KEGG   PATHWAY: hsa00520Help
Entry
hsa00520                    Pathway                                

Name
Amino sugar and nucleotide sugar metabolism - Homo sapiens (human)
Class
Metabolism; Carbohydrate metabolism
BRITE hierarchy
Pathway map
hsa00520  Amino sugar and nucleotide sugar metabolism
hsa00520

Ortholog table
Module
hsa_M00361  Nucleotide sugar biosynthesis, eukaryotes [PATH:hsa00520]
hsa_M00549  Nucleotide sugar biosynthesis, glucose => UDP-glucose [PATH:hsa00520]
hsa_M00554  Nucleotide sugar biosynthesis, galactose => UDP-galactose [PATH:hsa00520]
Disease
H00070  Galactosemia
H00147  Sialuria
H00235  Methemoglobinemia
H00594  Distal myopathy
H00596  Nonaka distal myopathy (NM)
H00770  Congenital myasthenic syndrome
H01954  Glycogen storage disease type XIV
Organism
Homo sapiens (human) [GN:hsa]
Gene
27159  CHIA; chitinase, acidic [KO:K01183] [EC:3.2.1.14]
1118  CHIT1; chitinase 1 [KO:K01183] [EC:3.2.1.14]
3073  HEXA; hexosaminidase subunit alpha [KO:K12373] [EC:3.2.1.52]
3074  HEXB; hexosaminidase subunit beta [KO:K12373] [EC:3.2.1.52]
55577  NAGK; N-acetylglucosamine kinase [KO:K00884] [EC:2.7.1.59]
5238  PGM3; phosphoglucomutase 3 [KO:K01836] [EC:5.4.2.3]
6675  UAP1; UDP-N-acetylglucosamine pyrophosphorylase 1 [KO:K00972] [EC:2.7.7.83 2.7.7.23]
91373  UAP1L1; UDP-N-acetylglucosamine pyrophosphorylase 1 like 1 [KO:K00972] [EC:2.7.7.83 2.7.7.23]
10020  GNE; glucosamine (UDP-N-acetyl)-2-epimerase/N-acetylmannosamine kinase [KO:K12409] [EC:2.7.1.60 3.2.1.183]
5973  RENBP; renin binding protein [KO:K01787] [EC:5.1.3.8]
54187  NANS; N-acetylneuraminate synthase [KO:K05304] [EC:2.5.1.132 2.5.1.57 2.5.1.56]
140838  NANP; N-acetylneuraminic acid phosphatase [KO:K01097] [EC:3.1.3.29]
80896  NPL; N-acetylneuraminate pyruvate lyase [KO:K01639] [EC:4.1.3.3]
55907  CMAS; cytidine monophosphate N-acetylneuraminic acid synthetase [KO:K21749] [EC:2.7.7.92 2.7.7.43]
51706  CYB5R1; cytochrome b5 reductase 1 [KO:K00326] [EC:1.6.2.2]
1727  CYB5R3; cytochrome b5 reductase 3 [KO:K00326] [EC:1.6.2.2]
51700  CYB5R2; cytochrome b5 reductase 2 [KO:K00326] [EC:1.6.2.2]
606495  CYB5RL; cytochrome b5 reductase like [KO:K00326] [EC:1.6.2.2]
51167  CYB5R4; cytochrome b5 reductase 4 [KO:K00326] [EC:1.6.2.2]
3101  HK3; hexokinase 3 [KO:K00844] [EC:2.7.1.1]
3098  HK1; hexokinase 1 [KO:K00844] [EC:2.7.1.1]
3099  HK2; hexokinase 2 [KO:K00844] [EC:2.7.1.1]
80201  HKDC1; hexokinase domain containing 1 [KO:K00844] [EC:2.7.1.1]
51005  AMDHD2; amidohydrolase domain containing 2 [KO:K01443] [EC:3.5.1.25]
64841  GNPNAT1; glucosamine-phosphate N-acetyltransferase 1 [KO:K00621] [EC:2.3.1.4]
10007  GNPDA1; glucosamine-6-phosphate deaminase 1 [KO:K02564] [EC:3.5.99.6]
