1.02011-09-21 00:24:54 UTC2015-07-21 06:57:31 UTCFDB023114Argininic acidTissue accumulation of argininic acid (AA) occurs in hyperargininemia. Hyperargininemia, an inborn error of the urea cycle, is caused by a severe deficiency of liver arginase, resulting in elevated tissue levels of arginine (Arg) and other guanidino compounds (GC). Affected patients present a neurological syndrome consisting of a variable degree of mental retardation, epilepsy and progressive spasticity whose pathophysiology is far from understood. Guanidino compounds accumulate in other pathological conditions such as uremia and epilepsy and some evidence supports the hypothesis that these compounds contribute to the neurological dysfunction characteristic of these diseases. The increase of these compounds occurs by blockage of the arginase reaction, activating secondary biochemical pathways. Thus, Arg is converted to α-keto-δ-guanidinovaleric acid by transamination, and this compound forms AA by hydrogenation. (PMID 12615083) [HMDB]Arginic acidArgininic acidC6H13N3O3175.1857175.095691297(2S)-5-carbamimidamido-2-hydroxypentanoic acidargininic acid157-07-3NC(=N)NCCC[C@H](O)C(O)=OInChI=1S/C6H13N3O3/c7-6(8)9-3-1-2-4(10)5(11)12/h4,10H,1-3H2,(H,11,12)(H4,7,8,9)/t4-/m0/s1BMFMQGXDDJALKQ-BYPYZUCNSA-N belongs to the class of organic compounds known as delta amino acids and derivatives. Delta amino acids and derivatives are compounds containing a carboxylic acid group and an amino group at the C5 carbon atom.Delta amino acids and derivativesOrganic compoundsOrganic acids and derivativesCarboxylic acids and derivativesAmino acids, peptides, and analoguesAliphatic acyclic compoundsAlpha hydroxy acids and derivativesCarbonyl compoundsCarboximidamidesCarboxylic acidsFatty acids and conjugatesGuanidinesHydrocarbon derivativesIminesMonocarboxylic acids and derivativesMonosaccharidesOrganic oxidesOrganopnictogen compoundsSecondary alcoholsAlcoholAliphatic acyclic compoundAlpha-hydroxy acidCarbonyl groupCarboximidamideCarboxylic acidDelta amino acid or derivativesFatty acidGuanidineHydrocarbon derivativeHydroxy acidImineMonocarboxylic acid or derivativesMonosaccharideOrganic nitrogen compoundOrganic oxideOrganic oxygen compoundOrganonitrogen compoundOrganooxygen compoundOrganopnictogen compoundSecondary alcoholSolidlogp-1.70logs-1.87solubility2.35e+00 g/llogp-3pka_strongest_acidic3.67pka_strongest_basic12.37iupac(2S)-5-carbamimidamido-2-hydroxypentanoic acidaverage_mass175.1857mono_mass175.095691297smilesNC(=N)NCCC[C@H](O)C(O)=OformulaC6H13N3O3inchiInChI=1S/C6H13N3O3/c7-6(8)9-3-1-2-4(10)5(11)12/h4,10H,1-3H2,(H,11,12)(H4,7,8,9)/t4-/m0/s1inchikeyBMFMQGXDDJALKQ-BYPYZUCNSA-Npolar_surface_area119.43refractivity52.27polarizability17.5rotatable_bond_count5acceptor_count6donor_count5physiological_charge0formal_charge0Specdb::MsIr1883Specdb::MsIr1884Specdb::MsIr1885Specdb::CMs24185Specdb::CMs38532Specdb::CMs169025Specdb::MsMs68415Specdb::MsMs68416Specdb::MsMs68417Specdb::MsMs126438Specdb::MsMs126439Specdb::MsMs126440Specdb::MsMs2295057Specdb::MsMs2295058Specdb::MsMs2295059Specdb::MsMs2640244Specdb::MsMs2640245Specdb::MsMs2640246Specdb::NmrOneD129318Specdb::NmrOneD129319Specdb::NmrOneD129320Specdb::NmrOneD129321Specdb::NmrOneD129322Specdb::NmrOneD129323Specdb::NmrOneD129324Specdb::NmrOneD129325Specdb::NmrOneD129326Specdb::NmrOneD129327Specdb::NmrOneD129328Specdb::NmrOneD129329Specdb::NmrOneD129330Specdb::NmrOneD129331Specdb::NmrOneD129332Specdb::NmrOneD129333Specdb::NmrOneD129334Specdb::NmrOneD129335Specdb::NmrOneD129336Specdb::NmrOneD129337HMDB03148#<Reference:0x00005556760d48b0>#<Reference:0x00005556760d4428>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 scrofa9823