1.02011-09-21 00:28:17 UTC2015-07-21 06:57:38 UTCFDB023336Biotinyl-5'-AMP5'-biotinyl-AMP (B-AMP) is the active form of biotin in mammals. In human cells, biotin is essential to maintain metabolic homeostasis and as regulator of gene expression. The vitamin biotin plays an essential role in gluconeogenesis, fatty acid synthesis, and carbohydrate metabolism because of its role as cofactor of five carboxylases; pyruvate carboxylase (PC), propionyl-CoA carboxylase (PCC), methylcrotonyl-CoA carboxylase, and two forms of acetyl-CoA carboxylase (ACC-1 and ACC-2).
Carboxylase biotinylation is catalyzed by the enzyme holocarboxylase synthetase (HCS) through a reaction that involves the transformation of biotin into B-AMP and its subsequent attachment to a specific lysine residue in the carboxylases. B-AMP is also required to activate a signal transduction cascade that includes a soluble guanylate cyclase (sGC) and cGMP-dependent protein kinase (PKG). The regulatory role of biotin in the biotin cycle seems to be limited to the expression of proteins involved in the transport and utilization of exogenous vitamin while having no effect on biotinidase mRNA levels, enzyme responsible for biotin recycling during carboxylase turnover.
Multiple carboxylase deficiency (MCD) is a life-threatening disease characterized by the lack of carboxylase activities because of deficiency of HCS activity. (PMID: 15905112, 11959985) [HMDB](+)-Biotinyl 5'-adenylate[3aS-(3aa,4b,6aa)]-5'-Adenylic acid monoanhydride with hexahydro-2-oxo-1H-thieno[3,4-d]imidazole-4-pentanoate[3aS-(3aa,4b,6aa)]-5'-Adenylic acid monoanhydride with hexahydro-2-oxo-1H-thieno[3,4-d]imidazole-4-pentanoic acid5'-Adenylic acid anhydride with biotin5'-Adenylic acid monoanhydride with biotinB-AMPbeta-AMPbio-5-AMPBiotin anhydride with 5'-adenylic acidBiotinoyl 5'-adenylateBiotinyl 5'-AMPC20H28N7O9PS573.517573.140682731{[(2R,3S,4R,5R)-5-(6-amino-9H-purin-9-yl)-3,4-dihydroxyoxolan-2-yl]methoxy}({5-[(4S)-2-oxo-hexahydro-1H-thieno[3,4-d]imidazolidin-4-yl]pentanoyl}oxy)phosphinic acid[(2R,3S,4R,5R)-5-(6-aminopurin-9-yl)-3,4-dihydroxyoxolan-2-yl]methoxy({5-[(4S)-2-oxo-hexahydrothieno[3,4-d]imidazolidin-4-yl]pentanoyl}oxy)phosphinic acid4130-20-5NC1=NC=NC2=C1N=CN2[C@@H]1O[C@H](COP(O)(=O)OC(=O)CCCC[C@@H]2SCC3NC(=O)NC23)[C@@H](O)[C@H]1OInChI=1S/C20H28N7O9PS/c21-17-14-18(23-7-22-17)27(8-24-14)19-16(30)15(29)10(35-19)5-34-37(32,33)36-12(28)4-2-1-3-11-13-9(6-38-11)25-20(31)26-13/h7-11,13,15-16,19,29-30H,1-6H2,(H,32,33)(H2,21,22,23)(H2,25,26,31)/t9?,10-,11+,13?,15-,16-,19-/m1/s1UTQCSTJVMLODHM-CFYKWCKVSA-N belongs to the class of organic compounds known as 5'-acylphosphoadenosines. These are ribonucleoside derivatives containing an adenoside moiety, where the phosphate group is acylated.5'-acylphosphoadenosinesOrganic compoundsNucleosides, nucleotides, and analoguesPurine nucleotidesPurine ribonucleotidesAromatic heteropolycyclic compounds1,2-diols6-aminopurinesAcyl phosphatesAmino acids and derivativesAminopyrimidines and derivativesAzacyclic compoundsBiotin and derivativesCarbonyl compoundsCarboxylic acid saltsDialkylthioethersGlycosylaminesHeteroaromatic compoundsHydrocarbon derivativesImidazolidinonesImidolactamsMonoalkyl phosphatesMonocarboxylic acids and derivativesMonosaccharide phosphatesN-substituted imidazolesOrganic oxidesOrganic saltsOrganopnictogen compoundsOxacyclic compoundsPentose phosphatesPrimary aminesSecondary alcoholsTetrahydrofuransThienoimidazolidinesThiolanesThiophenesUreas1,2-diol5'-acylphosphoadenosine6-aminopurineAcyl phosphateAlcoholAlkyl phosphateAmineAmino acid or derivativesAminopyrimidineAromatic heteropolycyclic compoundAzacycleAzoleBiotinBiotin_derivativeCarbonic acid derivativeCarbonyl groupCarboxylic acid derivativeCarboxylic acid saltDialkylthioetherGlycosyl compoundHeteroaromatic compoundHydrocarbon