132789  GNPDA2; glucosamine-6-phosphate deaminase 2 [KO:K02564] [EC:3.5.99.6]
9945  GFPT2; glutamine-fructose-6-phosphate transaminase 2 [KO:K00820] [EC:2.6.1.16]
2673  GFPT1; glutamine--fructose-6-phosphate transaminase 1 [KO:K00820] [EC:2.6.1.16]
80146  UXS1; UDP-glucuronate decarboxylase 1 [KO:K08678] [EC:4.1.1.35]
2645  GCK; glucokinase [KO:K12407] [EC:2.7.1.2]
2821  GPI; glucose-6-phosphate isomerase [KO:K01810] [EC:5.3.1.9]
5236  PGM1; phosphoglucomutase 1 [KO:K01835] [EC:5.4.2.2]
55276  PGM2; phosphoglucomutase 2 [KO:K15779] [EC:5.4.2.7 5.4.2.2]
7360  UGP2; UDP-glucose pyrophosphorylase 2 [KO:K00963] [EC:2.7.7.9]
7358  UGDH; UDP-glucose 6-dehydrogenase [KO:K00012] [EC:1.1.1.22]
2584  GALK1; galactokinase 1 [KO:K00849] [EC:2.7.1.6]
2592  GALT; galactose-1-phosphate uridylyltransferase [KO:K00965] [EC:2.7.7.12]
2582  GALE; UDP-galactose-4-epimerase [KO:K01784] [EC:5.1.3.2]
5373  PMM2; phosphomannomutase 2 [KO:K17497] [EC:5.4.2.8]
5372  PMM1; phosphomannomutase 1 [KO:K17497] [EC:5.4.2.8]
29925  GMPPB; GDP-mannose pyrophosphorylase B [KO:K00966] [EC:2.7.7.13]
29926  GMPPA; GDP-mannose pyrophosphorylase A [KO:K00966] [EC:2.7.7.13]
2762  GMDS; GDP-mannose 4,6-dehydratase [KO:K01711] [EC:4.2.1.47]
4351  MPI; mannose phosphate isomerase [KO:K01809] [EC:5.3.1.8]
197258  FUK; fucokinase [KO:K05305] [EC:2.7.1.52]
8790  FPGT; fucose-1-phosphate guanylyltransferase [KO:K00976] [EC:2.7.7.30]
7264  TSTA3; tissue specific transplantation antigen P35B [KO:K02377] [EC:1.1.1.271]
Compound
C00029  UDP-glucose
C00031  D-Glucose
C00043  UDP-N-acetyl-alpha-D-glucosamine
C00052  UDP-alpha-D-galactose
C00096  GDP-mannose
C00103  D-Glucose 1-phosphate
C00128  CMP-N-acetylneuraminate
C00140  N-Acetyl-D-glucosamine
C00159  D-Mannose
C00167  UDP-glucuronate
C00181  D-Xylose
C00190  UDP-D-xylose
C00191  D-Glucuronate
C00203  UDP-N-acetyl-D-galactosamine
C00259  L-Arabinose
C00267  alpha-D-Glucose
C00270  N-Acetylneuraminate
C00275  D-Mannose 6-phosphate
C00325  GDP-L-fucose
C00329  D-Glucosamine
C00333  D-Galacturonate
C00352  D-Glucosamine 6-phosphate
C00357  N-Acetyl-D-glucosamine 6-phosphate
C00394  GDP-glucose
C00446  alpha-D-Galactose 1-phosphate
C00461  Chitin
C00498  ADP-glucose
C00501  CDP-glucose
C00617  UDP-D-galacturonate
C00636  D-Mannose 1-phosphate
C00645  N-Acetyl-D-mannosamine
C00668  alpha-D-Glucose 6-phosphate
C00714  Pectin
C00734  Chitosan
C00935  UDP-L-arabinose
C00976  GDP-D-mannuronate
C00984  alpha-D-Galactose
C00996  Ferricytochrome b5
C00999  Ferrocytochrome b5
C01019  6-Deoxy-L-galactose
C01050  UDP-N-acetylmuramate
C01170  UDP-N-acetyl-D-mannosamine
C01219  CDP-4-dehydro-6-deoxy-D-glucose
C01222  GDP-4-dehydro-6-deoxy-D-mannose
