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Human Metabolome Database Version 2.5

 

Showing metabocard for D-Glucuronic acid (HMDB00127)

Legend: metabolite field enzyme field

Version 2.5
Creation Date 2005-11-16 15:48:42
Update Date 2009-05-29 15:46:35
Accession Number HMDB00127
Secondary Accession Numbers Not Available
Common Name D-Glucuronic acid
Description Glucuronic acid is a carboxylic acid that has the structure of a glucose molecule that has had its sixth carbon atom (of six total) oxidized. The salts of glucuronic acid are known as glucuronates. Glucuronic acid is highly soluble in water. In the animal body, glucuronic acid is often linked to poisonous substances to allow for subsequent elimination, and to hormones to allow for easier transport. These linkages involve O-glycosidic bonds. The process is known as glucuronidation, and the resulting substances are known as glucuronides (or glucuronosides). Glucuronidation uses UDP-glucuronic acid (glucuronic acid linked via a glycosidic bond to uridine diphosphate) as an intermediate. UDP-glucuronic acid is formed in the liver of all animals.
Synonyms
  1. D-(+)-glucuronate
  2. D-(+)-glucuronic acid
  3. D-glucuronate
  4. GCU
  5. Glucosiduronate
  6. Glucosiduronic acid
  7. Glucuronate
  8. Glucuronic acid
  9. alpha-D-glucopyranuronic acid
  10. alpha-D-glucuronic acid
  11. delta-(+)-glucuronate
  12. delta-(+)-glucuronic acid
  13. delta-glucuronate
  14. alpha-delta-glucopyranuronic acid
  15. alpha-delta-glucuronic acid
Chemical IUPAC Name (2S,3S,4S,5R,6S)-3,4,5,6-tetrahydroxyoxane-2-carboxylic acid
Chemical Formula C6H10O7
Chemical Structure Structure
Chemical Taxonomy
Kingdom
  • Organic
Super Class
  • Carbohydrates and Carbohydrate conjugates
Class
  • Carbohydrates
Sub Class
  • Monosaccharides
Family
  • Mammalian Metabolite
Species
  • hemiacetal
  • secondary alcohol
  • 1,2-diol
  • carboxylic acid
  • heterocyclic compound
Biofunction
  • Component of Chondroitin / Heparan sulfate biosynthesis
  • Component of Inositol phosphate metabolism
  • Component of Porphyrin and chlorophyll metabolism
  • Component of Starch and sucrose metabolism
Application
Source
  • Endogenous
Average Molecular Weight 194.139
Monoisotopic Molecular Weight 194.042648
Isomeric SMILES O[C@H]1O[C@@H]([C@@H](O)[C@H](O)[C@H]1O)C(O)=O
Canonical SMILES OC1OC(C(O)C(O)C1O)C(O)=O
KEGG Compound ID C00191 Link Image
BioCyc ID GLUCURONATE Link Image
BiGG ID 34196 Link Image
Wikipedia Link GCU Link Image
NuGOwiki Link HMDB00127 Link Image
Metagene Link HMDB00127 Link Image
METLIN ID 161 Link Image
PubChem Compound 444791 Link Image
PubChem Substance 10299584 Link Image
ChEBI ID 35182; 42717 Link Image
CAS Registry Number 6556-12-3
InChI Identifier InChI=1/C6H10O7/c7-1-2(8)4(5(10)11)13-6(12)3(1)9/h1-4,6-9,12H,(H,10,11)/t1-,2-,3+,4-,6-/m0/s1
Synthesis Reference Hamayasu, Kenichi; Tadokoro, Hiroki; Kishino, Eriko; Ito, Tetsuya; Fujita, Koki; Hara, Kozo. Preparation of glucuronic acid and/or glucuronolactone from sucrose. PCT Int. Appl. (2006), 21pp.
Melting Point (Experimental) 143-144 oC
Experimental Water Solubility 485 mg/mL [HMP experimental] Source: PhysProp
Predicted Water Solubility 1000 mg/mL at 25 oC [MEYLAN,WM et al. (1996)]; 295.0 mg/mL [Predicted by ALOGPS] Calculated using ALOGPS
Physiological Charge -1
State Solid
Experimental LogP/Hydrophobicity -2.57 [HANSCH,C ET AL. (1995)] Source: PhysProp
Predicted LogP/Hydrophobicity -2 [Predicted by PubChem via XLOGP]; -2.30 [Predicted by ALOGPS] Calculated using ALOGPS
Material Safety Data Sheet (MSDS)
MOL File Show
SDF File Show
PDB File Show
2D Structure
3D Structure
Experimental PDB ID Not Available
Experimental 1H NMR Spectrum Download Spectrum
Download FID (Varian)
Show Experimental Conditions Link Image
Experimental 13C NMR Spectrum Download Spectrum
Download FID (Bruker)
Show Experimental Conditions Link Image
Experimental 13C HSQC Spectrum Download Spectrum
Download FID (Bruker)
Show Experimental Conditions Link Image
Predicted 1H NMR Spectrum Show Image
Show Peaklist
Predicted 13C NMR Spectrum Show Image
Show Peaklist
Mass Spectrum
Low Energy
Download File
Show Experimental Conditions Link Image
Medium Energy
Download File
Show Experimental Conditions Link Image
High Energy
Download File
Show Experimental Conditions Link Image
Simplified TOCSY Spectrum Not Available
BMRB Spectrum Not Available
Cellular Location
  • Cytoplasm (Predicted from LogP)
  • endoplasmic reticulum
  • lysosome
Biofluid Location
  • Blood
  • Urine
Tissue Location
Tissue References
Liver
Concentrations (Normal)
Biofluid Blood
Value 165.0 +/- 32.0 uM
Age Adult:>18 yrs old
Sex Male
Patient information Normal
Comments Not Available
References
  • Geigy Scientific Tables, 8th Rev edition, pp. 165-177. Edited by C. Lentner, West Cadwell, N.J.: Medical education Div., Ciba-Geigy Corp., Basel, Switzerland c1981-1992.
