1.0 2011-09-21 00:20:25 UTC 2015-07-21 06:57:23 UTC FDB022841 Isovalerylglucuronide Isovaleryl glucuronide has been identified in the urine of patients with isovaleric acidemia made possible by using gas chromatograph-mass spectrometry (GC-MS). Isovaleryl glucuronide is more likely to be excreted when the amount of urinary 3-hydroxyisovaleric acid excretion is high. Isovaleryl glucuronide is more likely to be excreted when the amount of urinary 3-hydroxyisovaleric acid excretion is high. (PMID: 6547525) Isovaleric academia is caused by mutation in the isovaleryl CoA dehydrogenase gene and is closely related to maple syrup urine disease. It presents in two forms: the acute neonatal form, leading to massive metabolic acidosis from the first days of life and rapid death and a chronic form in which periodic attacks of severe ketoacidosis occur with asymptomatic intervening periods. (OMIM 243500) [HMDB] Isovaleryl-beta-D-glucuronide Isovaleryl-beta-delta-glucuronide C11H18O8 278.2558 278.100167552 (2S,3S,4S,5R,6S)-3,4,5-trihydroxy-6-[(3-methylbutanoyl)oxy]oxane-2-carboxylic acid isovalerylglucuronide 88070-93-3 CC(C)CC(=O)O[C@@H]1O[C@@H]([C@@H](O)[C@H](O)[C@H]1O)C(O)=O InChI=1S/C11H18O8/c1-4(2)3-5(12)18-11-8(15)6(13)7(14)9(19-11)10(16)17/h4,6-9,11,13-15H,3H2,1-2H3,(H,16,17)/t6-,7-,8+,9-,11+/m0/s1 VOJAALAAOYUSCT-ZCLKDUABSA-N belongs to the class of organic compounds known as o-glucuronides. These are glucuronides in which the aglycone is linked to the carbohydrate unit through an O-glycosidic bond. O-glucuronides Organic compounds Organic oxygen compounds Organooxygen compounds Carbohydrates and carbohydrate conjugates Aliphatic heteromonocyclic compounds Acetals Beta hydroxy acids and derivatives Carbonyl compounds Carboxylic acid esters Carboxylic acids Dicarboxylic acids and derivatives Fatty acid esters Hydrocarbon derivatives Monosaccharides Organic oxides Oxacyclic compounds Oxanes Polyols Pyrans Secondary alcohols 1-o-glucuronide Acetal Alcohol Aliphatic heteromonocyclic compound Beta-hydroxy acid Carbonyl group Carboxylic acid Carboxylic acid derivative Carboxylic acid ester Dicarboxylic acid or derivatives Fatty acid ester Fatty acyl Hydrocarbon derivative Hydroxy acid Monosaccharide O-glucuronide Organic oxide Organoheterocyclic compound Oxacycle Oxane Polyol Pyran Secondary alcohol Solid logp -0.98 ALOGPS logs -0.63 ALOGPS solubility 6.58e+01 g/l ALOGPS logp -0.74 ChemAxon pka_strongest_acidic 3.51 ChemAxon pka_strongest_basic -3.7 ChemAxon iupac (2S,3S,4S,5R,6S)-3,4,5-trihydroxy-6-[(3-methylbutanoyl)oxy]oxane-2-carboxylic acid ChemAxon average_mass 278.2558 ChemAxon mono_mass 278.100167552 ChemAxon smiles CC(C)CC(=O)O[C@@H]1O[C@@H]([C@@H](O)[C@H](O)[C@H]1O)C(O)=O ChemAxon formula C11H18O8 ChemAxon inchi InChI=1S/C11H18O8/c1-4(2)3-5(12)18-11-8(15)6(13)7(14)9(19-11)10(16)17/h4,6-9,11,13-15H,3H2,1-2H3,(H,16,17)/t6-,7-,8+,9-,11+/m0/s1 ChemAxon inchikey VOJAALAAOYUSCT-ZCLKDUABSA-N ChemAxon polar_surface_area 133.52 ChemAxon refractivity 58.72 ChemAxon polarizability 