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Article

EXTL2, a member of the EXT family of tumor suppressors, controls glycosaminoglycan biosynthesis in a xylose kinase-dependent manner.

The Journal of biological chemistry - 29 Mar 2013

Nadanaka Satomi, Zhou Shaobo, Kagiyama Shoji, Shoji Naoko, Sugahara Kazuyuki, Sugihara Kazushi, Asano Masahide, Kitagawa Hiroshi

Abstract excerpt

Mutant alleles of EXT1 or EXT2, two members of the EXT gene family, are causative agents in hereditary multiple exostoses, and their gene products function together as a polymerase in the biosynthesis of heparan sulfate. EXTL2, one of three EXT-like genes in the human genome that are homologous to EXT1 and EXT2, encodes a transferase that adds not only GlcNAc but also N-acetylgalactosamine to the...

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Share to Gene Expression Regulation, Enzymologic
Share to Genes, Tumor Suppressor

Methodology Data sources Editorial policy Privacy About Contact Helena Bioinformatics

Share to Glycosaminoglycans

Share to Glycosyltransferases

Share to N-Acetylglucosaminyltransferases

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Source

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Identifiers

PMID: 23395820
DOI: 10.1074/jbc.M112.416909
PMCID: PMC3611003

Genes

EXTL2
EXT1
EXT2

Provenance

PubMed metadata and Literature Corpus relationships. Corpus snapshot updated 7 Sept 2026. Manifest publication-graph-cohort-2026-09-07.1.

Source receipt: pubmed, retrieved 7 Sept 2026.

Related research

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Research trail→

Click a neighbor to travelStep 1 · 12 closest

EXTL2, a member of the EXT family of tumor suppressors, controls glycosaminoglycan biosynthesis in a xylose kinase-dependent manner.PMID 23395820Read ↗

The putative tumor suppressors EXT1 and EXT2 form a stable complex that accumulates in the Golgi apparatus and catalyzes the synthesis of heparan sulfate.Semantic proximity Read ↗

Embryonic fibroblasts with a gene trap mutation in Ext1 produce short heparan sulfate chains.Semantic proximity Read ↗

A direct interaction between EXT proteins and glycosyltransferases is defective in hereditary multiple exostoses.Semantic proximity Read ↗

Contribution of EXT1, EXT2, and EXTL3 to heparan sulfate chain elongation.Semantic proximity Read ↗

A novel mutation in ext2 caused hereditary multiple exostoses through reducing the synthesis of heparan sulfateSemantic proximity Read ↗

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

Hereditary multiple exostoses and heparan sulfate polymerization.Semantic proximity Read ↗

EXT genes are differentially expressed in bone and cartilage during mouse embryogenesisSemantic proximity Read ↗

A splice mutation and mRNA decay of EXT2 provoke hereditary multiple exostoses.Semantic proximity Read ↗

Identification of four novel EXT1 and EXT2 mutations in five Chinese pedigrees with hereditary multiple exostoses.Semantic proximity Read ↗

Identification and Characterization of a Novel Member of the EXT Gene Family, EXTL2Semantic proximity Read ↗

Mice deficient in Ext2 lack heparan sulfate and develop exostosesSemantic proximity Read ↗

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