SAB4200453

Anti-Dysferlin (N-terminal) antibody produced in rabbit

~1.0 mg/mL, affinity isolated antibody, antigen mol wt ~250 kDa

• Synonyme(s) : Anti-DYSF, Anti-Limb girdle muscular dystrophy 2B
• Code UNSPSC : 12352203
• Nomenclature NACRES : NA.41

Propriétés

• Source biologique: rabbit
• Niveau de qualité: 200
• Conjugué: unconjugated
• Forme d'anticorps: affinity isolated antibody
• Type de produit anticorps: primary antibodies
• Clone: polyclonal
• Forme: buffered aqueous solution
• Poids mol.: antigen ~250 kDa
• Espèces réactives: rat, human, mouse
• Validation améliorée: recombinant expression; Learn more about Antibody Enhanced Validation
• Concentration: ~1.0 mg/mL
• Technique(s): dot blot: 0.1-0.2 μg/mL using extracts of HEK-293T cells over-expressing human dysferlin and 1-2 mg/mL using A10 cells.; immunohistochemistry: 20-30 μg/mL using methanol-acetone fixed frozen sections of mouse skeletal muscle.; indirect immunofluorescence: 5-10 μg/mL using differentiated C2C12 myoblasts.
• Numéro d'accès UniProt: O75923
• Conditions d'expédition: dry ice
• Température de stockage: −20°C
• Modification post-traductionnelle de la cible: unmodified
• Informations sur le gène: human ... DYSF(8291) ; mouse ... Dysf(26903) ; rat ... Dysf(312492)

Description

Description générale

Dysferlin is a 230 kDa transmembrane protein that belongs to the ferlin family of proteins including myoferlin and otoferlin and is homologous to the C. elegans fer-1 protein. It is expressed early during human development.

Immunogène

synthetic peptide corresponding to a sequence in the N-terminal region of human dysferlin, conjugated to KLH. The corresponding sequence is identical in human dysferlin isoforms 1-14 and identical in mouse dysferlin.

Application

Anti-Dysferlin (N-terminal) antibody produced in rabbit has been used in several immunochemical techniques including immunoblotting, immunofluorescence and immunohistochemistry.

Actions biochimiques/physiologiques

Dysferlin is implicated in membrane fusion events. It has also been involved in membrane repair processes, such as the ability to reseal the sarcolemma upon muscle injury. The integral membrane protein caveolin 3 have been shown to regulate the endocytosis of dysferlin. Dysferlin localization in the membrane and trafficking is impaired by mutations in caveolin-1 and 3, resulting in mistargeting and redistribution of dysferlin from the plasma membrane to the Golgi complex. Mutations in the dysferlin gene leads to the development of limb-girdle muscle dystrophy type 2B (LGMD2B), an autosomal recessive disorder and the related Miyoshi myopathy.

Forme physique

Solution in 0.01 M phos­phate buffered saline, pH 7.4, containing 15 mM sodium azide.

Clause de non-responsabilité

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.

Informations sur la sécurité

• Code de la classe de stockage: 10 - Combustible liquids
• Point d'éclair (°F): Not applicable
• Point d'éclair (°C): Not applicable