Dysferlin Rabbit anti-Human, Polyclonal, Bioss 

Rabbit Polyclonal Antibody

Manufacturer:  Bioss BS2429R

Catalog No. 50-198-4720

Description

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
• Applications: Immunofluorescence, Immunohistochemistry (Paraffin), Western Blot
• Conjugate: Unconjugated
• Gene: DYSF
• Format: Liquid
• Host Species: Rabbit
• Isotype: IgG
• Quantity: 100 μL
• Storage Requirements: -20°C
• Primary or Secondary: Primary
• Gene ID (Entrez): 8291

• Gene Symbol: DYSF
• Concentration: 1 μg/mL
• Formulation: PBS with 1% BSA, 50% glycerol and 0.09% sodium azide; pH
• Gene Accession No.: O75923
• 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
• Immunogen: KLH conjugated synthetic peptide derived from human Dysferlin.
• Purification Method: Protein A
• Regulatory Status: RUO
• Type: Antibody
• Monoclonal or Polyclonal: Polyclonal
• Target Species: Human