Proteoglycan biosynthesis (Homo sapiens)

From WikiPathways

Revision as of 01:06, 30 January 2020 by Rlee (Talk | contribs)
(diff) ←Older revision | Current revision (diff) | Newer revision→ (diff)
Jump to: navigation, search
43, 155, 9-11, 13...3, 1515151515, 9-11, 13...2, 78, 123, 1563, 156GlcUA-Gal-Gal-Xyl-proteinPG Core ProteinGolgi apparatusChondroitin sulfateCytoplasmExtracellular spaceNewly synthesizedproteoglycanGlycosylated proteoglycanProteoglycan core synthesized inRough Endoplasmic ReticulumnSulfur-containing amino acid metabolismSulfation ofproteoglycan14B3GAT3uridine diphosphateglucuronic acidUMPCSGALNACT1D-glucuronic acidXYLT2Sulfate ion (SO42-)SLC35B3XYLT1XyloseCHST14PAPSN-AcetylgalactosamineIMPAD1CANT1SLC35B2CHST3PAPSS2ADENOSINE MONOPHOSPHATEUdp xylosePhosphoadenosine phosphateUdp galactoseB3GALT6EXT2chlorideEXT1SLC26A2PO4(.2-)galactoseCHSY1EXTL3UDPB4GALT7Sulfate ion (SO42-)chloridePAPSgalactoseUdp galactoseGlcUA-Gal-Gal-Xyl-proteinN-AcetylgalactosamineGlcUA-Gal-Gal-Xyl-proteinN-acetylglucosaminesD-glucuronic acidD-glucuronic acidL-Iduronic acidN-AcetylgalactosamineN-AcetylgalactosamineN-acetylglucosaminesNewly synthesizedproteoglycanDermatan sulfateHeparan sulfatennOMIM:600972Achondrogenesis, Type IBOMIM:256050Atelosteogenesis, Type IIOMIM:615777Desbuquois Dysplasia 2OMIM:605822Spondyloocular SyndromeOMIM:612847Brachyolmia Type 4 With Mild Epiphyseal And Metaphyseal ChangesOMIM:150230Trichorhinophalangeal Syndrome, Type IIOMIM:614078Chondrodysplasia With Joint Dislocations, gPAPP TypeOMIM:222600Diastrophic DysplasiaOMIM:143095Spondyloepiphyseal Dysplasia With Congenital Joint DislocationsOMIM:130070Ehlers-Danlos Syndrome, Spondylodysplastic Type, 1OMIM:605282Temtamy Preaxial Brachydactyly SyndromeOMIM:616615CSGALNACT1 deficiency (joint dislocations and mild skeletal dysplasia)OMIM:226900Epiphyseal Dysplasia, Multiple, 4OMIM:133700Exostoses, Multiple, Type IOMIM:617719Epiphyseal Dysplasia, Multiple, 7OMIM:271640Spondyloepimetaphyseal Dysplasia With Joint Laxity, Type 1, With Or Without FracturesOMIM:251450Desbuquois Dysplasia 1OMIM:245600Multiple Joint Dislocations, Short Stature, And Craniofacial Dysmorphism With Or Without Congenital Heart DefectsOMIM:601776Ehlers-Danlos Syndrome, Musculocontractural Type, 1OMIM:617425Immunoskeletal Dysplasia With Neurodevelopmental Abnormalities