Biofluid Blood
Value 165.0 +/- 23.0 uM
Age Adult:>18 yrs old
Sex Female
Patient information Normal
Comments Not Available
References
  • Geigy Scientific Tables, 8th Rev edition, pp. 165-177. Edited by C. Lentner, West Cadwell, N.J.: Medical education Div., Ciba-Geigy Corp., Basel, Switzerland c1981-1992.
Biofluid Urine
Value 33.82 +/- 20.95 umol/mmol creatinine
Age Infant:0-1 yr old
Sex Both
Patient information Normal
Comments Not Available
References
  • Shoemaker JD, Elliott WH: Automated screening of urine samples for carbohydrates, organic and amino acids after treatment with urease. J Chromatogr. 1991 Jan 2;562(1-2):125-38. [PubMed Link Image]
Biofluid Urine
Value 5.9 umol/mmol creatinine
Age Newborn:0-30 days old
Sex Both
Patient information Normal
Comments Not Available
References
  • Karasawa E: [Supplementary findings on urinary glucuronic acid excretion in pediatrics. I. Total glucuronic acid in 24-hour urine of normal infants determined by the modified Fishman's method with NaBH4] Nippon Shonika Gakkai Zasshi. 1968 Aug 1;72(8):1122-7. [PubMed Link Image]
Biofluid Urine
Value 55.8 umol/mmol creatinine
Age Adult:>18 yrs old
Sex Both
Patient information Normal
Comments Not Available
References
  • Murano PS, Robichaud VY, Webb RE: Urinary excretion of sulfate and glucuronate conjugates in a free living population of adult males. Bull Environ Contam Toxicol. 1989 Jul;43(1):13-6. [PubMed Link Image]
Biofluid Urine
Value 30.5 umol/mmol creatinine
Age Infant:0-1 yr old
Sex Both
Patient information Normal
Comments Not Available
References
  • Karasawa E: [Supplementary findings on urinary glucuronic acid excretion in pediatrics. I. Total glucuronic acid in 24-hour urine of normal infants determined by the modified Fishman's method with NaBH4] Nippon Shonika Gakkai Zasshi. 1968 Aug 1;72(8):1122-7. [PubMed Link Image]
Biofluid Urine
Value 144.7 +/- 27.0 umol/mmol creatinine
Age Adult:>18 yrs old
Sex Male
Patient information Normal
Comments Not Available
References
  • Geigy Scientific Tables, 8th Rev edition, pp. 130. Edited by C. Lentner, West Cadwell, N.J.: Medical education Div., Ciba-Geigy Corp. Basel, Switzerland c1981-1992.
Biofluid Urine
Value 125.0 +/- 30.0 umol/mmol creatinine
Age Adult:>18 yrs old
Sex Female
Patient information Normal
Comments Not Available
References
  • Geigy Scientific Tables, 8th Rev edition, pp. 130. Edited by C. Lentner, West Cadwell, N.J.: Medical education Div., Ciba-Geigy Corp. Basel, Switzerland c1981-1992.
Biofluid Urine
Value 3.0 (1.38-5.00) umol/mmol creatinine
Age Children:1-13 yrs old
Sex Both
Patient information Normal
Comments Not Available
References
  • Geigy Scientific Tables, 8th Rev edition, pp. 130. Edited by C. Lentner, West Cadwell, N.J.: Medical education Div., Ciba-Geigy Corp. Basel, Switzerland c1981-1992.
Concentrations (Abnormal) Not Available
Associated Disorders Not Available
OMIM ID Not Available
Pathways
Name SMPDB Link KEGG Link
Inositol Metabolism SMP00011 Link Image map00562 Link Image
Starch and Sucrose Metabolism SMP00058 Link Image map00500 Link Image
General References
  1. Jowett TP, Slater JD: A radioimmunoassay for the measurement of tetrahydroaldosterone 3-glucosiduronic acid in human plasma. Clin Chim Acta. 1981 Jan 22;109(2):133-44. [PubMed Link Image]
  2. Schmitt G, Aderjan R, Keller T, Wu M: Ethyl glucuronide: an unusual ethanol metabolite in humans. Synthesis, analytical data, and determination in serum and urine. J Anal Toxicol. 1995 Mar-Apr;19(2):91-4. [PubMed Link Image]
