Invitrogen™ Dysferlin Polyclonal Antibody

Rabbit Polyclonal Antibody

Brand:  Invitrogen™ PA551347

Product Code. 15685265

Description

The antibody detects endogenous levels of total Dysferlin protein.

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.

Specifications

• Antigen: Dysferlin
• Classification: Polyclonal
• Conjugate: Unconjugated
• Gene: DYSF
• Gene Alias: 2310004N10Rik; AI604795; D6Pas3; Dysf; Dysferlin; dysferlin variant a; dysferlin_a; dystrophy-associated fer-1-like 1; dystrophy-associated fer-1-like protein; FER1L1; fer-1-like family member 1; fer-1-like protein 1; FLJ00175; FLJ90168; LGMD2B; limb girdle muscular dystrophy 2B (autosomal recessive); MMD1
• Host Species: Rabbit
• Purification Method: Antigen affinity chromatography
• Regulatory Status: RUO
• Gene ID (Entrez): 26903, 8291
• Content And Storage: -20°C
• Form: Liquid

• Applications: Immunohistochemistry (Paraffin)
• Concentration: 1.8 mg/mL
• Formulation: PBS with 40% glycerol and 0.05% sodium azide; pH 7.4
• Gene Accession No.: O75923, Q9ESD7
• Gene Symbols: DYSF
• Immunogen: Synthetic peptide of human DYSF.
• Quantity: 100 μL
• Primary or Secondary: Primary
• Target Species: Human, Mouse
• Product Type: Antibody
• Isotype: IgG