Dysferlin Rabbit pAb
Cat No.: APA3072
Size:
Product Name: | Dysferlin Rabbit pAb |
Cat No.: | APA3072 |
source: | Rabbit |
reactivity: | Human, Mouse |
applications: | WB,IHC,ICC/IF |
clonality: | Polyclonal |
recommended dilution: | WB: 1:1000 IHC: 1:100 ICC/IF: 1:50 |
format: | Liquid |
isotype: | IgG |
immunogen: | A synthetic peptide of human Dysferlin |
calculated molecular weight: | 237 kDa |
observed molecular weight: | 260 kDa |
genbank accession number: | O75923 |
gene id (ncbi): | 8291 |
purification method: | Affinity Purification |
conjugate: | Un-conjugated |
storage: | Store at -20°C. Supplied in 50nM Tris-Glycine(pH 7.4), 0.15M NaCl, 40%Glycerol, 0.01% sodium azide and 0.05% BSA. Stable for 12 months from date of receipt. |
synonyms: | MMD1; FER1L1; LGMD2B |
category: | Primary Ab |
concentration: | 0.5mg/ml |
background: | 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] |