Anti-EXT1 Antibody | MyBioSource MBS8245727 product information

product summary

company name :

MyBioSource

product type :

antibody

product name :

Anti-EXT1 Antibody

catalog :

MBS8245727

quantity :

0.05 mL

price :

175 USD

clonality :

polyclonal

host :

rabbit

conjugate :

nonconjugated

reactivity :

human, mouse, rat

application :

western blot

more info or order :

MyBioSource product webpage

image

image 1 :

Western blot analysis of EXT1 expression in K562 (A), PC3 (B), mouse liver (C), mouse lung (D) whole cell lysates.

product information

catalog number :

MBS8245727

products type :

Antibody

products full name :

Anti-EXT1 Antibody

products short name :

[EXT1]

products name syn :

[Exostosin-1; Glucuronosyl-N-acetylglucosaminyl-proteoglycan/N-acetylglucosaminyl-proteoglycan 4-alpha-N-acetylglucosaminyltransferase; Multiple exostoses protein 1; Putative tumor suppressor protein EXT1]

other names :

[exostosin-1; Exostosin-1; exostosin-1; exostosin glycosyltransferase 1; Glucuronosyl-N-acetylglucosaminyl-proteoglycan/N-acetylglucosaminyl-proteoglycan 4-alpha-N-acetylglucosaminyltransferase; Multiple exostoses protein 1; Putative tumor suppressor protein EXT1]

products gene name :

[EXT1]

other gene names :

[EXT1; EXT1; EXT; LGS; TTV; LGCR; TRPS2]

uniprot entry name :

EXT1_HUMAN

clonality :

Polyclonal

host :

Rabbit

reactivity :

Human, Mouse, Rat

sequence length :

746

specificity :

Recognizes endogenous levels of EXT1 protein.

purity :

The antibody was purified by immunogen affinity chromatography.

form :

Liquid in 0.42% Potassium phosphate, 0.87% Sodium chloride, pH 7.3, 30% glycerol, and 0.01% sodium azide.

storage stability :

Shipped at 4 degree C. Upon delivery aliquot and store at -20 degree C for one year. Avoid freeze/thaw cycles.

tested application :

Western Blot (WB)

app notes :

WB: 1/500 - 1/2000

image1 heading :

Western Blot (WB)

other info1 :

Immunogen: Recombinant full length protein of human EXT1

products description :

Rabbit polyclonal antibody to EXT1

ncbi gi num :

46370066

ncbi acc num :

NP_000118.2

ncbi gb acc num :

NM_000127.2

uniprot acc num :

Q16394

ncbi mol weight :

86,255 Da

ncbi pathways :

Glycosaminoglycan Biosynthesis - Heparan Sulfate / Heparin Pathway (82984); Glycosaminoglycan Biosynthesis - Heparan Sulfate / Heparin Pathway (358); Glycosaminoglycan Biosynthesis, Heparan Sulfate Backbone Pathway (413365); Glycosaminoglycan Biosynthesis, Heparan Sulfate Backbone Pathway (468252); Glycosaminoglycan Metabolism Pathway (1269972); HS-GAG Biosynthesis Pathway (1269982); Heparan Sulfate/heparin (HS-GAG) Metabolism Pathway (1269980); Metabolic Pathways (132956); Metabolism Pathway (1269956); Metabolism Of Carbohydrates Pathway (1269957)

ncbi summary :

This gene encodes an endoplasmic reticulum-resident type II transmembrane glycosyltransferase involved in the chain elongation step of heparan sulfate biosynthesis. Mutations in this gene cause the type I form of multiple exostoses. [provided by RefSeq, Jul 2008]

uniprot summary :

EXT1: 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 EXT1 are a cause of hereditary multiple exostoses type 1 (EXT1). 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 EXT1 are a cause of tricho-rhino-phalangeal syndrome type 2 (TRPS2). A syndrome that combines the clinical features of trichorhinophalangeal syndrome type 1 and multiple exostoses type 1. Affected individuals manifest multiple dysmorphic facial features including large, laterally protruding ears, a bulbous nose, an elongated upper lip, as well as sparse scalp hair, winged scapulae, multiple cartilaginous exostoses, redundant skin, and mental retardation. A chromosomal aberration resulting in the loss of functional copies of TRPS1 and EXT1 has been found in TRPS2 patients. Defects in EXT1 are a cause of chondrosarcoma (CHDSA). It is a malignant neoplasm derived from cartilage cells. Chondrosarcomas range from slow-growing non-metastasizing lesions to highly aggressive metastasizing sarcomas. Belongs to the glycosyltransferase 47 family. Protein type: Motility/polarity/chemotaxis; EC 2.4.1.224; Transferase; Tumor suppressor; EC 2.4.1.225; Membrane protein, integral; Glycan Metabolism - heparan sulfate biosynthesis. Chromosomal Location of Human Ortholog: 8q24.11. Cellular Component: endoplasmic reticulum; endoplasmic reticulum membrane; Golgi apparatus; Golgi membrane; integral to endoplasmic reticulum membrane; integral to membrane. Molecular Function: acetylglucosaminyltransferase activity; glucuronosyl-N-acetylglucosaminyl-proteoglycan 4-alpha-N-acetylglucosaminyltransferase activity; glucuronosyltransferase activity; heparan sulfate N-acetylglucosaminyltransferase activity; metal ion binding; N-acetylglucosaminyl-proteoglycan 4-beta-glucuronosyltransferase activity; protein heterodimerization activity; protein homodimerization activity; transferase activity, transferring glycosyl groups. Biological Process: axon guidance; cellular polysaccharide biosynthetic process; endoderm development; gastrulation; glycosaminoglycan biosynthetic process; heparan sulfate proteoglycan biosynthetic process; heparan sulfate proteoglycan biosynthetic process, polysaccharide chain biosynthetic process; mesoderm development; olfactory bulb development; ossification; protein amino acid glycosylation; signal transduction; skeletal development. Disease: Chondrosarcoma; Exostoses, Multiple, Type I

size1 :

0.05 mL

price1 :

175 USD

size2 :

0.1 mL

price2 :

220

size3 :

0.2 mL

price3 :

295

more info or order :

MyBioSource product webpage