Name: Anti-EXT2 Antibody (CAB1973)
Brand: Cell Biology Antibodies 8
SKU: CAB1973
Availability: OutOfStock

Antibody Name:	Anti-EXT2 Antibody
Antibody SKU:	CAB1973
Antibody Size:	20uL, 50uL, 100uL
Application:	WB IF
Reactivity:	Human, Mouse, Rat
Host Species:	Rabbit
Immunogen:	Recombinant fusion protein containing a sequence corresponding to amino acids 80-340 of human EXT2 (NP_000392.3).

Application:	WB IF
Recommended Dilution:	WB 1:500 - 1:2000 IF 1:50 - 1:200
Reactivity:	Human, Mouse, Rat
Positive Samples:	U-87MG, HeLa, SKOV3, SW480, HT-1080, Mouse liver, Rat kidney

Immunogen:	Recombinant fusion protein containing a sequence corresponding to amino acids 80-340 of human EXT2 (NP_000392.3).
Purification Method:	Affinity purification
Storage Buffer:	Store at -20'C. Avoid freeze / thaw cycles. Buffer: PBS with 0.02% sodium azide, 50% glycerol, pH7.3.
Isotype:	IgG
Sequence:	PHSI ESSN DWNV EKRS IRDV PVVR LPAD SPIP ERGD LSCR MHTC FDVY RCGF NPKN KIKV YIYA LKKY VDDF GVSV SNTI SREY NELL MAIS DSDY YTDD INRA CLFV PSID VLNQ NTLR IKET AQAM AQLS RWDR GTNH LLFN MLPG GPPD YNTA LDVP RDRA LLAG GGFS TWTY RQGY DVSI PVYS PLSA EVDL PEKG PGPR QYFL LSSQ VGLH PEYR EDLE ALQV KHGE SVLV LDKC TNLS EGVL SVRK RCHK HQVF D
Gene ID:	2132
Uniprot:	Q93063
Cellular Location:	Endoplasmic reticulum membrane, Golgi apparatus membrane, Single-pass type II membrane protein
Calculated MW:	82kDa/83kDa/85kDa
Observed MW:	82kDa

Synonyms:	EXT2, SOTV, SSMS
Background:	This gene encodes one of two glycosyltransferases involved in the chain elongation step of heparan sulfate biosynthesis. Mutations in this gene cause the type II form of multiple exostoses. Alternatively spliced transcript variants encoding different isoforms have been noted for this gene.

UniProt Protein Function:	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.
UniProt Protein Details:	Protein type:Glycan Metabolism - heparan sulfate biosynthesis; Transferase; EC 2.4.1.225; Membrane protein, integral; EC 2.4.1.224; Tumor suppressor Chromosomal Location of Human Ortholog: 11p12-p11 Cellular Component: endoplasmic reticulum; endoplasmic reticulum membrane; Golgi apparatus; Golgi membrane; membrane Molecular Function:acetylglucosaminyltransferase activity; glucuronosyltransferase activity; protein heterodimerization activity; protein homodimerization activity; transferase activity, transferring glycosyl groups Biological Process: cellular polysaccharide biosynthetic process; glycosaminoglycan biosynthetic process; heparan sulfate proteoglycan biosynthetic process; heparan sulfate proteoglycan biosynthetic process, polysaccharide chain biosynthetic process; ossification; signal transduction Disease: Exostoses, Multiple, Type Ii
NCBI Summary:	This gene encodes one of two glycosyltransferases involved in the chain elongation step of heparan sulfate biosynthesis. Mutations in this gene cause the type II form of multiple exostoses. Alternatively spliced transcript variants encoding different isoforms have been noted for this gene. [provided by RefSeq, Jul 2008]
UniProt Code:	Q93063
NCBI GenInfo Identifier:	3023739
NCBI Gene ID:	2132
NCBI Accession:	Q93063.1
UniProt Secondary Accession:	Q93063,O15288, B2R5Z6, C9JU51, J3KPT2,
UniProt Related Accession:	Q93063
Molecular Weight:	85,815 Da
NCBI Full Name:	Exostosin-2
NCBI Synonym Full Names:	exostosin glycosyltransferase 2
NCBI Official Symbol:	EXT2
NCBI Official Synonym Symbols:	SOTV; SSMS
NCBI Protein Information:	exostosin-2
UniProt Protein Name:	Exostosin-2
UniProt Synonym Protein Names:	Glucuronosyl-N-acetylglucosaminyl-proteoglycan/N-acetylglucosaminyl-proteoglycan 4-alpha-N-acetylglucosaminyltransferase; Multiple exostoses protein 2; Putative tumor suppressor protein EXT2
Protein Family:	Extensin
UniProt Gene Name:	EXT2
UniProt Entry Name:	EXT2_HUMAN

	Western blot analysis of extracts of various cell lines, using EXT2 antibody (CAB1973) at 1:1000 dilution. Secondary antibody: HRP Goat Anti-Rabbit IgG (H+L) (CABS014) at 1:10000 dilution. Lysates/proteins: 25ug per lane. Blocking buffer: 3% nonfat dry milk in TBST. Detection: ECL Basic Kit (RM00020). Exposure time: 30s.
	Immunofluorescence analysis of L929 cells using EXT2 Rabbit pAb (CAB1973) at dilution of 1:100 (40x lens). Blue: DAPI for nuclear staining.