Dysferlin Rabbit anti-Human, Polyclonal, Bioss

Rabbit Polyclonal Antibody

Supplier:  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
• 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: Protein A
• Regulatory Status: RUO
• Gene ID (Entrez): 8291
• Content And Storage: -20°C
• Form: Liquid

• Applications: Immunofluorescence, Immunohistochemistry (Paraffin), Western Blot
• Concentration: 1 μg/mL
• Formulation: PBS with 1% BSA, 50% glycerol and 0.09% sodium azide; pH
• Gene Accession No.: O75923
• Gene Symbols: DYSF
• Immunogen: KLH conjugated synthetic peptide derived from human Dysferlin.
• Quantity: 100 μL
• Primary or Secondary: Primary
• Target Species: Human
• Product Type: Antibody
• Isotype: IgG