No description

Quality Tags

Ontology Terms



View all...
  1. Frederick JP, Tafari AT, Wu SM, Megosh LC, Chiou ST, Irving RP, York JD; ''A role for a lithium-inhibited Golgi nucleotidase in skeletal development and sulfation.''; Proc Natl Acad Sci U S A, 2008 PubMed Europe PMC Scholia
  2. Mihov D, Spiess M; ''Glycosaminoglycans: Sorting determinants in intracellular protein traffic.''; Int J Biochem Cell Biol, 2015 PubMed Europe PMC Scholia
  3. Mizumoto S, Yamada S, Sugahara K; ''Mutations in Biosynthetic Enzymes for the Protein Linker Region of Chondroitin/Dermatan/Heparan Sulfate Cause Skeletal and Skin Dysplasias.''; Biomed Res Int, 2015 PubMed Europe PMC Scholia
  4. Paganini C, Costantini R, Superti-Furga A, Rossi A; ''Bone and connective tissue disorders caused by defects in glycosaminoglycan biosynthesis: a panoramic view.''; FEBS J, 2019 PubMed Europe PMC Scholia
  5. Sato T, Gotoh M, Kiyohara K, Akashima T, Iwasaki H, Kameyama A, Mochizuki H, Yada T, Inaba N, Togayachi A, Kudo T, Asada M, Watanabe H, Imamura T, Kimata K, Narimatsu H; ''Differential roles of two N-acetylgalactosaminyltransferases, CSGalNAcT-1, and a novel enzyme, CSGalNAcT-2. Initiation and elongation in synthesis of chondroitin sulfate.''; J Biol Chem, 2003 PubMed Europe PMC Scholia
  6. Hästbacka J, Kaitila I, Sistonen P, de la Chapelle A; ''Diastrophic dysplasia gene maps to the distal long arm of chromosome 5.''; Proc Natl Acad Sci U S A, 1990 PubMed Europe PMC Scholia
  7. Prydz K; ''Determinants of Glycosaminoglycan (GAG)Structure.''; Biomolecules, 2015 PubMed Europe PMC Scholia
  8. Kamiyama S, Suda T, Ueda R, Suzuki M, Okubo R, Kikuchi N, Chiba Y, Goto S, Toyoda H, Saigo K, Watanabe M, Narimatsu H, Jigami Y, Nishihara S; ''Molecular cloning and identification of 3'-phosphoadenosine 5'-phosphosulfate transporter.''; J Biol Chem, 2003 PubMed Europe PMC Scholia
  9. Izumikawa T, Uyama T, Okuura Y, Sugahara K, Kitagawa H; ''Involvement of chondroitin sulfate synthase-3 (chondroitin synthase-2) in chondroitin polymerization through its interaction with chondroitin synthase-1 or chondroitin-polymerizing factor.''; Biochem J, 2007 PubMed Europe PMC Scholia
  10. Kitagawa H, Uyama T, Sugahara K; ''Molecular cloning and expression of a human chondroitin synthase.''; J Biol Chem, 2001 PubMed Europe PMC Scholia
  11. Uyama T, Kitagawa H, Tamura Ji J, Sugahara K; ''Molecular cloning and expression of human chondroitin N-acetylgalactosaminyltransferase: the key enzyme for chain initiation and elongation of chondroitin/dermatan sulfate on the protein linkage region tetrasaccharide shared by heparin/heparan sulfate.''; J Biol Chem, 2002 PubMed Europe PMC Scholia
  12. Kamiyama S, Sasaki N, Goda E, Ui-Tei K, Saigo K, Narimatsu H, Jigami Y, Kannagi R, Irimura T, Nishihara S; ''Molecular cloning and characterization of a novel 3'-phosphoadenosine 5'-phosphosulfate transporter, PAPST2.''; J Biol Chem, 2006 PubMed Europe PMC Scholia
  13. Izumikawa T, Koike T, Shiozawa S, Sugahara K, Tamura J, Kitagawa H; ''Identification of chondroitin sulfate glucuronyltransferase as chondroitin synthase-3 involved in chondroitin polymerization: chondroitin polymerization is achieved by multiple enzyme complexes consisting of chondroitin synthase family members.''; J Biol Chem, 2008 PubMed Europe PMC Scholia
  14. Vynios DH; ''Metabolism of cartilage proteoglycans inhealth and disease.''; Biomed Res Int, 2014 PubMed Europe PMC Scholia
  15. Mizumoto S, Yamada S, Sugahara K; ''Human genetic disorders and knockout mice deficient in glycosaminoglycan.''; Biomed Res Int, 2014 PubMed Europe PMC Scholia
  16. Kitagawa H, Izumikawa T, Uyama T, Sugahara K; ''Molecular cloning of a chondroitin polymerizing factor that cooperates with chondroitin synthase for chondroitin polymerization.''; J Biol Chem, 2003 PubMed Europe PMC Scholia


View all...
111633view02:45, 1 September 2020AzanklModified description
110986view14:55, 25 June 2020EgonwConverted three mim-conversions to Arrow (they're translocations)
110015view18:37, 12 April 2020EgonwReplaced secondary ChEBI identifiers with a primary identifiers.
109334view23:36, 13 March 2020KhanspersOntology Term : 'classic metabolic pathway' added !
108790view01:08, 30 January 2020RleeModified description
108789view01:06, 30 January 2020RleeNew pathway

External references


View all...
NameTypeDatabase referenceComment
B3GALT6GeneProductENSG00000176022 (Ensembl)
B3GAT3GeneProductENSG00000149541 (Ensembl)
B4GALT7GeneProductENSG00000027847 (Ensembl)
CANT1GeneProductENSG00000171302 (Ensembl)
CHST14GeneProductENSG00000169105 (Ensembl)
CHST3GeneProductENSG00000122863 (Ensembl)
CHSY1GeneProductENSG00000131873 (Ensembl)
CSGALNACT1GeneProductENSG00000147408 (Ensembl)
D-glucuronic acidMetaboliteCHEBI:4178 (ChEBI)
EXT1GeneProductENSG00000182197 (Ensembl)
EXT2GeneProductENSG00000151348 (Ensembl)
EXTL3GeneProductENSG00000012232 (Ensembl)
IMPAD1GeneProductENSG00000104331 (Ensembl)
L-Iduronic acidMetaboliteCHEBI:24769 (ChEBI)
N-AcetylgalactosamineMetaboliteCHEBI:7201 (ChEBI)
N-acetylglucosaminesMetaboliteCHEBI:59640 (ChEBI)
PAPSMetaboliteCHEBI:1353 (ChEBI)
PAPSS2GeneProductENSG00000198682 (Ensembl)
PO4(.2-)MetaboliteCHEBI:29932 (ChEBI)
Phosphoadenosine phosphateMetaboliteCHEBI:2475 (ChEBI)
SLC26A2GeneProductENSG00000155850 (Ensembl)
SLC35B2GeneProductENSG00000157593 (Ensembl)
SLC35B3GeneProductENSG00000124786 (Ensembl)
Sulfate ion (SO42-)MetaboliteCHEBI:16189 (ChEBI)
UDPMetaboliteCHEBI:9802 (ChEBI)
UMPMetaboliteCHEBI:9849 (ChEBI)
Udp galactoseMetaboliteCHEBI:18307 (ChEBI)
Udp xyloseMetaboliteCHEBI:16082 (ChEBI)
XYLT1GeneProductENSG00000103489 (Ensembl)
XYLT2GeneProductENSG00000015532 (Ensembl)
XyloseMetaboliteCHEBI:10085 (ChEBI)
chlorideMetaboliteCHEBI:13970 (ChEBI)
galactoseMetaboliteCHEBI:28260 (ChEBI)
uridine diphosphate glucuronic acidMetaboliteCHEBI:17200 (ChEBI)

Annotated Interactions

No annotated interactions

Personal tools