SAB4503260

Anti-Dysferlin, antibody produced in rabbit

affinity isolated antibody

• Synonym(s): dysferlin, limb girdle muscular dystrophy 2B (autosomal recessive)
• NACRES: NA.41

Properties

• biological source: rabbit
• Quality Level: 100
• conjugate: unconjugated
• antibody form: affinity isolated antibody
• antibody product type: primary antibodies
• clone: polyclonal
• form: buffered aqueous solution
• mol wt: antigen 237 kDa
• species reactivity: mouse, human
• concentration: ~1 mg/mL
• technique(s): ELISA: 1:10000; western blot: 1:500-1:1000
• NCBI accession no.: O75923
• UniProt accession no.: O75923
• shipped in: wet ice
• storage temp.: −20°C
• Gene Information: human ... DYSF(8291)

Description

General description

Dysferlin (DYSF) is a large transmembrane protein that belongs to the ferlin family of Ca²⁺-dependent phospholipid-binding proteins. It is expressed in skeletal and cardiac muscles. DYSF gene is mapped to human chromosome 2p13. Anti-Dysferlin antibody detects endogenous levels of total dysferlin protein.

Immunogen

The antiserum was produced against synthesized peptide derived from human Dysferlin.

Immunogen Range: 1981-2030

Application

Anti-Dysferlin, antibody produced in rabbit has been used in western blotting.

Biochem/physiol Actions

Dysferlin (DYSF) plays a key role in membrane repair, vesicle trafficking, and Ttubule structure. Lack of DYSF protein expression in skeletal muscles leads to dysferlinopathy. Mutations in the DYSF gene are associated with Miyoshi myopathy, limb-girdle muscular dystrophy type 2B (LGMD2B), and distal myopathy with anterior tibial onset (DMAT).

Features and Benefits

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Physical form

Rabbit IgG in phosphate buffered saline (without Mg2+ and Ca2+), pH 7.4, 150mM NaCl, 0.02% sodium azide and 50% glycerol.

Disclaimer

Unless otherwise stated in our catalog or other company documentation accompanying the product(s), our products are intended for research use only and are not to be used for any other purpose, which includes but is not limited to, unauthorized commercial uses, in vitro diagnostic uses, ex vivo or in vivo therapeutic uses or any type of consumption or application to humans or animals.

Safety Information

• Storage Class Code: 12 - Non Combustible Liquids
• WGK: nwg
• Flash Point(F): Not applicable
• Flash Point(C): Not applicable