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Protein Page:
EXT2 (human)
p Phosphorylation
ac Acetylation
me Methylation
m1 Mono-methylation
m2 Di-methylation
m3 Tri-methylation
ub Ubiquitination
sm Sumoylation
ne Neddylation
gl O-GlcNAc
ga O-GalNAc
pa Palmitoylation
ad Adenylylation
sn S-Nitrosylation
ca Caspase cleavage
sc Succinylation

Overview
EXT2 Glycosyltransferase required for the biosynthesis of heparan-sulfate. The EXT1/EXT2 complex possesses substantially higher glycosyltransferase activity than EXT1 or EXT2 alone. Appears to be a tumor suppressor. Defects in EXT2 are a cause of hereditary multiple exostoses type 2 (EXT2). EXT is a genetically heterogeneous bone disorder caused by genes segregating on human chromosomes 8, 11, and 19 and designated EXT1, EXT2 and EXT3 respectively. EXT is a dominantly inherited skeletal disorder primarily affecting endochondral bone during growth. The disease is characterized by formation of numerous cartilage-capped, benign bone tumors (osteocartilaginous exostoses or osteochondromas) that are often accompanied by skeletal deformities and short stature. In a small percentage of cases exostoses have exhibited malignant transformation resulting in an osteosarcoma or chondrosarcoma. Osteochondromas development can also occur as a sporadic event. Defects in EXT2 are a cause of Potocki-Shaffer syndrome (POSHS). It is a contiguous gene syndrome due to proximal deletion of chromosome 11p11.2, including EXT2 and ALX4. Belongs to the glycosyltransferase 47 family. 2 isoforms of the human protein are produced by alternative splicing. Note: This description may include information from UniProtKB.
Protein type: Transferase; Membrane protein, integral; Glycan Metabolism - heparan sulfate biosynthesis; EC 2.4.1.224; EC 2.4.1.225
Cellular Component: Golgi membrane; intrinsic to endoplasmic reticulum membrane; Golgi apparatus; endoplasmic reticulum membrane; membrane; endoplasmic reticulum; integral to membrane
Molecular Function: acetylglucosaminyltransferase activity; transferase activity, transferring glycosyl groups; protein homodimerization activity; glucuronosyl-N-acetylglucosaminyl-proteoglycan 4-alpha-N-acetylglucosaminyltransferase activity; glucuronosyltransferase activity; protein heterodimerization activity; N-acetylglucosaminyl-proteoglycan 4-beta-glucuronosyltransferase activity; heparan sulfate N-acetylglucosaminyltransferase activity
Biological Process: glycosaminoglycan biosynthetic process; cellular polysaccharide biosynthetic process; ossification; heparan sulfate proteoglycan biosynthetic process, polysaccharide chain biosynthetic process; glycosaminoglycan metabolic process; mesoderm formation; heparan sulfate proteoglycan biosynthetic process; carbohydrate metabolic process; protein amino acid glycosylation; cell differentiation; signal transduction
Reference #:  Q93063 (UniProtKB)
Alt. Names/Synonyms: exostoses (multiple) 2; Exostosin-2; EXT2; Glucuronosyl-N-acetylglucosaminyl-proteoglycan 4-alpha-N- acetylglucosaminyltransferase; Glucuronosyl-N-acetylglucosaminyl-proteoglycan/N-acetylglucosaminyl-proteoglycan 4-alpha-N-acetylglucosaminyltransferase; Multiple exostoses protein 2; N-acetylglucosaminyl-proteoglycan 4-beta-glucuronosyltransferase; Putative tumor suppressor protein EXT2; SOTV
Gene Symbols: EXT2
Molecular weight: 82,255 Da
Basal Isoelectric point: 6.12  Predict pI for various phosphorylation states
Select Structure to View Below

EXT2

Protein Structure Not Found.


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Modification Sites and Domains Show Modification Legend
Click here to view phosphorylation modifications only

Modification Sites in Parent Protein, Orthologs, and Isoforms Show Modification Legend
 

Show Multiple Sequence Alignment


 SS 

SS: The number of records in which this modification site was determined using site-specific methods. SS methods include amino acid sequencing, site-directed mutagenesis, modification site-specific antibodies, specific MS strategies, etc.


 MS 

MS: The number of records in which this modification site was assigned using ONLY proteomic discovery-mode mass spectrometry.


       human

 
0 1 S75-p VVRLPADsPIPERGD
0 1 K168-ub NQNTLRIkETAQAMA
0 1 K245-ub AEVDLPEkGPGPRQY
0 1 K275-ub DLEALQVkHGESVLV
0 1 K369-ub SVVVPEEkMSDVYSI
0 1 K478-ub RVITEVSkVPSLSkL
0 1 K484-ub SkVPSLSkLLVVWNN
0 1 K494-ub VVWNNQNkNPPEDSL
0 1 K518-ub VVRTAENkLSNRFFP
0 1 K690-ub VFGTMPLkVVEHRAD
  mouse

 
S75 VVRLPTDSPIPERGD
K168 NQNPLRIKETAQALA
K245 AEMALPEKAPGPRRY
K275 ELEALQAKHQESVLV
K369 SVVVPEEKMSDVYSI
K478 RVITEVSKVPSLSKL
K484 SKVPSLSKLLVVWNN
K494 VVWNNQNKNPPEESL
K518 VVRTAENKLSNRFFP
K690 VFGTMPLKVVEHRAD
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