5 C1979 1456.5 908 1170 938 1170 5true1813817M1680 1240 C1519 1206 1113 1112 1088 1170 5false18trueM 1480.740270679691 1284.6540433723294 L 1495 1280 L 1483.8396155490937 1269.9777338436872false13818M1795 1485 C1765 1485 1059.5 1218 1088 1170 5false1813819M1663 1456.5 C1633 1456.5 2502 1155 2472 1155 5true18trueM 25.946855044164835 13.26155629629604 L 11 12 L 17.380887721185843 25.575134323078345false13820M1820 1350 C1850 1350 2345 1163 2390 1200 5false1813821M1710 1490 C1842 1436 2420 1232 2390 1200 5false18trueM 1670.1834613041385 1578.13981288194 L 1661 1590 L 1675.862953989946 1592.0230172250253false13822M1707 1456.5 C1737 1456.5 908 1170 938 1170 5true1813823M1670 1350 C1495 1294 1060 1177 1013 1205 5false18trueM 1522.9903810567666 1467.5 L 1510 1475 L 1522.9903810567666 1482.5false13824M1560 1490 C1530 1490 962 1247 1013 1205 5false1825603482129148186856713124Left8187856813125Right493377013127Left494377113128Right495377213130Right496377313131Right2561348212714497377613137Left498377213138Left499377713141Right25623482128148188857513143Left8189857613144Right8190857713145Right2595199237772573348197088206857613212Left8207859913213Right522381213215Right26061816381326963481990238427887413813Right629401213814Left630401313815Right26941834381226973481996238428887413816Left631401213817Left632401313818Right26951841401426983482000238429887513819Right633401513820Left634401613821Right26961845381226993481999238430887513822Left635401513823Left636401613824Right26971844401476668834814true8015516regular443377413133Left444377513135Right771692348217false3105102016regular727859913251Left772693348217false3105112016regular728859913252Left773694348217false3110127016regular729859913253Left80115195160515260525360535460545560555660566008785015701.51.50214327267185770145413400.20.2055430001764582M21 392 C21 342 71 292 121 292 C1058 292 2275 292 3212 292 C3262 292 3312 342 3312 392 C3312 870 3312 1491 3312 1969 C3312 2019 3262 2069 3212 2069 C2275 2069 1058 2069 121 2069 C71 2069 21 2019 21 1969 C21 1491 21 870 21 392 1true63291.01777.052415Extracellular Space1565195201.01.01601552515Intracellular Space1600645201.01.01601554015Regulatory Subunits2405895201.01.01601554115Regulatory Subunits26401035201.01.01601555315Nucleus10051580201.01.01601561415Activation2030430201.01.01601561515Activation320200201.01.01601561615Activation2490830201.01.01601561715Activation29051125201.01.01601561815Regulatory Subunit8151150201.01.01601544948515Transcription17981535201.01.01601518018511431758144511212049172039#FFF9C94604599