  3. Karasawa E: [Supplementary findings on urinary glucuronic acid excretion in pediatrics. I. Total glucuronic acid in 24-hour urine of normal infants determined by the modified Fishman's method with NaBH4] Nippon Shonika Gakkai Zasshi. 1968 Aug 1;72(8):1122-7. [PubMed Link Image]
  4. Carpenter PC, Mattox VR: Isolation, determination of structure and synthesis of the acid-labile conjugate of aldosterone. Biochem J. 1976 Jul 1;157(1):1-14. [PubMed Link Image]
  5. Raju U, Sklarew RJ, Post J, Levitz M: Steroid metabolism in human breast cancer cell lines. Steroids. 1978 Dec;32(5):669-80. [PubMed Link Image]
  6. Stearns RA, Miller RR, Doss GA, Chakravarty PK, Rosegay A, Gatto GJ, Chiu SH: The metabolism of DuP 753, a nonpeptide angiotensin II receptor antagonist, by rat, monkey, and human liver slices. Drug Metab Dispos. 1992 Mar-Apr;20(2):281-7. [PubMed Link Image]
  7. Little JM, St Pyrek J, Lester R: Hepatic metabolism of 3 alpha-hydroxy-5 beta-etianic acid (3 alpha-hydroxy-5 beta-androstan-17 beta-carboxylic acid) in the adult rat. J Clin Invest. 1983 Jan;71(1):73-80. [PubMed Link Image]
  8. Shoemaker JD, Elliott WH: Automated screening of urine samples for carbohydrates, organic and amino acids after treatment with urease. J Chromatogr. 1991 Jan 2;562(1-2):125-38. [PubMed Link Image]
  9. Pfeiffer E, Graf E, Gerstner S, Metzler M: Stimulation of estradiol glucuronidation: a protective mechanism against estradiol-mediated carcinogenesis? Mol Nutr Food Res. 2006 Apr;50(4-5):385-9. [PubMed Link Image]
  10. McDonagh AF, Palma LA, Lauff JJ, Wu TW: Origin of mammalian biliprotein and rearrangement of bilirubin glucuronides in vivo in the rat. J Clin Invest. 1984 Sep;74(3):763-70. [PubMed Link Image]
  11. Murano PS, Robichaud VY, Webb RE: Urinary excretion of sulfate and glucuronate conjugates in a free living population of adult males. Bull Environ Contam Toxicol. 1989 Jul;43(1):13-6. [PubMed Link Image]
  12. Nishida T, Gatmaitan Z, Roy-Chowdhry J, Arias IM: Two distinct mechanisms for bilirubin glucuronide transport by rat bile canalicular membrane vesicles. Demonstration of defective ATP-dependent transport in rats (TR-) with inherited conjugated hyperbilirubinemia. J Clin Invest. 1992 Nov;90(5):2130-5. [PubMed Link Image]
  13. Wikipedia Link Image
Metabolic Enzymes
  1. Beta-glucuronidase precursor
  2. MIOX protein
  3. Dermatan-sulfate epimerase
Enzyme 1 [top]
Enzyme 1 ID 6120
Enzyme 1 Name Beta-glucuronidase precursor
Enzyme 1 Synonyms
  1. Beta-G1
Enzyme 1 Gene Name GUSB
Enzyme 1 Protein Sequence >Beta-glucuronidase precursor
MARGSAVAWAALGPLLWGCALGLQGGMLYPQESPSRECKELDGLWSFRADFSDNRRRGFE
EQWYRRPLWESGPTVDMPVPSSFNDISQDWRLRHFVGWVWYEREVILPERWTQDLRTRVV
LRIGSAHSYAIVWVNGVDTLEHEGGYLPFEADISNLVQVGPLPSRLRITIAINNTLTPTT
LPPGTIQYLTDTSKYPKGYFVQNTYFDFFNYAGLQRSVLLYTTPTTYIDDITVTTSVEQD
SGLVNYQISVKGSNLFKLEVRLLDAENKVVANGTGTQGQLKVPGVSLWWPYLMHERPAYL
YSLEVQLTAQTSLGPVSDFYTLPVGIRTVAVTKSQFLINGKPFYFHGVNKHEDADIRGKG
FDWPLLVKDFNLLRWLGANAFRTSHYPYAEEVMQMCDRYGIVVIDECPGVGLALPQFFNN
VSLHHHMQVMEEVVRRDKNHPAVVMWSVANEPASHLESAGYYLKMVIAHTKSLDPSRPVT
FVSNSNYAADKGAPYVDVICLNSYYSWYHDYGHLELIQLQLATQFENWYKKYQKPIIQSE
YGAETIAGFHQDPPLMFTEEYQKSLLEQYHLGLDQKRRKYVVGELIWNFADFMTEQSPTR
VLGNKKGIFTRQRQPKSAAFLLRERYWKIANETRYPHSVAKSQCLENSLFT
Enzyme 1 Number of Residues 651
Enzyme 1 Molecular Weight 74733
Enzyme 1 Theoretical pI 7.02