25.89 ChemAxon rotatable_bond_count 5 ChemAxon acceptor_count 7 ChemAxon donor_count 4 ChemAxon physiological_charge -1 ChemAxon formal_charge 0 ChemAxon Specdb::CMs 24963 Specdb::CMs 38262 Specdb::CMs 166425 Specdb::MsMs 80166 Specdb::MsMs 80167 Specdb::MsMs 80168 Specdb::MsMs 140955 Specdb::MsMs 140956 Specdb::MsMs 140957 Specdb::MsMs 2780365 Specdb::MsMs 2780366 Specdb::MsMs 2780367 Specdb::MsMs 2925463 Specdb::MsMs 2925464 Specdb::MsMs 2925465 Specdb::NmrOneD 109318 Specdb::NmrOneD 109319 Specdb::NmrOneD 109320 Specdb::NmrOneD 109321 Specdb::NmrOneD 109322 Specdb::NmrOneD 109323 Specdb::NmrOneD 109324 Specdb::NmrOneD 109325 Specdb::NmrOneD 109326 Specdb::NmrOneD 109327 Specdb::NmrOneD 109328 Specdb::NmrOneD 109329 Specdb::NmrOneD 109330 Specdb::NmrOneD 109331 Specdb::NmrOneD 109332 Specdb::NmrOneD 109333 Specdb::NmrOneD 109334 Specdb::NmrOneD 109335 Specdb::NmrOneD 109336 Specdb::NmrOneD 109337 HMDB02091 #<Reference:0x000055ce30e05cc8> #<Reference:0x000055ce30e05b10> Anatidae Type 1 specific Anatidae 8830 Beefalo Type 1 specific Bos taurus X Bison bison 297284 Bison Type 1 specific Bison bison 9901 Buffalo Type 1 specific Bubalus bubalis 89462 Cattle (Beef, Veal) Type 1 specific Bos taurus 9913 Chicken Type 1 specific Gallus gallus 9031 Columbidae (Dove, Pigeon) Type 1 specific Columbidae 8930 Deer Type 1 specific Cervidae 9850 Domestic goat Type 1 specific Capra aegagrus hircus 9925 Domestic pig Type 1 specific Sus scrofa domestica 9825 Elk Type 1 specific Cervus canadensis 1574408 Emu Type 1 specific Dromaius novaehollandiae 8790 European rabbit Type 1 specific Oryctolagus 9984 Greylag goose Type 1 specific Anser anser 8843 Guinea hen Type 1 specific Numida meleagris 8996 Horse Type 1 specific Equus caballus 9796 Mallard duck Type 1 specific Anas platyrhynchos 8839 Mountain hare Type 1 specific Lepus timidus 62621 Mule deer Type 1 specific Odocoileus 9871 Ostrich Type 1 specific Struthio camelus 8801 Pheasant Type 1 specific Phasianus colchicus 9054 Quail Type 1 specific Phasianidae 9005 Rabbit Type 1 specific Leporidae 9979 Rock ptarmigan Type 1 specific Lagopus muta 64668 Sheep (Mutton, Lamb) Type 1 specific Ovis aries 9940 Squab Type 1 specific Columba 8931 Turkey Type 1 specific Meleagris gallopavo 9103 Velvet duck Type 1 specific Melanitta fusca 371864 Wild boar Type 1 specific Sus scrofa 9823 Beta-glucuronidase P08236 GUSB UDP-glucuronosyltransferase 1-1 P22309 UGT1A1 UDP-glucuronosyltransferase 1-3 P35503 UGT1A3 UDP-glucuronosyltransferase 1-4 P22310 UGT1A4 UDP-glucuronosyltransferase 1-5 P35504 UGT1A5 UDP-glucuronosyltransferase 1-6 P19224 UGT1A6 UDP-glucuronosyltransferase 1-9 O60656 UGT1A9 UDP-glucuronosyltransferase 2A1 Q9Y4X1 UGT2A1 UDP-glucuronosyltransferase 2A3 Q6UWM9 UGT2A3 UDP-glucuronosyltransferase 2B11 O75310 UGT2B11 UDP-glucuronosyltransferase 2B15 P54855 UGT2B15 UDP-glucuronosyltransferase 2B17 O75795 UGT2B17 UDP-glucuronosyltransferase 2B28 Q9BY64 UGT2B28 UDP-glucuronosyltransferase 2B4 P06133 UGT2B4 UDP-glucuronosyltransferase 2B7 P16662 UGT2B7