Thermo Fisher Scientific EXT1 Monoclonal Antibody (5A5)
Thermo Fisher Scientific의 EXT1 Monoclonal Antibody (Clone 5A5)는 인간 EXT1 단백질을 인식하는 mouse IgG2a 항체로, Western blot 및 ELISA에 최적화되어 있습니다. 액상 형태로 제공되며, PBS buffer에 보존제가 포함되지 않습니다. 연구용으로만 사용 가능합니다.
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Applications and Tested Dilution
| Application | Tested Dilution |
|---|---|
| Western Blot (WB) | 1–5 µg/mL |
| ELISA | 3 ng/mL |
Product Specifications
| 항목 | 내용 |
|---|---|
| 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. |
| Conjugate | 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 harbors D-glucuronyl (GlcA) and N-acetyl-D-glucosaminyl (GlcNAc) transferase activities required for HS biosynthesis. 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.
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