Dysferlin (DYSF) Rabbit Polyclonal Antibody
Specifications
| Product Data | |
| Application | IHC, WB |
|---|---|
| Reactivity | Human |
| Antibody Host | Rabbit |
| Clonality | Polyclonal |
| Immunogen | The immunogen is a synthetic peptide directed towards the middle region of human DYSF |
| Specificity | Expected reactivity: Cow, Dog, Guinea Pig, Horse, Human, Mouse, Rabbit, Rat, Zebrafish Homology: Cow: 100%; Dog: 93%; Guinea Pig: 100%; Horse: 100%; Human: 100%; Mouse: 93%; Rabbit: 93%; Rat: 100%; Zebrafish: 92% |
| Buffer | Liquid. Purified antibody supplied in 1x PBS buffer with 0.09% (w/v) sodium azide and 2% sucrose. Note that this product is shipped as lyophilized powder to China customers. |
| Concentration | lot specific |
| Purification | Affinity Purified |
| Conjugation | Unconjugated |
| Storage | For short term use, store at 2-8°C up to 1 week. For long term storage, store at -20°C in small aliquots to prevent freeze-thaw cycles. |
| Stability | Shelf life: one year from despatch. |
| Shipping | Blue Ice |
| Predicted Protein Size | 237kDa |
| Gene Name | dysferlin |
| Database Link | |
| Background | DYSF 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, DYSF 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.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. Publication Note: This RefSeq record includes a subset of the publications that are available for this gene. Please see the Entrez Gene record to access additional publications. |
| Synonyms | dysferlin; FER1L1; FLJ00175; FLJ90168; LGMD2B; OTTHUMP00000202233; OTTHUMP00000202234; OTTHUMP00000202235; OTTHUMP00000202236; OTTHUMP00000202237; OTTHUMP00000202240 |
| Reference Data | |
| Protein Categories | Intracellular Proteins, Membrane Proteins |
| Protein Families | Transmembrane |
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