Enzyme 1 GO Classification
Function
  • catalytic activity
  • hydrolase activity
  • hydrolase activity, acting on glycosyl bonds
  • hydrolase activity, hydrolyzing O-glycosyl compounds
Process
  • carbohydrate metabolism
  • macromolecule metabolism
  • metabolism
  • physiological process
Component
Enzyme 1 General Function Carbohydrate transport and metabolism
Enzyme 1 Specific Function Plays an important role in the degradation of dermatan and keratan sulfates
Enzyme 1 Pathways
Enzyme 1 Reactions
  • a beta-D-glucuronoside + H2O = D-glucuronate + an alcohol
Enzyme 1 Pfam Domain Function
Enzyme 1 Signals
  • 1-22
Enzyme 1 Transmembrane Regions Not Available
Enzyme 1 Essentiality Not Available
Enzyme 1 GenBank ID Protein 183233 Link Image
Enzyme 1 UniProtKB/Swiss-Prot ID P08236 Link Image
Enzyme 1 UniProtKB/Swiss-Prot Entry Name BGLR_HUMAN Link Image
Enzyme 1 PDB ID 1BHG Link Image
Enzyme 1 PDB File Show
Enzyme 1 3D Structure
Enzyme 1 Cellular Location Not Available
Enzyme 1 Gene Sequence >1956 bp
ATGGCCCGGGGGTCGGCGGTTGCCTGGGCGGCGCTCGGGCCGTTGTTGTGGGGCTGCGCG
CTGGGGCTGCAGGGCGGGATGCTGTACCCCCAGGAGAGCCCGTCGCGGGAGTGCAAGGAG
CTGGACGGCCTCTGGAGCTTCCGCGCCGACTTCTCTGACAACCGACGCCGGGGCTTCGAG
GAGCAGTGGTACCGGCGGCCGCTGTGGGAGTCAGGCCCCACCGTGGACATGCCAGTTCCC
TCCAGCTTCAATGACATCAGCCAGGACTGGCGTCTGCGGCATTTTGTCGGCTGGGTGTGG
TACGAACGGGAGGTGATCCTGCCGGAGCGATGGACCCAGGACCTGCGCACAAGAGTGGTG
CTGAGGATTGGCAGTGCCCATTCCTATGCCATCGTGTGGGTGAATGGGGTCGACACGCTA
GAGCATGAGGGGGGCTACCTCCCCTTCGAGGCCGACATCAGCAACCTGGTCCAGGTGGGG
CCCCTGCCCTCCCGGCTCCGAATCACTATCGCCATCAACAACACACTCACCCCCACCACC
CTGCCACCAGGGACCATCCAATACCTGACTGACACCTCCAAGTATCCCAAGGGTTACTTT
GTCCAGAACACATATTTTGACTTTTTCAACTACGCTGGACTGCAGCGGTCTGTACTTCTG
TACACGACACCCACCACCTACATCGATGACATCACCGTCACCACCAGCGTGGAGCAAGAC
AGTGGGCTGGTGAATTACCAGATCTCTGTCAAGGGCAGTAACCTGTTCAAGTTGGAAGTG
CGTCTTTTGGATGCAGAAAACAAAGTCGTGGCGAATGGGACTGGGACCCAGGGCCAACTT
AAGGTGCCAGGTGTCAGCCTCTGGTGGCCGTACCTGATGCACGAACGCCCTGCCTATCTG
TATTCATTGGAGGTGCAGCTGACTGCACAGACGTCACTGGGGCCTGTGTCTGACTTCTAC
ACACTCCCTGTGGGGATCCGCACTGTGGCTGTCACCAAGAGCCAGTTCCTCATCAATGGG
AAACCTTTCTATTTCCACGGTGTCAACAAGCATGAGGATGCGGACATCCGAGGGAAGGGC
TTCGACTGGCCGCTGCTGGTGAAGGACTTCAACCTGCTTCGCTGGCTTGGTGCCAACGCT
TTCCGTACCAGCCACTACCCCTATGCAGAGGAAGTGATGCAGATGTGTGACCGCTATGGG
ATTGTGGTCATCGATGAGTGTCCCGGCGTGGGCCTGGCGCTGCCGCAGTTCTTCAACAAC
GTTTCTCTGCATCACCACATGCAGGTGATGGAAGAAGTGGTGCGTAGGGACAAGAACCAC
CCCGCGGTCGTGATGTGGTCTGTGGCCAACGAGCCTGCGTCCCACCTAGAATCTGCTGGC
TACTACTTGAAGATGGTGATCGCTCACACCAAATCCTTGGACCCCTCCCGGCCTGTGACC
TTTGTGAGCAACTCTAACTATGCAGCAGACAAGGGGGCTCCGTATGTGGATGTGATCTGT
TTGAACAGCTACTACTCTTGGTATCACGACTACGGGCACCTGGAGTTGATTCAGCTGCAG
CTGGCCACCCAGTTTGAGAACTGGTATAAGAAGTATCAGAAGCCCATTATTCAGAGCGAG
TATGGAGCAGAAACGATTGCAGGGTTTCACCAGGATCCACCTCTGATGTTCACTGAAGAG
TACCAGAAAAGTCTGCTAGAGCAGTACCATCTGGGTCTGGATCAAAAACGCAGAAAATAT
GTGGTTGGAGAGCTCATTTGGAATTTTGCCGATTTCATGACTGAACAGTCACCGACGAGA
GTGCTGGGGAATAAAAAGGGGATCTTCACTCGGCAGAGACAACCAAAAAGTGCAGCGTTC
CTTTTGCGAGAGAGATACTGGAAGATTGCCAATGAAACCAGGTATCCCCACTCAGTAGCC
AAGTCACAATGTTTGGAAAACAGCCCGTTTACTTGA
Enzyme 1 GenBank Gene ID M15182 Link Image
Enzyme 1 GeneCard ID GUSB Link Image
Enzyme 1 GenAtlas ID GUSB Link Image
Enzyme 1 HGNC ID HGNC:4696 Link Image
Enzyme 1 Chromosome Location 7
Enzyme 1 Locus 7q21.11
Enzyme 1 SNPs SNPJam Report Link Image
Enzyme 1 General References
  1. Oshima A, Kyle JW, Miller RD, Hoffmann JW, Powell PP, Grubb JH, Sly WS, Tropak M, Guise KS, Gravel RA: Cloning, sequencing, and expression of cDNA for human beta-glucuronidase. Proc Natl Acad Sci U S A. 1987 Feb;84(3):685-9. [PubMed Link Image]