C01623  UDP-apiose
C01674  Chitobiose
C01788  CDP-abequose
C02199  UDP-L-rhamnose
C02280  GDP-L-galactose
C02330  UDP-L-iduronate
C02336  beta-D-Fructose
C02352  1,4-beta-D-Xylan
C02474  Arabinan
C02713  N-Acetylmuramate
C02977  GDP-6-deoxy-D-talose
C02985  L-Fucose 1-phosphate
C03117  GDP-6-deoxy-D-mannose
C03410  N-Glycoloyl-neuraminate
C03598  CDP-3,6-dideoxy-D-glucose
C03599  CDP-3,6-dideoxy-D-mannose
C03691  CMP-N-glycoloylneuraminate
C03733  UDP-alpha-D-galactofuranose
C03906  beta-L-Arabinose 1-phosphate
C04037  1-Phospho-alpha-D-galacturonate
C04089  UDP-4-dehydro-6-deoxy-D-glucose
C04257  N-Acetyl-D-mannosamine 6-phosphate
C04297  CDP-4-dehydro-3,6-dideoxy-D-glucose
C04501  N-Acetyl-alpha-D-glucosamine 1-phosphate
C04573  UDP-N-acetyl-2-amino-2-deoxy-D-glucuronate
C04613  UDP-2-acetamido-4-dehydro-2,6-dideoxyglucose
C04630  UDP-2-acetamido-4-amino-2,4,6-trideoxy-alpha-D-glucose
C04631  UDP-N-acetyl-3-(1-carboxyvinyl)-D-glucosamine
C05345  beta-D-Fructose 6-phosphate
C05385  D-Glucuronate 1-phosphate
C06023  D-Glucosaminide
C06156  alpha-D-Glucosamine 1-phosphate
C06240  UDP-N-acetyl-D-mannosaminouronate
C06241  N-Acetylneuraminate 9-phosphate
C11521  UDP-6-sulfoquinovose
C13952  UDP-N-acetyl-D-galactosaminuronic acid
C15925  GDP-L-gulose
C16153  UDP-L-Ara4N
C16154  UDP-L-Ara4FN
C16155  UDP-L-Ara4O
C16156  Undecaprenyl phosphate alpha-L-Ara4FN
C16157  Undecaprenyl phosphate alpha-L-Ara4N
C16698  N-Acetylmuramic acid 6-phosphate
C17326  CDP-4-dehydro-3,6-dideoxy-D-glucose epimer
C17327  CDP-ascarylose
C17328  UDP-4-keto-rhamnose
C18094  UDP-L-arabinofuranose
C19725  UDP-2,3-diacetamido-2,3-dideoxy-alpha-D-glucuronate
C19745  UDP-2,3-diacetamido-2,3-dideoxy-alpha-D-mannuronate
C19823  UDP-2-acetamido-2,6-dideoxy-beta-L-arabino-hexos-4-ulose
C19961  UDP-4-amino-4,6-dideoxy-N-acetyl-beta-L-altrosamine
C19971  UDP-2,4-bis(acetamido)-2,4,6-trideoxy-beta-L-altropyranose
C19972  2,4-Bis(acetamido)-2,4,6-trideoxy-beta-L-altropyranose
C20082  Pseudaminic acid
C20083  CMP-pseudaminic acid
C20357  UDP-N,N'-Diacetylbacillosamine
C20359  UDP-2-acetamido-3-amino-2,3-dideoxy-alpha-D-glucuronate
C20395  UDP-2-acetamido-2-deoxy-alpha-D-ribo-hex-3-uluronate
C20418  N,N'-Diacetyllegionaminate
C20419  CMP-N,N'-diacetyllegionaminate
C20424  2,4-Diacetamido-2,4,6-trideoxy-D-mannopyranose
C20638  GDP-4-amino-4,6-dideoxy-alpha-D-mannose
C20672  GDP-4-acetamido-4,6-dideoxy-alpha-D-mannose
C21027  N-Acetylmuramic acid alpha-1-phosphate
Reference
PMID:2793832
  Authors
Wyk P, Reeves P
  Title
Identification and sequence of the gene for abequose synthase, which confers antigenic specificity on group B salmonellae: homology with galactose epimerase.
