Thermo Fisher Scientific EXT1 Monoclonal Antibody (5A5)

Applications

Tested Dilution

Publications

Western Blot (WB)

1-5 µg/mL

-

ELISA (ELISA)

3 ng/mL

-

Product Specifications

Species Reactivity

Human

Host/Isotype

Mouse / IgG2a, kappa

Class

Monoclonal

Type

Antibody

Clone

5A5

Immunogen

EXT1 (AAH01174, 246 a.a. approximately 345 a.a) partial recombinant protein with GST tag. MW of the GST tag alone is 26 KDa. if (typeof window.$mangular === undefined || !window.$mangular) { window.$mangular = {}; } $mangular.antigenJson = \[{targetFamily:EXT1,uniProtId:Q16394-1,ncbiNodeId:9606,antigenRange:246-345,antigenLength:746,antigenImageFileName:H00002131-M01_EXT1_Q16394-1_House_mouse.svg,antigenImageFileNamePDP:H00002131-M01_EXT1_Q16394-1_House_mouse_PDP.jpeg,sortOrder:1}\]; $mangular.isB2BCMGT = false; $mangular.isEpitopesModalImageMultiSizeEnabled = true;

View immunogen .st0{fill:#FFFFFF;} .st1{fill:#1E8AE7;}

Conjugate

Unconjugated Unconjugated Unconjugated

Form

Liquid

Concentration

See Label

Purification

Affinity chromatography

Storage buffer

PBS, pH 7.4

Contains

no preservative

Storage conditions

-20° C, Avoid Freeze/Thaw Cycles

Shipping conditions

Ambient (domestic); Wet ice (international)

Product Specific Information

Sequence of this protein is as follows: GERGFLKFNT IPPLRKYMLV FKGKRYLTGI GSDTRNALYH VHNGEDVVLL TTCKHGKDWQ KHKDSRCDRD NTEYEKYDYR EMLHNATFCL VPRGRRLGSF

Target Information

Hereditary multiple exostoses (HME) is an autosomal dominant disorder characterized by the formation of exostoses (EXT), which are cartilage-capped bony protuberances mainly located on long bones. Two proteins associated with EXT, EXT1 and EXT2, form homo/heteromeric complexes in vivo, which leads to the accumulation of both proteins in the Golgi apparatus. EXT1 and EXT2 are endoplasmic reticulum-localized type II transmembrane glycoproteins that possess, or are tightly associated with, glycosyltransferase activities involved in the polymerization of the glycosaminoglycan, heparan sulfate (HS). EXT2 is a protein that harbors the D-glucuronyl (GlcA) and N-acetyl-D-glucosaminyl (GlcNAc) transferase activities required for biosynthesis of HS. EXT1 rescues defective HS biosynthesis and elevates low GlcA and GlcNAc transferase levels in mutated cells.

For Research Use Only. Not for use in diagnostic procedures. Not for resale without express authorization.