Dysferlin (Romeo) Rabbit Monoclonal Antibody (CAB19572)
- SKU:
- CAB19572
- Product Type:
- Antibody
- Antibody Type:
- Monoclonal Antibody
- Reactivity:
- Human
- Mouse
- Host Species:
- Rabbit
- Isotype:
- IgG
- Synonyms:
- FER1L1
- LGMD2B
- MMD1
Frequently bought together:
Description
Product Name: | Dysferlin (Romeo) Rabbit Monoclonal Antibody |
SKU: | CAB19572 |
Size: | 20uL, 100uL |
Isotype: | IgG |
Host Species: | Rabbit |
Reactivity: | Human |
Immunogen: | A synthetic peptide corresponding to a sequence within amino acids 50-150 of human Dysferlin (Romeo) (O75923). |
Sequence: | FEWD LKGI PLDQ GSEL HVVV KDHE TMGR NRFL GEAK VPLR EVLA TPSL SASF NAPL LDTK KQPT GASL VLQV SYTP LPGA VPLF PPPT PLEP SPTL PDLD V |
Tested Applications: | WB IHC-P ELISA |
Recommended Dilution: | WB,1:500 - 1:2000 IHC-P,1:50 - 1:200 |
Synonyms: | MMD1; FER1L1; LGMD2B; LGMDR2; Dysferlin (Romeo) |
Positive Sample: | RD |
Conjugate: | Unconjugated |
Cellular Localization: | Cytoplasmic vesicle, Endosome, Extracellular exosome, Plasma membrane, T-tubule |
Calculated MW: | 237kDa |
Observed MW: | 280kDa |
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.
Purification Method: | Affinity purification |
Gene ID: | 8291 |
Clone Number: | ARC2178 |
Storage Buffer: | Store at -20℃. Avoid freeze / thaw cycles.Buffer: PBS with 0.02% sodium azide,0.05% BSA,50% glycerol,pH7.3. |