Product Name: Dysferlin antibody (ready-to-use)
Applications: IHC, IHC-P
Predicted Target Size:
Positive Controls: Skeletal muscle
Form Supplied: Liquid
Concentration: Ready-to-use
Purification: Immunogen affinity purified
Full Name: dysferlin, limb girdle muscular dystrophy 2B (autosomal recessive)
Background: Dysferlin is the protein product of the 2p13 gene that is defective in patients with Limb-Girdle Muscular Dystrophy type 2B (LGMD2B) and Miyoshi Myopathy (MM). Dysferlin is normally localized to the muscle plasma membrane. In patients with LGMD2B and MM, immunoreactivity to dysferlin is severely reduced or lost, depending on the type of mutation. This antibody is used for the characterization of LGMD2B and MM.
Synonyms: LGMD2B, FER1L1, 253601, DYSF, O75923, FLJ00175, Dysferlin, 8291
Cellular Localization: Type II membrane protein. Localizes to the sarcolemma.
CAS NO: 511-28-4
Product: AM966
Host: Rabbit
Clonality: Polyclonal
Isotype: IgG
Immunogen: Synthetic peptide (unfortunately, the amino acid sequence is considered to be commercially sensitive) (Human) (C terminal).
Antigen Species: Human
Species Reactivity: Human
Conjugation: Unconjugated
Storage Buffer: PBS, pH 7.4 containing 1%BSA and 0.1% sodium azide
Storage Instruction: Keep as concentrated solution and store at 4°C. Do not freeze.
Notes: For In vitro laboratory use only. Not for any clinical, therapeutic, or diagnostic use in humans or animals. Not for animal or human consumption.
Specificity:
PubMed ID:http://www.ncbi.nlm.nih.gov/pubmed/25804737?dopt=Abstract