  2. Hillier LW, Fulton RS, Fulton LA, Graves TA, Pepin KH, Wagner-McPherson C, Layman D, Maas J, Jaeger S, Walker R, Wylie K, Sekhon M, Becker MC, O'Laughlin MD, Schaller ME, Fewell GA, Delehaunty KD, Miner TL, Nash WE, Cordes M, Du H, Sun H, Edwards J, Bradshaw-Cordum H, Ali J, Andrews S, Isak A, Vanbrunt A, Nguyen C, Du F, Lamar B, Courtney L, Kalicki J, Ozersky P, Bielicki L, Scott K, Holmes A, Harkins R, Harris A, Strong CM, Hou S, Tomlinson C, Dauphin-Kohlberg S, Kozlowicz-Reilly A, Leonard S, Rohlfing T, Rock SM, Tin-Wollam AM, Abbott A, Minx P, Maupin R, Strowmatt C, Latreille P, Miller N, Johnson D, Murray J, Woessner JP, Wendl MC, Yang SP, Schultz BR, Wallis JW, Spieth J, Bieri TA, Nelson JO, Berkowicz N, Wohldmann PE, Cook LL, Hickenbotham MT, Eldred J, Williams D, Bedell JA, Mardis ER, Clifton SW, Chissoe SL, Marra MA, Raymond C, Haugen E, Gillett W, Zhou Y, James R, Phelps K, Iadanoto S, Bubb K, Simms E, Levy R, Clendenning J, Kaul R, Kent WJ, Furey TS, Baertsch RA, Brent MR, Keibler E, Flicek P, Bork P, Suyama M, Bailey JA, Portnoy ME, Torrents D, Chinwalla AT, Gish WR, Eddy SR, McPherson JD, Olson MV, Eichler EE, Green ED, Waterston RH, Wilson RK: The DNA sequence of human chromosome 7. Nature. 2003 Jul 10;424(6945):157-64. [PubMed Link Image]
  3. Shipley JM, Miller RD, Wu BM, Grubb JH, Christensen SG, Kyle JW, Sly WS: Analysis of the 5' flanking region of the human beta-glucuronidase gene. Genomics. 1991 Aug;10(4):1009-18. [PubMed Link Image]
  4. Tanaka J, Gasa S, Sakurada K, Miyazaki T, Kasai M, Makita A: Characterization of the subunits and sugar moiety of human placental and leukemic beta-glucuronidase. Biol Chem Hoppe Seyler. 1992 Jan;373(1):57-62. [PubMed Link Image]
  5. Guise KS, Korneluk RG, Waye J, Lamhonwah AM, Quan F, Palmer R, Ganschow RE, Sly WS, Gravel RA: Isolation and expression in Escherichia coli of a cDNA clone encoding human beta-glucuronidase. Gene. 1985;34(1):105-10. [PubMed Link Image]
  6. Zhang H, Li XJ, Martin DB, Aebersold R: Identification and quantification of N-linked glycoproteins using hydrazide chemistry, stable isotope labeling and mass spectrometry. Nat Biotechnol. 2003 Jun;21(6):660-6. Epub 2003 May 18. [PubMed Link Image]
  7. Jain S, Drendel WB, Chen ZW, Mathews FS, Sly WS, Grubb JH: Structure of human beta-glucuronidase reveals candidate lysosomal targeting and active-site motifs. Nat Struct Biol. 1996 Apr;3(4):375-81. [PubMed Link Image]
  8. Vervoort R, Lissens W, Liebaers I: Molecular analysis of a patient with hydrops fetalis caused by beta-glucuronidase deficiency, and evidence for additional pseudogenes. Hum Mutat. 1993;2(6):443-5. [PubMed Link Image]
  9. Wu BM, Sly WS: Mutational studies in a patient with the hydrops fetalis form of mucopolysaccharidosis type VII. Hum Mutat. 1993;2(6):446-57. [PubMed Link Image]
  10. Tomatsu S, Fukuda S, Sukegawa K, Ikedo Y, Yamada S, Yamada Y, Sasaki T, Okamoto H, Kuwahara T, Yamaguchi S, et al.: Mucopolysaccharidosis type VII: characterization of mutations and molecular heterogeneity. Am J Hum Genet. 1991 Jan;48(1):89-96. [PubMed Link Image]
  11. Shipley JM, Klinkenberg M, Wu BM, Bachinsky DR, Grubb JH, Sly WS: Mutational analysis of a patient with mucopolysaccharidosis type VII, and identification of pseudogenes. Am J Hum Genet. 1993 Mar;52(3):517-26. [PubMed Link Image]
