Thermo Fisher Scientific Dysferlin Recombinant Rabbit Monoclonal Antibody (ARC2178)

Applications

Tested Dilution

Publications

Western Blot (WB)

1:500-1:2,000

-

Immunohistochemistry (Paraffin) (IHC (P))

1:50-1:200

-

ELISA (ELISA)

1 µg/mL

-

Product Specifications

Species Reactivity

Human

Host/Isotype

Rabbit / IgG

Expression System

HEK293 cells

Class

Recombinant Monoclonal

Type

Antibody

Clone

ARC2178

Immunogen

A synthetic peptide corresponding to a sequence within amino acids 50-150 of human Dysferlin (Romeo) (O75923) if (typeof window.$mangular === undefined || !window.$mangular) { window.$mangular = {}; } $mangular.antigenJson = \[{targetFamily:Dysferlin,uniProtId:O75923-1,ncbiNodeId:9606,antigenRange:50-150,antigenLength:2080,antigenImageFileName:MA5-37920_Dysferlin_O75923-1_Rabbit.svg,antigenImageFileNamePDP:MA5-37920_Dysferlin_O75923-1_Rabbit_PDP.jpeg,sortOrder:1}\]; $mangular.isB2BCMGT = false; $mangular.isEpitopesModalImageMultiSizeEnabled = true;

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Conjugate

Unconjugated Unconjugated Unconjugated

Form

Liquid

Concentration

0.8 mg/mL

Purification

Affinity chromatography

Storage buffer

PBS, pH 7.3, with 50% glycerol, 0.05% BSA

Contains

0.02% sodium azide

Storage conditions

-20° C, Avoid Freeze/Thaw Cycles

Shipping conditions

Ambient (domestic); Wet ice (international)

RRID

AB_2897840

Product Specific Information

Positive Samples: RD

Immunogen sequence: FEWDLKGIPL DQGSELHVVV KDHETMGRNR FLGEAKVPLR EVLATPSLSA SFNAPLLDTK KQPTGASLVL QVSYTPLPGA VPLFPPPTPL EPSPTLPDLD V

Target Information

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.

For Research Use Only. Not for use in diagnostic procedures. Not for resale without express authorization.