  Journal
J Bacteriol 171:5687-93 (1989)
DOI:10.1128/JB.171.10.5687-5693.1989
Reference
PMID:8071227
  Authors
Thorson JS, Lo SF, Ploux O, He X, Liu HW
  Title
Studies of the biosynthesis of 3,6-dideoxyhexoses: molecular cloning and characterization of the asc (ascarylose) region from Yersinia pseudotuberculosis serogroup VA.
  Journal
J Bacteriol 176:5483-93 (1994)
DOI:10.1128/JB.176.17.5483-5493.1994
Reference
PMID:8444803
  Authors
Kessler AC, Haase A, Reeves PR
  Title
Molecular analysis of the 3,6-dideoxyhexose pathway genes of Yersinia pseudotuberculosis serogroup IIA.
  Journal
J Bacteriol 175:1412-22 (1993)
DOI:10.1128/JB.175.5.1412-1422.1993
Reference
PMID:9811644
  Authors
Danese PN, Oliver GR, Barr K, Bowman GD, Rick PD, Silhavy TJ
  Title
Accumulation of the enterobacterial common antigen lipid II biosynthetic intermediate stimulates degP transcription in Escherichia coli.
  Journal
J Bacteriol 180:5875-84 (1998)
Reference
  Authors
Skurnik M, Peippo A, Ervela E
  Title
Characterization of the O-antigen gene clusters of Yersinia pseudotuberculosis and the cryptic O-antigen gene cluster of Yersinia pestis shows that the plague bacillus is most closely related to and has evolved from Y. pseudotuberculosis serotype O:1b.
  Journal
Mol Microbiol 37:316-30 (2000)
DOI:10.1046/j.1365-2958.2000.01993.x
Reference
  Authors
Stover CK, Pham XQ, Erwin AL, Mizoguchi SD, Warrener P, Hickey MJ, Brinkman FS, Hufnagle WO, Kowalik DJ, Lagrou M, Garber RL, Goltry L, Tolentino E, Westbrock-Wadman S, Yuan Y, Brody LL, Coulter SN, Folger KR, Kas A, Larbig K, Lim R, Smith K, Spencer D, Wong GK, Wu Z, Paulsen IT, Reizer J, Saier MH, Hancock RE, Lory S, Olson MV
  Title
Complete genome sequence of Pseudomonas aeruginosa PAO1, an opportunistic pathogen.
  Journal
Nature 406:959-64 (2000)
DOI:10.1038/35023079
Reference
  Authors
Ringenberg M, Lichtensteiger C, Vimr E
  Title
Redirection of sialic acid metabolism in genetically engineered Escherichia coli.
  Journal
Glycobiology 11:533-9 (2001)
DOI:10.1093/glycob/11.7.533
Reference
  Authors
McCoy AJ, Sandlin RC, Maurelli AT
  Title
In vitro and in vivo functional activity of Chlamydia MurA, a UDP-N-acetylglucosamine enolpyruvyl transferase involved in peptidoglycan synthesis and fosfomycin resistance.
  Journal
J Bacteriol 185:1218-28 (2003)
DOI:10.1128/JB.185.4.1218-1228.2003
Reference
  Authors
JACOBSON B, DAVIDSON EA
  Title
Biosynthesis of uronic acids by skin enzymes. I. Uridine diphosphate-D-glucuronic acid-5-epimerase.
  Journal
J Biol Chem 237:638-42 (1962)
Reference
  Authors
Seifert GJ
  Title
Nucleotide sugar interconversions and cell wall biosynthesis: how to bring the inside to the outside.
  Journal
Curr Opin Plant Biol 7:277-84 (2004)
DOI:10.1016/j.pbi.2004.03.004
Reference
  Authors
Uehara T, Park JT
  Title
The N-acetyl-D-glucosamine kinase of Escherichia coli and its role in murein recycling.
  Journal
J Bacteriol 186:7273-9 (2004)
DOI:10.1128/JB.186.21.7273-7279.2004
Reference
  Authors
Breazeale SD, Ribeiro AA, McClerren AL, Raetz CR.
  Title
A formyltransferase required for polymyxin resistance in Escherichia coli and the modification of lipid A with 4-Amino-4-deoxy-L-arabinose. Identification and function oF UDP-4-deoxy-4-formamido-L-arabinose.