Enzyme 1 Metabolite References Not Available
Enzyme 2 [top]
Enzyme 2 ID 13064
Enzyme 2 Name MIOX protein
Enzyme 2 Synonyms
  1. Myo-inositol oxygenase, isoform CRA_d
  2. Myo-inositol oxygenase
Enzyme 2 Gene Name MIOX
Enzyme 2 Protein Sequence >MIOX protein
MKVTVGPDPSLVYRPDVDPEVAKDKASFRNYTSGPLLDRVFTTYKLMHTHQTVDFVRSKH
AQFGGFSYKKMTVMEAVDLLDGLVDESDPDVDFPNSFHAFQTAEGIRKAHPDKVPNLPCP
SPSQTGSTSSGSCTTWGRSWPCSGSPSGLSSATPSPSDAVRRPPWFSATPPSRTTLTSRI
LDTAQSSGCISPTVGSTGSSCPGAMMQVRPLHQVPGPAGRGQAAALLPGAH
Enzyme 2 Number of Residues 231
Enzyme 2 Molecular Weight 24516
Enzyme 2 Theoretical pI 8.23
Enzyme 2 GO Classification Not Available
Enzyme 2 General Function Not Available
Enzyme 2 Specific Function Not Available
Enzyme 2 Pathways Not Available
Enzyme 2 Reactions Not Available
Enzyme 2 Pfam Domain Function
Enzyme 2 Signals
  • None
Enzyme 2 Transmembrane Regions
  • None
Enzyme 2 Essentiality Not Available
Enzyme 2 GenBank ID Protein 116283362 Link Image
Enzyme 2 UniProtKB/Swiss-Prot ID Q05DJ6 Link Image
Enzyme 2 UniProtKB/Swiss-Prot Entry Name Q05DJ6_HUMAN Link Image
Enzyme 2 PDB ID Not Available
Enzyme 2 Cellular Location Not Available
Enzyme 2 Gene Sequence Not Available
Enzyme 2 GenBank Gene ID BC012025 Link Image
Enzyme 2 GeneCard ID Q05DJ6 Link Image
Enzyme 2 GenAtlas ID MIOX Link Image
Enzyme 2 HGNC ID HGNC:14522 Link Image
Enzyme 2 Chromosome Location Not Available
Enzyme 2 Locus Not Available
Enzyme 2 SNPs SNPJam Report Link Image
Enzyme 2 General References
  1. Strausberg RL, Feingold EA, Grouse LH, Derge JG, Klausner RD, Collins FS, Wagner L, Shenmen CM, Schuler GD, Altschul SF, Zeeberg B, Buetow KH, Schaefer CF, Bhat NK, Hopkins RF, Jordan H, Moore T, Max SI, Wang J, Hsieh F, Diatchenko L, Marusina K, Farmer AA, Rubin GM, Hong L, Stapleton M, Soares MB, Bonaldo MF, Casavant TL, Scheetz TE, Brownstein MJ, Usdin TB, Toshiyuki S, Carninci P, Prange C, Raha SS, Loquellano NA, Peters GJ, Abramson RD, Mullahy SJ, Bosak SA, McEwan PJ, McKernan KJ, Malek JA, Gunaratne PH, Richards S, Worley KC, Hale S, Garcia AM, Gay LJ, Hulyk SW, Villalon DK, Muzny DM, Sodergren EJ, Lu X, Gibbs RA, Fahey J, Helton E, Ketteman M, Madan A, Rodrigues S, Sanchez A, Whiting M, Madan A, Young AC, Shevchenko Y, Bouffard GG, Blakesley RW, Touchman JW, Green ED, Dickson MC, Rodriguez AC, Grimwood J, Schmutz J, Myers RM, Butterfield YS, Krzywinski MI, Skalska U, Smailus DE, Schnerch A, Schein JE, Jones SJ, Marra MA: Generation and initial analysis of more than 15,000 full-length human and mouse cDNA sequences. Proc Natl Acad Sci U S A. 2002 Dec 24;99(26):16899-903. Epub 2002 Dec 11. [PubMed Link Image]
Enzyme 2 Metabolite References Not Available
Enzyme 3 [top]
Enzyme 3 ID 14922
Enzyme 3 Name Dermatan-sulfate epimerase
Enzyme 3 Synonyms
  1. DS epimerase
  2. Chondroitin-glucuronate 5-epimerase
  3. Squamous cell carcinoma antigen recognized by T-cells 2
  4. SART-2
Enzyme 3 Gene Name DSE
Enzyme 3 Protein Sequence >Dermatan-sulfate epimerase
MRTHTRGAPSVFFIYLLCFVSAYITDENPEVMIPFTNANYDSHPMLYFSRAEVAELQLRA
ASSHEHIAARLTEAVHTMLSSPLEYLPPWDPKDYSARWNEIFGNNLGALAMFCVLYPENI
EARDMAKDYMERMAAQPSWLVKDAPWDEVPLAHSLVGFATAYDFLYNYLSKTQQEKFLEV
IANASGYMYETSYRRGWGFQYLHNHQPTNCMALLTGSLVLMNQGYLQEAYLWTKQVLTIM
EKSLVLLREVTDGSLYEGVAYGSYTTRSLFQYMFLVQRHFNINHFGHPWLKQHFAFMYRT
ILPGFQRTVAIADSNYNWFYGPESQLVFLDKFVMRNGSGNWLADQIRRNRVVEGPGTPSK
GQRWCTLHTEFLWYDGSLKSVPPPDFGTPTLHYFEDWGVVTYGSALPAEINRSFLSFKSG