derivativeImidazoleImidazolidineImidazolidinoneImidazopyrimidineImidolactamMonoalkyl phosphateMonocarboxylic acid or derivativesMonosaccharideMonosaccharide phosphateN-glycosyl compoundN-substituted imidazoleOrganic nitrogen compoundOrganic oxideOrganic oxygen compoundOrganic phosphoric acid derivativeOrganic saltOrganoheterocyclic compoundOrganonitrogen compoundOrganooxygen compoundOrganopnictogen compoundOxacyclePentose monosaccharidePentose phosphatePentose-5-phosphatePhosphoric acid esterPrimary aminePurinePyrimidineSecondary alcoholTetrahydrofuranThienoimidazolidineThioetherThiolaneThiopheneUreaSolidlogp-1.24logs-2.41solubility2.24e+00 g/llogp-4.1pka_strongest_acidic0.83pka_strongest_basic4.99iupac{[(2R,3S,4R,5R)-5-(6-amino-9H-purin-9-yl)-3,4-dihydroxyoxolan-2-yl]methoxy}({5-[(4S)-2-oxo-hexahydro-1H-thieno[3,4-d]imidazolidin-4-yl]pentanoyl}oxy)phosphinic acidaverage_mass573.517mono_mass573.140682731smilesNC1=NC=NC2=C1N=CN2[C@@H]1O[C@H](COP(O)(=O)OC(=O)CCCC[C@@H]2SCC3NC(=O)NC23)[C@@H](O)[C@H]1OformulaC20H28N7O9PSinchiInChI=1S/C20H28N7O9PS/c21-17-14-18(23-7-22-17)27(8-24-14)19-16(30)15(29)10(35-19)5-34-37(32,33)36-12(28)4-2-1-3-11-13-9(6-38-11)25-20(31)26-13/h7-11,13,15-16,19,29-30H,1-6H2,(H,32,33)(H2,21,22,23)(H2,25,26,31)/t9?,10-,11+,13?,15-,16-,19-/m1/s1inchikeyUTQCSTJVMLODHM-CFYKWCKVSA-Npolar_surface_area233.27refractivity130.63polarizability53.92rotatable_bond_count11acceptor_count11donor_count6physiological_charge-1formal_charge0Biotin MetabolismSMP00066map00780Specdb::MsMs27284Specdb::MsMs27285Specdb::MsMs27286Specdb::MsMs33842Specdb::MsMs33843Specdb::MsMs33844Specdb::MsMs2709473Specdb::MsMs2709474Specdb::MsMs2709475Specdb::MsMs2995838Specdb::MsMs2995839Specdb::MsMs2995840Specdb::NmrOneD47902Specdb::NmrOneD47903Specdb::NmrOneD47904Specdb::NmrOneD47905Specdb::NmrOneD47906Specdb::NmrOneD47907Specdb::NmrOneD47908Specdb::NmrOneD47909Specdb::NmrOneD47910Specdb::NmrOneD47911Specdb::NmrOneD47912Specdb::NmrOneD47913Specdb::NmrOneD47914Specdb::NmrOneD47915Specdb::NmrOneD47916Specdb::NmrOneD47917Specdb::NmrOneD47918Specdb::NmrOneD47919Specdb::NmrOneD47920Specdb::NmrOneD47921Specdb::CMs26041Specdb::CMs38732Specdb::CMs282399Specdb::CMs371015Specdb::CMs371016Specdb::CMs371017Specdb::CMs371018Specdb::CMs371019Specdb::CMs371020Specdb::CMs371021Specdb::CMs371022Specdb::CMs371023Specdb::CMs371024Specdb::CMs371025Specdb::CMs371026Specdb::CMs371027Specdb::CMs371028Specdb::CMs371029Specdb::CMs371030Specdb::CMs371031Specdb::CMs371032Specdb::CMs371033Specdb::CMs371034Specdb::CMs371035Specdb::CMs371036HMDB04220#<Reference:0x00005556759dcd78>#<Reference:0x00005556759dcb70>AnatidaeType 1specificAnatidae8830BeefaloType 1specificBos taurus X Bison bison297284BisonType 1specificBison bison9901BuffaloType 1specificBubalus bubalis89462Cattle (Beef, Veal)Type 1specificBos taurus9913ChickenType 1specificGallus gallus9031Columbidae (Dove, Pigeon)Type 1specificColumbidae8930DeerType 1specificCervidae9850Domestic goatType 1specificCapra aegagrus hircus9925Domestic pigType 1specificSus scrofa domestica9825ElkType 1specificCervus canadensis1574408EmuType 1specificDromaius novaehollandiae8790European rabbitType 1specificOryctolagus9984Greylag gooseType 1specificAnser anser8843Guinea henType 1specificNumida meleagris8996HorseType 1specificEquus caballus9796Mallard duckType 1specificAnas platyrhynchos8839Mountain hareType 1specificLepus timidus62621Mule deerType 1specificOdocoileus9871OstrichType 1specificStruthio camelus8801PheasantType 1specificPhasianus colchicus9054QuailType 1specificPhasianidae9005RabbitType 1specificLeporidae9979Rock ptarmiganType 1specificLagopus muta64668Sheep (Mutton, Lamb)Type 1specificOvis aries9940SquabType 1specificColumba8931TurkeyType 1specificMeleagris gallopavo9103Velvet duckType 1specificMelanitta fusca371864Wild boarType 1specificSus scrofa9823Biotin--protein ligaseP50747HLCS