Dysferline (Romeo) Konijn mAb (AMR11603N)

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. [provided by RefSeq, Aug 2008]

We constantly strive to ensure we provide our customers with the best antibodies. As a result of this work we offer this antibody in purified format. We are in the process of updating our datasheets. If you have any questions regarding this update, please feel free to contact our technical support team. This product is a high quality Dysferlin (Romeo) Rabbit mAb (APR30380N2) .

FER1L1; LGMD2B; MMD1

8291

O75923

WB 1:500 - 1:2000 IHC 1:50 - 1:200

Liquid

Buffer: PBS with 0.02% sodium azide, 0.05% BSA, 50% glycerol, pH7.3.

Calculated MW: 280kDa Observed MW: 280KDa

Store at 4°C short term. For long-term storage, aliquot and store at -20°C or below. Stable for 12 months at -20°C. Avoid repeated freeze-thaw cycles.

https://www.ncbi.nlm.nih.gov/entrez/query.fcgi?db=gene&cmd=Retrieve&dopt=Graphics&list_uids=8291

https://www.uniprot.org/uniprot/O75923

Email for sequence