KLGGRAIYDIVHRNKYKDWIKGWRNFNAGHEHPDQNSFTFAPNGVPFITEALYGPKYTFF
NNVLMFSPAVSKSCFSPWVGQVTEDCSSKWSKYKHDLAASCQGRVVAAEEKNGVVFIRGE
GVGAYNPQLNLKNVQRNLILLHPQLLLLVDQIHLGEESPLETAASFFHNVDVPFEETVVD
GVHGAFIRQRDGLYKMYWMDDTGYSEKATFASVTYPRGYPYNGTNYVNVTMHLRSPITRA
AYLFIGPSIDVQSFTVHGDSQQLDVFIATSKHAYATYLWTGEATGQSAFAQVIADRHKIL
FDRNSAIKSSIVPEVKDYAAIVEQNLQHFKPVFQLLEKQILSRVRNTASFRKTAERLLRF
SDKRQTEEAIDRIFAISQQQQQQSKSKKNRRAGKRYKFVDAVPDIFAQIEVNEKKIRQKA
QILAQKELPIDEDEEMKDLLDFADVTYEKHKNGGLIKGRFGQARMVTTTHSRAPSLSASY
TRLFLILNIAIFFVMLAMQLTYFQRAQSLHGQRCLYAVLLIDSCILLWLYSSCSQSQC
Enzyme 3 Number of Residues 958
Enzyme 3 Molecular Weight 109774
Enzyme 3 Theoretical pI 8.19
Enzyme 3 GO Classification Not Available
Enzyme 3 General Function Not Available
Enzyme 3 Specific Function Converts D-glucuronic acid to L-iduronic acid (IdoUA) residues
Enzyme 3 Pathways Not Available
Enzyme 3 Reactions Not Available
Enzyme 3 Pfam Domain Function Not Available
Enzyme 3 Signals
  • 1-22
Enzyme 3 Transmembrane Regions
  • 903-923 934-954
Enzyme 3 Essentiality Not Available
Enzyme 3 GenBank ID Protein 6002669 Link Image
Enzyme 3 UniProtKB/Swiss-Prot ID Q9UL01 Link Image
Enzyme 3 UniProtKB/Swiss-Prot Entry Name DSE_HUMAN Link Image
Enzyme 3 PDB ID Not Available
Enzyme 3 Cellular Location Not Available
Enzyme 3 Gene Sequence >2877 bp
ATGAGGACTCACACACGGGGGGCTCCCAGTGTGTTTTTCATATATTTGCTTTGCTTTGTG
TCAGCCTACATCACCGACGAGAACCCAGAAGTTATGATTCCCTTCACCAATGCCAACTAC
GACAGCCATCCCATGCTGTACTTCTCCAGGGCAGAAGTGGCGGAGCTGCAGCTCAGGGCT
GCCAGCTCGCACGAGCACATTGCAGCCCGCCTCACGGAGGCTGTGCACACGATGCTGTCC
AGCCCCTTGGAATACCTCCCTCCCTGGGATCCCAAGGACTACAGTGCCCGCTGGAATGAA
ATTTTTGGAAACAACTTGGGTGCCTTGGCAATGTTCTGTGTGCTGTATCCTGAGAACATT
GAAGCCCGAGACATGGCCAAAGACTACATGGAGAGGATGGCAGCGCAGCCTAGTTGGTTG
GTGAAAGATGCTCCTTGGGATGAGGTCCCGCTTGCTCACTCCCTGGTTGGTTTTGCCACT
GCTTATGACTTCTTGTACAACTACCTGAGCAAGACACAACAGGAGAAGTTTCTTGAAGTG
ATTGCCAATGCCTCAGGGTATATGTATGAAACTTCATACAGGAGAGGATGGGGATTTCAA
TACCTGCACAATCATCAGCCCACCAACTGTATGGCTTTGCTCACGGGAAGCCTAGTCCTG
ATGAATCAAGGATATCTTCAAGAAGCCTACTTATGGACCAAACAAGTTCTGACCATCATG
GAGAAATCTCTGGTCTTGCTCAGGGAGGTGACGGATGGCTCCCTCTATGAAGGAGTTGCG
TATGGCAGCTACACCACTAGATCACTCTTCCAATACATGTTTCTCGTCCAGAGGCACTTC
AACATCAACCACTTTGGCCATCCGTGGCTTAAACAACACTTTGCATTTATGTATAGAACC
ATCCTGCCAGGGTTTCAAAGGACTGTGGCTATTGCGGACTCAAATTACAACTGGTTTTAT
GGTCCAGAAAGCCAATTAGTGTTCCTTGATAAATTTGTCATGCGTAATGGCAGTGGTAAC
TGGCTAGCTGACCAAATCAGAAGGAACCGTGTGGTGGAAGGTCCAGGAACACCATCCAAA
GGGCAGCGCTGGTGCACTCTGCACACAGAATTTCTCTGGTATGATGGCAGCTTGAAATCG
GTTCCTCCTCCAGACTTTGGCACCCCTACACTGCATTATTTTGAAGACTGGGGTGTCGTG
ACTTATGGAAGTGCACTACCTGCAGAAATCAATAGATCTTTCCTTTCCTTCAAGTCTGGA
AAACTGGGGGGACGTGCAATATATGACATTGTCCACAGAAACAAATACAAAGATTGGATC
AAAGGATGGAGAAATTTTAATGCAGGGCATGAACATCCTGATCAAAACTCATTTACTTTT
GCTCCCAATGGTGTGCCTTTCATTACTGAGGCTCTGTACGGGCCAAAGTACACCTTCTTC
AACAATGTTTTGATGTTTTCCCCAGCTGTGTCAAAGAGCTGCTTTTCTCCCTGGGTGGGT
CAGGTCACAGAAGACTGCTCATCAAAATGGTCTAAATACAAGCATGACCTGGCAGCTAGT
TGTCAGGGGAGGGTGGTTGCAGCAGAGGAGAAAAATGGGGTGGTTTTCATCCGAGGAGAA
GGTGTGGGAGCTTATAACCCCCAGCTCAACCTGAAGAATGTTCAGAGGAATCTCATCCTC
CTACATCCACAGCTGCTTCTCCTTGTAGACCAAATACACCTGGGAGAGGAGAGTCCCTTG
GAGACAGCAGCGAGCTTCTTCCATAATGTGGATGTTCCTTTTGAGGAGACTGTGGTAGAT
GGTGTCCATGGGGCTTTCATCAGGCAGAGAGATGGTCTCTATAAAATGTACTGGATGGAC
GATACTGGCTACAGCGAGAAAGCAACCTTTGCCTCAGTGACATATCCTCGGGGCTATCCC
TACAACGGGACAAACTATGTGAATGTCACCATGCACCTCCGAAGTCCCATCACCAGGGCA
GCTTACCTCTTCATAGGGCCATCTATAGATGTTCAGAGCTTCACTGTCCACGGAGACTCT
CAGCAACTGGATGTGTTCATAGCCACCAGCAAACATGCCTACGCCACATACCTGTGGACA
