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The exostosin family: proteins with many functions.

Busse-Wicher M, Wicher KB, Kusche-Gullberg M.

Matrix Biol. 2014 Apr;35:25-33. doi: 10.1016/j.matbio.2013.10.001. Epub 2013 Oct 12. Review.


Etiological point mutations in the hereditary multiple exostoses gene EXT1: a functional analysis of heparan sulfate polymerase activity.

Cheung PK, McCormick C, Crawford BE, Esko JD, Tufaro F, Duncan G.

Am J Hum Genet. 2001 Jul;69(1):55-66. Epub 2001 Jun 5.


Contribution of EXT1, EXT2, and EXTL3 to heparan sulfate chain elongation.

Busse M, Feta A, Presto J, Wilén M, Grønning M, Kjellén L, Kusche-Gullberg M.

J Biol Chem. 2007 Nov 9;282(45):32802-10. Epub 2007 Aug 29.


The putative tumor suppressors EXT1 and EXT2 are glycosyltransferases required for the biosynthesis of heparan sulfate.

Lind T, Tufaro F, McCormick C, Lindahl U, Lidholt K.

J Biol Chem. 1998 Oct 9;273(41):26265-8.


Hereditary multiple exostoses and heparan sulfate polymerization.

Zak BM, Crawford BE, Esko JD.

Biochim Biophys Acta. 2002 Dec 19;1573(3):346-55. Review.


Human tumor suppressor EXT gene family members EXTL1 and EXTL3 encode alpha 1,4- N-acetylglucosaminyltransferases that likely are involved in heparan sulfate/ heparin biosynthesis.

Kim BT, Kitagawa H, Tamura J, Saito T, Kusche-Gullberg M, Lindahl U, Sugahara K.

Proc Natl Acad Sci U S A. 2001 Jun 19;98(13):7176-81. Epub 2001 Jun 5.


Distinct and collaborative roles of Drosophila EXT family proteins in morphogen signalling and gradient formation.

Han C, Belenkaya TY, Khodoun M, Tauchi M, Lin X, Lin X.

Development. 2004 Apr;131(7):1563-75. Epub 2004 Mar 3.


Mutation in the heparan sulfate biosynthesis enzyme EXT1 influences growth factor signaling and fibroblast interactions with the extracellular matrix.

Osterholm C, Barczyk MM, Busse M, Grønning M, Reed RK, Kusche-Gullberg M.

J Biol Chem. 2009 Dec 11;284(50):34935-43. doi: 10.1074/jbc.M109.005264. Epub 2009 Oct 22.


Heparan sulfate polymerization in Drosophila.

Izumikawa T, Egusa N, Taniguchi F, Sugahara K, Kitagawa H.

J Biol Chem. 2006 Jan 27;281(4):1929-34. Epub 2005 Nov 21.


Reduced Expression of EXTL2, a Member of the Exostosin (EXT) Family of Glycosyltransferases, in Human Embryonic Kidney 293 Cells Results in Longer Heparan Sulfate Chains.

Katta K, Imran T, Busse-Wicher M, Grønning M, Czajkowski S, Kusche-Gullberg M.

J Biol Chem. 2015 May 22;290(21):13168-77. doi: 10.1074/jbc.M114.631754. Epub 2015 Mar 31.


In vitro polymerization of heparan sulfate backbone by the EXT proteins.

Busse M, Kusche-Gullberg M.

J Biol Chem. 2003 Oct 17;278(42):41333-7. Epub 2003 Aug 7.


Location of the glucuronosyltransferase domain in the heparan sulfate copolymerase EXT1 by analysis of Chinese hamster ovary cell mutants.

Wei G, Bai X, Gabb MM, Bame KJ, Koshy TI, Spear PG, Esko JD.

J Biol Chem. 2000 Sep 8;275(36):27733-40.


EXT genes are differentially expressed in bone and cartilage during mouse embryogenesis.

Stickens D, Brown D, Evans GA.

Dev Dyn. 2000 Jul;218(3):452-64.


The EXT1/EXT2 tumor suppressors: catalytic activities and role in heparan sulfate biosynthesis.

Senay C, Lind T, Muguruma K, Tone Y, Kitagawa H, Sugahara K, Lidholt K, Lindahl U, Kusche-Gullberg M.

EMBO Rep. 2000 Sep;1(3):282-6.


A direct interaction between EXT proteins and glycosyltransferases is defective in hereditary multiple exostoses.

Simmons AD, Musy MM, Lopes CS, Hwang LY, Yang YP, Lovett M.

Hum Mol Genet. 1999 Nov;8(12):2155-64.


Transgenic expression of the EXT2 gene in developing chondrocytes enhances the synthesis of heparan sulfate and bone formation in mice.

Morimoto K, Shimizu T, Furukawa K, Morio H, Kurosawa H, Shirasawa T.

Biochem Biophys Res Commun. 2002 Apr 12;292(4):999-1009.


Biosynthesis of heparan sulfate in EXT1-deficient cells.

Okada M, Nadanaka S, Shoji N, Tamura J, Kitagawa H.

Biochem J. 2010 May 27;428(3):463-71. doi: 10.1042/BJ20100101.


Demonstration of a novel gene DEXT3 of Drosophila melanogaster as the essential N-acetylglucosamine transferase in the heparan sulfate biosynthesis: chain initiation and elongation.

Kim BT, Kitagawa H, Tamura Ji J, Kusche-Gullberg M, Lindahl U, Sugahara K.

J Biol Chem. 2002 Apr 19;277(16):13659-65. Epub 2002 Feb 6.

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