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Dysferlin (Romeo) Rabbit mAb

Catalog Number:

78837
other_names: FER1L1, LGMD2B, MMD1

Amount:

100μg
calculated_mw: 280kDa
host_species: Rabbit

Price:

$318

Swiss-Prot No:

O75923

Form of Antibody:

Storage/Stability:

Immunogen:

A synthesized peptide derived from human Dysferlin (Romeo)

Purification:

Affinity purification

Specificity/Sensitivity:

Applications:

WB,IHC

Background:

The protein encoded by this gene belongs to the ferlin family and is a skeletal muscle protein found associated with the sarcolemma. It is involved in muscle contraction and contains C2 domains that play a role in calcium-mediated membrane fusion events, suggesting that it may be involved in membrane regeneration and repair. In addition, the protein encoded by this gene binds caveolin-3, a skeletal muscle membrane protein which is important in the formation of caveolae. Specific mutations in this gene have been shown to cause autosomal recessive limb girdle muscular dystrophy type 2B (LGMD2B) as well as Miyoshi myopathy. Alternative splicing results in multiple transcript variants. [provided by RefSeq, Aug 2008]

References:

appl_detail:

WB 1:500 - 1:2000
IHC 1:50 - 1:200

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