GGTGAGGCCACAGGACAGTCTGCCTTTGCACAGGTCATTGCTGATCGTCACAAAATTCTG
TTTGACCGGAATTCAGCCATCAAGAGCAGCATTGTCCCTGAGGTGAAGGACTATGCTGCT
ATTGTGGAACAGAACTTGCAGCATTTTAAACCAGTGTTTCAGCTGCTGGAGAAGCAGATA
CTGTCCCGAGTCCGGAACACAGCTAGCTTTAGGAAGACTGCTGAACGCCTGCTGAGATTT
TCAGATAAGAGACAGACTGAGGAGGCCATTGACAGGATTTTTGCCATATCACAGCAACAG
CAGCAGCAAAGCAAGTCAAAGAAAAACCGAAGGGCAGGCAAACGCTATAAATTTGTGGAT
GCTGTCCCTGATATTTTTGCACAGATTGAAGTCAATGAGAAAAAGATTAGACAGAAAGCT
CAGATTTTGGCACAGAAAGAACTACCCATAGATGAAGATGAAGAAATGAAAGACCTTTTA
GATTTTGCAGATGTAACATACGAGAAACATAAAAATGGGGGCTTGATTAAAGGCCGGTTT
GGACAGGCACGGATGGTGACAACTACACACAGCAGGGCCCCATCACTGTCTGCTTCCTAT
ACCAGGTTGTTCCTGATTCTGAACATTGCTATTTTCTTTGTCATGTTGGCAATGCAACTG
ACTTATTTCCAGAGGGCCCAGAGCCTACATGGCCAAAGATGTCTTTATGCAGTTCTTCTC
ATAGATAGCTGTATTTTATTATGGTTGTACTCTTCTTGTTCCCAATCACAGTGTTAG
Enzyme 3 GenBank Gene ID AF098066 Link Image
Enzyme 3 GeneCard ID Q9UL01 Link Image
Enzyme 3 GenAtlas ID DSE Link Image
Enzyme 3 HGNC ID HGNC:21144 Link Image
Enzyme 3 Chromosome Location Not Available
Enzyme 3 Locus Not Available
Enzyme 3 SNPs SNPJam Report Link Image
Enzyme 3 General References
  1. Nakao M, Shichijo S, Imaizumi T, Inoue Y, Matsunaga K, Yamada A, Kikuchi M, Tsuda N, Ohta K, Takamori S, Yamana H, Fujita H, Itoh K: Identification of a gene coding for a new squamous cell carcinoma antigen recognized by the CTL. J Immunol. 2000 Mar 1;164(5):2565-74. [PubMed Link Image]
  2. Mungall AJ, Palmer SA, Sims SK, Edwards CA, Ashurst JL, Wilming L, Jones MC, Horton R, Hunt SE, Scott CE, Gilbert JG, Clamp ME, Bethel G, Milne S, Ainscough R, Almeida JP, Ambrose KD, Andrews TD, Ashwell RI, Babbage AK, Bagguley CL, Bailey J, Banerjee R, Barker DJ, Barlow KF, Bates K, Beare DM, Beasley H, Beasley O, Bird CP, Blakey S, Bray-Allen S, Brook J, Brown AJ, Brown JY, Burford DC, Burrill W, Burton J, Carder C, Carter NP, Chapman JC, Clark SY, Clark G, Clee CM, Clegg S, Cobley V, Collier RE, Collins JE, Colman LK, Corby NR, Coville GJ, Culley KM, Dhami P, Davies J, Dunn M, Earthrowl ME, Ellington AE, Evans KA, Faulkner L, Francis MD, Frankish A, Frankland J, French L, Garner P, Garnett J, Ghori MJ, Gilby LM, Gillson CJ, Glithero RJ, Grafham DV, Grant M, Gribble S, Griffiths C, Griffiths M, Hall R, Halls KS, Hammond S, Harley JL, Hart EA, Heath PD, Heathcott R, Holmes SJ, Howden PJ, Howe KL, Howell GR, Huckle E, Humphray SJ, Humphries MD, Hunt AR, Johnson CM, Joy AA, Kay M, Keenan SJ, Kimberley AM, King A, Laird GK, Langford C, Lawlor S, Leongamornlert DA, Leversha M, Lloyd CR, Lloyd DM, Loveland JE, Lovell J, Martin S, Mashreghi-Mohammadi M, Maslen GL, Matthews L, McCann OT, McLaren SJ, McLay K, McMurray A, Moore MJ, Mullikin JC, Niblett D, Nickerson T, Novik KL, Oliver K, Overton-Larty EK, Parker A, Patel R, Pearce AV, Peck AI, Phillimore B, Phillips S, Plumb RW, Porter KM, Ramsey Y, Ranby SA, Rice CM, Ross MT, Searle SM, Sehra HK, Sheridan E, Skuce CD, Smith S, Smith M, Spraggon L, Squares SL, Steward CA, Sycamore N, Tamlyn-Hall G, Tester J, Theaker AJ, Thomas DW, Thorpe A, Tracey A, Tromans A, Tubby B, Wall M, Wallis JM, West AP, White SS, Whitehead SL, Whittaker H, Wild A, Willey DJ, Wilmer TE, Wood JM, Wray PW, Wyatt JC, Young L, Younger RM, Bentley DR, Coulson A, Durbin R, Hubbard T, Sulston JE, Dunham I, Rogers J, Beck S: The DNA sequence and analysis of human chromosome 6. Nature. 2003 Oct 23;425(6960):805-11. [PubMed Link Image]
Enzyme 3 Metabolite References Not Available