  Journal
J Biol Chem 280:14154-67 (2005)
DOI:10.1074/jbc.M414265200
Reference
  Authors
Williams GJ, Breazeale SD, Raetz CR, Naismith JH.
  Title
Structure and function of both domains of ArnA, a dual function decarboxylase and a formyltransferase, involved in 4-amino-4-deoxy-L-arabinose biosynthesis.
  Journal
J Biol Chem 280:23000-8 (2005)
DOI:10.1074/jbc.M501534200
Reference
  Authors
Maliekal P, Vertommen D, Delpierre G, Van Schaftingen E
  Title
Identification of the sequence encoding N-acetylneuraminate-9-phosphate phosphatase.
  Journal
Glycobiology 16:165-72 (2006)
DOI:10.1093/glycob/cwj050
Reference
  Authors
Campbell CT, Yarema KJ
  Title
Large-scale approaches for glycobiology.
  Journal
Genome Biol 6:236 (2005)
DOI:10.1186/gb-2005-6-11-236
Reference
  Authors
Uehara T, Suefuji K, Jaeger T, Mayer C, Park JT
  Title
MurQ Etherase is required by Escherichia coli in order to metabolize anhydro-N-acetylmuramic acid obtained either from the environment or from its own cell wall.
  Journal
J Bacteriol 188:1660-2 (2006)
DOI:10.1128/JB.188.4.1660-1662.2006
Reference
  Authors
Lanot A, Hodge D, Jackson RG, George GL, Elias L, Lim EK, Vaistij FE, Bowles DJ
  Title
The glucosyltransferase UGT72E2 is responsible for monolignol 4-O-glucoside production in Arabidopsis thaliana.
  Journal
Plant J 48:286-95 (2006)
DOI:10.1111/j.1365-313X.2006.02872.x
Reference
  Authors
Muller R, Morant M, Jarmer H, Nilsson L, Nielsen TH
  Title
Genome-wide analysis of the Arabidopsis leaf transcriptome reveals interaction of phosphate and sugar metabolism.
  Journal
Plant Physiol 143:156-71 (2007)
DOI:10.1104/pp.106.090167
Reference
  Authors
Oka T, Nemoto T, Jigami Y
  Title
Functional analysis of Arabidopsis thaliana RHM2/MUM4, a multidomain protein involved in UDP-D-glucose to UDP-L-rhamnose conversion.
  Journal
J Biol Chem 282:5389-403 (2007)
DOI:10.1074/jbc.M610196200
Reference
  Authors
Greenberg DE, Porcella SF, Zelazny AM, Virtaneva K, Sturdevant DE, Kupko JJ 3rd, Barbian KD, Babar A, Dorward DW, Holland SM.
  Title
Genome sequence analysis of the emerging human pathogenic acetic acid bacterium Granulibacter bethesdensis.
  Journal
J Bacteriol 189:8727-36 (2007)
DOI:10.1128/JB.00793-07
Reference
  Authors
Kotake T, Hojo S, Tajima N, Matsuoka K, Koyama T, Tsumuraya Y
  Title
A bifunctional enzyme with L-fucokinase and GDP-L-fucose pyrophosphorylase activities salvages free L-fucose in Arabidopsis.
  Journal
J Biol Chem 283:8125-35 (2008)
DOI:10.1074/jbc.M710078200
Reference
  Authors
Vorholter FJ, Schneiker S, Goesmann A, Krause L, Bekel T, Kaiser O, Linke B, Patschkowski T, Ruckert C, Schmid J, Sidhu VK, Sieber V, Tauch A, Watt SA, Weisshaar B, Becker A, Niehaus K, Puhler A.
  Title
The genome of Xanthomonas campestris pv. campestris B100 and its use for the reconstruction of metabolic pathways involved in xanthan biosynthesis.
  Journal
J Biotechnol 134:33-45 (2008)
DOI:10.1016/j.jbiotec.2007.12.013
Reference
  Authors
Albermann C, Piepersberg W
  Title
Expression and identification of the RfbE protein from Vibrio cholerae O1 and its use for the enzymatic synthesis of GDP-D-perosamine.
  Journal
Glycobiology 11:655-61 (2001)
DOI:10.1093/glycob/11.8.655
KO pathway
ko00520   

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