Anti-Dysferlin/DYSF Antibody Picoband®

SKU: A01234-3

Size: 100 μg

Storage: Store at -20℃ for one year from date of receipt. After reconstitution, at 4℃ for one month. It can also be aliquotted and stored frozen at -20℃ for six months. Avoid repeated freeze-thaw cycles.

Form: Lyophilized

Reactivity: Human,Mouse,Rat

Applications: ELISA,Flow Cytometry,IF,IHC,ICC,WB

Application Details: Western blot, 0.25-0.5μg/ml, Human, Mouse, Rat
Immunohistochemistry (Paraffin-embedded Section), 2-5μg/ml, Human, Mouse
Immunocytochemistry/Immunofluorescence, 5μg/ml, Human
Flow Cytometry (Fixed), 1-3μg/1x10⁶ cells, Human
ELISA, 0.1-0.5μg/ml, -

Gene Name: DYSF

Specificity:

Background: Dysferlin also known as dystrophy-associated fer-1-like protein is a protein that in humans is encoded by the DYSF gene. The protein encoded by this gene belongs to the ferlin family and is a skeletal muscle protein found associated with the sarcolemma. It is involved in muscle contraction and contains C2 domains that play a role in calcium-mediated membrane fusion events, suggesting that it may be involved in membrane regeneration and repair. In addition, the protein encoded by this gene binds caveolin-3, a skeletal muscle membrane protein which is important in the formation of caveolae. Specific mutations in this gene have been shown to cause autosomal recessive limb girdle muscular dystrophy type 2B (LGMD2B) as well as Miyoshi myopathy. Alternative splicing results in multiple transcript variants.

Immunogen: E.coli-derived human DYSF recombinant protein (Position: E51-H747).

Clonality: Polyclonal

Contents: Each vial contains 4mg Trehalose, 0.9mg NaCl, 0.2mg Na2HPO4.

Purification: Immunogen affinity purified.

Reconstitution: Add 0.2ml of distilled water will yield a concentration of 500ug/ml.

Reference: 1. Anderson, L. V. B., Davison, K., Moss, J. A., Young, C., Cullen, M. J., Walsh, J., Johnson, M. A., Bashir, R., Britton, S., Keers, S., Argov, Z., Mahjneh, I., Fougerousse, F., Beckmann, J. S., Bushby, K. M. D. Dysferlin is a plasma membrane protein and is expressed early in human development. Hum. Molec. Genet. 8: 855-861, 1999. Note: Erratum: Hum. Molec. Genet. 8: 1141 only, 1999. 2. Aoki, M., Liu, J., Richard, I., Bashir, R., Britton, S., Keers, S. M., Oeltjen, J., Brown, H. E., Marchand, S., Bourg, N., Beley, C., McKenna-Yasek, D., and 13 others. Genomic organization of the dysferlin gene and novel mutations in Miyoshi myopathy. Neurology 57: 271-278, 2001. 3. Bansal, D., Miyake, K., Vogel, S. S., Groh, S., Chen, C.-C., Williamson, R., McNeil, P. L., Campbell, K. P. Defective membrane repair in dysferlin-deficient muscular dystrophy. Nature 423: 168-172, 2003.

Uniprot ID: O75923

Host: Rabbit

Concentration: Adding 0.2 ml of distilled water will yield a concentration of 500 μg/ml.

Conjugate:

Cross Reactivity: No cross-reactivity with other proteins.

Isotype: Rabbit IgG

Phospho_site:

Clone Number:

Observed Molecular Weight: 280 kDa

Calculated Molecular Weight: 123799 MW

Gene ID: 8291

Protein Name: Dysferlin

Gene Full Name: dysferlin

Synonyms: Dysferlin; FER1L1; LGMD2B; LGMDR2; MMD1; DYSF