Manufacturer |
GENWAY
|
Category |
|
Type |
Antibody |
Specific against |
other |
Clone |
450-9D |
Applications |
FC |
Amount |
0.025 mg |
Item no. |
20-783-310098 |
eClass 6.1 |
32160702 |
eClass 9.0 |
32160702 |
Available |
|
Genway ID: |
GWB-DF5C70 |
Specificity: |
CD104NCBI |
Gene ID: |
3691 |
Specificity: |
CD104 |
Clone: |
450-9D |
Immunogen: |
Purified a6b4 integrin from A431 cells |
Fusion Partner: |
Spleen cells from immunised BALB/c mice were fused with cells of the mouse Sp2/0 myeloma cell line. |
Preparation: |
Purified IgG prepared by affinity chromatography on Protein G from tissue culture supernatant |
Buffer Solution: |
Phosphate buffered saline pH7. 4 |
|
Preservative Stabilisers: |
| 0. 09%Sodium Azide1%Bovine Serum AlbuminSuggested |
Flow Cytometry: |
Use 10ul of the suggested working dilution to label 106 cells in 100ul. Suggested |
Dilution: |
Flow Cytometry - Neat - 1/10 |
Function: |
Integrin alpha-6/beta-4 is a receptor for laminin. It plays a critical structural role in the hemidesmosome of epithelial cells. |
Subunit: |
Heterodimer of an alpha and a beta subunit. Beta-4 associates with alpha-6. |
Subcellular Location: |
Membrane; Single-pass type I membrane protein. |
Tissue Specificity: |
Integrin alpha-6/beta-4 is predominantly expressed by epithelia. Isoform beta-4D is also expressed in colon and placenta. Isoform beta-4E is also expressed in epidermis lung duodenum heart spleen and stomach. |
Domain: |
The fibronectin type-III-like domains bind BPAG1 and plectin and probably also recruit BP230. |
Disease: |
Defects in ITGB4 are a cause of epidermolysis bullosa letalis with pyloric atresia (EB-PA) [MIM:226730]; also known as junctional epidermolysis bullosa with pyloric atresia (PA-JEB) or aplasia cutis congenita with gastrointestinal atresia. EB-PA is an autosomal recessive frequently lethal epidermolysis bullosa with variable involvement of skin nails mucosa and with variable effects on the digestive system. It is characterized by mucocutaneous fragility aplasia cutis congenita and gastrointestinal atresia which most commonly affects the pylorus. Pyloric atresia is a primary manifestation rather than a scarring process secondary to epidermolysis bullosa. |
Disease: |
Defects in ITGB4 are a cause of generalized atrophic benign epidermolysis bullosa (GABEB) [MIM:226650]. GABEB is a non-lethal adult form of junctional epidermolysis bullosa characterized by life-long blistering of the skin associated with hair and tooth abnormalities. |
Similarity: |
Belongs to the integrin beta chain family. |
Similarity: |
Contains 1 Calx-beta domain. |
Similarity: |
Contains 4 fibronectin type-III domains. |
Similarity: |
Contains 1 VWFA domain. 1. Kennel. S. J. et al. (1990) Second generation monoclonal antibodies to the human integrin a6b4. |
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