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SAB4200453

Sigma-Aldrich

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

enhanced validation

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

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Synonym(s):
Anti-DYSF, Anti-Limb girdle muscular dystrophy 2B
NACRES:
NA.41

biological source

rabbit

Quality Level

conjugate

unconjugated

antibody form

affinity isolated antibody

antibody product type

primary antibodies

clone

polyclonal

form

buffered aqueous solution

mol wt

antigen ~250 kDa

species reactivity

rat, human, mouse

enhanced validation

recombinant expression
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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.

UniProt accession no.

shipped in

dry ice

storage temp.

−20°C

target post-translational modification

unmodified

Gene Information

human ... DYSF(8291)
mouse ... Dysf(26903)
rat ... Dysf(312492)

General description

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.

Immunogen

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.

Biochem/physiol Actions

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.

Physical form

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

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.

Storage Class

10 - Combustible liquids

flash_point_f

Not applicable

flash_point_c

Not applicable


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