Dysferlin (P2013) Antibody

貨號 Cat. No.

BS1094

規格 Size

100μg

品牌 Brand

Bioworld

Description

stock

Host: Rabbit

Reactivity: Human,Mouse

Product

1 mg/ml in Phosphate buffered saline (PBS) with 15 mM sodium azide, approx. pH 7.2.

Purification & Purity

The antibody was affinity-purified from rabbit antiserum by affinity-chromatography using epitope-specific immunogen and the purity is > 95% (by SDS-PAGE).

Specificity

Dysferlin (P2013) antibody detects endogenous levels of DP-1/TFDP1 protein.

Applications (Recommended Dilutions)

WB: 1:500~1:1000 IF: 1:50~1:200

Western Blot(WB)

Western blot (WB) analysis of Dysferlin (P2013) antibody in extracts from K562 cells.

Storage&Stability

Store at 4°C short term. Aliquot and store at -20°C long term. Avoid freeze-thaw cycles.

Background

Dysferlin is a muscle-specific protein that is essential for normal muscle function and development. Mutations in the human dysferlin gene, DYSF, which maps to chromosome 2p13.3-p13.1, are associated with limb girdle muscular dystrophy-2B (LGMD-2B) and a related, adult-onset, distal dystrophy known as Miyoshi myopathy (MM). Dysferlin, a protein with a molecular mass of approximately 230 kD,a localizes to the muscle fiber membrane, but is absent in MM and LGMD-2B muscle. Dysferlin is detected in 5-6 week embryos, when limbs begin to form regional differentiation. Although it is not essential for initial myogenesis, dysferlin appears to be critical for sustained normal function in mature muscle. It has been suggested that the absence of dysferlin during development gives rise to the disease phenotype in adulthood. Identical mutations in the dysferlin gene can produce more than one myopathy phenotype, indicating that additional genes and/or other factors are also nvolved in the clinical phenotype. The DYSF gene has no homology to any other known mammalian gene, but the protein product is related to the spermatogenesis factor fer-1 of Caenorhabditis elegans.

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