Exostosin-like 2

Details

Name
Exostosin-like 2
Kind
protein
Synonyms
  • 2.4.1.223
  • Alpha-1,4-N-acetylhexosaminyltransferase EXTL2
  • Alpha-GalNAcT EXTL2
  • EXT-related protein 2
  • EXTR2
  • Glucuronyl-galactosyl-proteoglycan 4-alpha-N-acetylglucosaminyltransferase
Gene Name
EXTL2
UniProtKB Entry
Q9UBQ6Swiss-Prot
Organism
Humans
NCBI Taxonomy ID
9606
Amino acid sequence
>lcl|BSEQ0013226|Exostosin-like 2
MRCCHICKLPGRVMGIRVLRLSLVVILVLLLVAGALTALLPSVKEDKMLMLRREIKSQGK
STMDSFTLIMQTYNRTDLLLKLLNHYQAVPNLHKVIVVWNNIGEKAPDELWNSLGPHPIP
VIFKQQTANRMRNRLQVFPELETNAVLMVDDDTLISTPDLVFAFSVWQQFPDQIVGFVPR
KHVSTSSGIYSYGSFEMQAPGSGNGDQYSMVLIGASFFNSKYLELFQRQPAAVHALIDDT
QNCDDIAMNFIIAKHIGKTSGIFVKPVNMDNLEKETNSGYSGMWHRAEHALQRSYCINKL
VNIYDSMPLRYSNIMISQFGFPYANYKRKI
Number of residues
330
Molecular Weight
37465.365
Theoretical pI
Not Available
GO Classification
Functions
glucuronylgalactosylproteoglycan 4-beta-N-acetylgalactosaminyltransferase activity / glycosaminoglycan binding / manganese ion binding
Components
cytosol / extracellular region / nucleoplasm
General Function
Glycosyltransferase required for the biosynthesis of heparan-sulfate and responsible for the alternating addition of beta-1-4-linked glucuronic acid (GlcA) and alpha-1-4-linked N-acetylglucosamine (GlcNAc) units to nascent heparan sulfate chains
Specific Function
alpha-1,4-N-acetylgalactosaminyltransferase activity
Pfam Domain Function
Signal Regions
Not Available
Transmembrane Regions
23-43
Cellular Location
Endoplasmic reticulum membrane
Gene sequence
>lcl|BSEQ0013227|Exostosin-like 2 (EXTL2)
ATGAGGTGTTGCCACATCTGCAAACTTCCTGGGAGAGTAATGGGGATTCGAGTGCTTCGA
TTATCTTTGGTGGTCATCCTCGTATTATTACTGGTAGCTGGTGCTTTGACTGCCTTACTT
CCCAGTGTTAAAGAAGACAAGATGCTCATGTTGCGTAGGGAAATAAAATCCCAGGGCAAG
TCCACCATGGACTCCTTTACTCTCATAATGCAGACGTACAACAGAACAGATCTCTTATTG
AAACTTTTAAATCATTATCAGGCTGTACCAAATCTGCACAAAGTGATTGTGGTATGGAAC
AATATTGGAGAGAAGGCACCAGATGAATTATGGAATTCTCTAGGGCCCCACCCTATCCCT
GTGATCTTCAAACAACAGACAGCAAACAGGATGAGAAATCGACTCCAGGTCTTTCCTGAA
CTGGAAACCAATGCAGTGTTGATGGTAGATGATGACACACTCATCAGCACCCCAGACCTT
GTTTTTGCTTTCTCAGTTTGGCAGCAATTTCCTGATCAAATTGTAGGATTTGTTCCTAGA
AAGCACGTCTCTACTTCATCAGGTATCTACAGTTATGGAAGTTTTGAAATGCAAGCACCA
GGGTCTGGAAATGGTGACCAGTACTCTATGGTGCTGATTGGAGCCTCATTCTTCAATAGC
AAATATCTTGAATTATTTCAGAGGCAACCTGCAGCTGTCCATGCTTTGATAGATGATACT
CAAAACTGTGATGATATTGCCATGAATTTTATCATTGCCAAGCATATTGGCAAGACTTCA
GGGATATTTGTGAAGCCTGTAAACATGGACAATTTGGAAAAAGAAACCAACAGTGGCTAT
TCTGGAATGTGGCATCGAGCTGAGCACGCTCTGCAGAGGTCTTATTGTATAAATAAGCTT
GTTAATATCTATGATAGCATGCCCTTAAGATACTCCAACATTATGATTTCCCAGTTTGGT
TTTCCATATGCCAACTACAAAAGAAAAATATAA
Chromosome Location
1
Locus
1p21.2
External Identifiers
ResourceLink
UniProtKB IDQ9UBQ6
UniProtKB Entry NameEXTL2_HUMAN
GeneCard IDEXTL2
HGNC IDHGNC:3516
KEGG IDhsa:2135
NCBI Gene ID2135
General References
  1. Wuyts W, Van Hul W, Hendrickx J, Speleman F, Wauters J, De Boulle K, Van Roy N, Van Agtmael T, Bossuyt P, Willems PJ: Identification and characterization of a novel member of the EXT gene family, EXTL2. Eur J Hum Genet. 1997 Nov-Dec;5(6):382-9. [Article]
