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Dysferlin Rabbit anti-Human, Polyclonal, Bioss
SDP

Catalog No. 501984720
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50-198-4720 100 μL
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Catalog No. 50-198-4720 Supplier Bioss Supplier No. BS2429R
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Description

Rabbit Polyclonal Antibody

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
Classification Polyclonal
Concentration 1 μg/mL
Conjugate Unconjugated
Formulation PBS with 1% BSA, 50% glycerol and 0.09% sodium azide; pH
Gene DYSF
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
Gene Symbols DYSF
Host Species Rabbit
Immunogen KLH conjugated synthetic peptide derived from human Dysferlin.
Purification Method Protein A
Quantity 100 μL
Regulatory Status RUO
Primary or Secondary Primary
Gene ID (Entrez) 8291
Target Species Human
Content And Storage -20°C
Product Type Antibody
Form Liquid
Isotype IgG
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