  2. Saito T, Seki N, Yamauchi M, Tsuji S, Hayashi A, Kozuma S, Hori T: Structure, chromosomal location, and expression profile of EXTR1 and EXTR2, new members of the multiple exostoses gene family. Biochem Biophys Res Commun. 1998 Feb 4;243(1):61-6. [Article]
  3. McCormick C, Duncan G, Goutsos KT, Tufaro F: The putative tumor suppressors EXT1 and EXT2 form a stable complex that accumulates in the Golgi apparatus and catalyzes the synthesis of heparan sulfate. Proc Natl Acad Sci U S A. 2000 Jan 18;97(2):668-73. [Article]
  4. Kitagawa H, Shimakawa H, Sugahara K: The tumor suppressor EXT-like gene EXTL2 encodes an alpha1, 4-N-acetylhexosaminyltransferase that transfers N-acetylgalactosamine and N-acetylglucosamine to the common glycosaminoglycan-protein linkage region. The key enzyme for the chain initiation of heparan sulfate. J Biol Chem. 1999 May 14;274(20):13933-7. [Article]
  5. Ota T, Suzuki Y, Nishikawa T, Otsuki T, Sugiyama T, Irie R, Wakamatsu A, Hayashi K, Sato H, Nagai K, Kimura K, Makita H, Sekine M, Obayashi M, Nishi T, Shibahara T, Tanaka T, Ishii S, Yamamoto J, Saito K, Kawai Y, Isono Y, Nakamura Y, Nagahari K, Murakami K, Yasuda T, Iwayanagi T, Wagatsuma M, Shiratori A, Sudo H, Hosoiri T, Kaku Y, Kodaira H, Kondo H, Sugawara M, Takahashi M, Kanda K, Yokoi T, Furuya T, Kikkawa E, Omura Y, Abe K, Kamihara K, Katsuta N, Sato K, Tanikawa M, Yamazaki M, Ninomiya K, Ishibashi T, Yamashita H, Murakawa K, Fujimori K, Tanai H, Kimata M, Watanabe M, Hiraoka S, Chiba Y, Ishida S, Ono Y, Takiguchi S, Watanabe S, Yosida M, Hotuta T, Kusano J, Kanehori K, Takahashi-Fujii A, Hara H, Tanase TO, Nomura Y, Togiya S, Komai F, Hara R, Takeuchi K, Arita M, Imose N, Musashino K, Yuuki H, Oshima A, Sasaki N, Aotsuka S, Yoshikawa Y, Matsunawa H, Ichihara T, Shiohata N, Sano S, Moriya S, Momiyama H, Satoh N, Takami S, Terashima Y, Suzuki O, Nakagawa S, Senoh A, Mizoguchi H, Goto Y, Shimizu F, Wakebe H, Hishigaki H, Watanabe T, Sugiyama A, Takemoto M, Kawakami B, Yamazaki M, Watanabe K, Kumagai A, Itakura S, Fukuzumi Y, Fujimori Y, Komiyama M, Tashiro H, Tanigami A, Fujiwara T, Ono T, Yamada K, Fujii Y, Ozaki K, Hirao M, Ohmori Y, Kawabata A, Hikiji T, Kobatake N, Inagaki H, Ikema Y, Okamoto S, Okitani R, Kawakami T, Noguchi S, Itoh T, Shigeta K, Senba T, Matsumura K, Nakajima Y, Mizuno T, Morinaga M, Sasaki M, Togashi T, Oyama M, Hata H, Watanabe M, Komatsu T, Mizushima-Sugano J, Satoh T, Shirai Y, Takahashi Y, Nakagawa K, Okumura K, Nagase T, Nomura N, Kikuchi H, Masuho Y, Yamashita R, Nakai K, Yada T, Nakamura Y, Ohara O, Isogai T, Sugano S: Complete sequencing and characterization of 21,243 full-length human cDNAs. Nat Genet. 2004 Jan;36(1):40-5. Epub 2003 Dec 21. [Article]

Associated Data

Drug Relations
DrugDrug groupPharmacological action?TypeActionsDetails
Uridine-Diphosphate-N-AcetylgalactosamineexperimentalunknowntargetDetails
Uridine-Diphosphate-N-AcetylglucosamineexperimentalunknowntargetDetails
Uridine-5'-DiphosphateexperimentalunknowntargetDetails