Dysferlin Recombinant Rabbit Monoclonal Antibody [JF05-54]
Catalog# ET1702-45
Dysferlin Recombinant Rabbit Monoclonal Antibody [JF05-54]
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WB
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IF-Tissue
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IHC-P
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IHC-Fr
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Human
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Mouse
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unconjugated
Overview
Product Name
Dysferlin Recombinant Rabbit Monoclonal Antibody [JF05-54]
Antibody Type
Recombinant Rabbit monoclonal Antibody
Immunogen
Synthetic peptide within Human Dysferlin aa 113-162 / 2080.
Species Reactivity
Human, Mouse
Validated Applications
WB, IF-Tissue, IHC-P, IHC-Fr
Molecular Weight
Predicted band size: 237 kDa
Positive Control
Human skeletal muscle tissue lysates, human spleen tissue, mouse skeletal muscle tissue.
Conjugation
unconjugated
Clone Number
JF05-54
RRID
Product Features
Form
Liquid
Concentration
Storage Instructions
Shipped at 4℃. Store at +4℃ short term (1-2 weeks). Store at -20℃ long term.
Storage Buffer
1*TBS (pH7.4), 0.05% BSA, 40% Glycerol. Preservative: 0.05% Sodium Azide.
Isotype
IgG
Purification Method
Protein A affinity purified.
Application Dilution
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WB
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1:1,000-1:5,000
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IF-Tissue
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1:50-1:200
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IHC-P
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1:200
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IHC-Fr
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1:200
Target
Function
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 2p13.2, are associated with limb girdle muscular dystrophy-2B (LGMD-2B) and a related, adult-onset, distal dystrophy known as Miyoshi myopathy (MM). Dysferlin 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 involved 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. The name “dysferlin” combines the role of the gene in producing muscular dystrophy with its homology to C. elegans.
Background References
1. Tzeng HP et al. Dysferlin mediates the cytoprotective effects of TRAF2 following myocardial ischemia reperfusion injury. J Am Heart Assoc 3:e000662 (2014).
2. Guo LT et al. Evaluation of commercial dysferlin antibodies on canine, mouse and human skeletal muscle. Neuromuscul Disord : (2010).
Sequence Similarity
Belongs to the ferlin family.
Tissue Specificity
Expressed in skeletal muscle, myoblast, myotube and in the syncytiotrophoblast (STB) of the placenta (at protein level). Ubiquitous. Highly expressed in skeletal muscle. Also found in heart, brain, spleen, intestine, placenta and at lower levels in liver, lung, kidney and pancreas.
Subcellular Location
Cell membrane, Cytoplasmic vesicle membrane.
Synonyms
DMAT antibody
DYSF antibody
DYSF_HUMAN antibody
Dysferlin antibody
Dysferlin limb girdle muscular dystrophy 2B (autosomal recessive) antibody
Dysferlin limb girdle muscular dystrophy 2B antibody
Dystrophy associated fer 1 like 1 antibody
Dystrophy associated fer 1 like protein antibody
Dystrophy associated fer1 like 1 antibody
Dystrophy associated fer1 like protein antibody
ExpandImages
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Western blot analysis of Dysferlin on human skeletal muscle tissue lysates with Rabbit anti-Dysferlin antibody (ET1702-45) at 1/1,000 dilution.
Lysates/proteins at 20 µg/Lane.
Predicted band size: 237 kDa
Observed band size: 237 kDa
Exposure time: 3 minutes; ECL: K1801;
6% SDS-PAGE gel.
Proteins were transferred to a PVDF membrane and blocked with 5% NFDM/TBST for 1 hour at room temperature. The primary antibody (ET1702-45) at 1/1,000 dilution was used in 5% NFDM/TBST at room temperature for 2 hours. Goat Anti-Rabbit IgG - HRP Secondary Antibody (HA1001) at 1/50,000 dilution was used for 1 hour at room temperature. -
Immunohistochemical analysis of paraffin-embedded human spleen tissue with Rabbit anti-Dysferlin antibody (ET1702-45) at 1/200 dilution.
The section was pre-treated using heat mediated antigen retrieval with Tris-EDTA buffer (pH 9.0) for 20 minutes. The tissues were blocked in 1% BSA for 20 minutes at room temperature, washed with ddH2O and PBS, and then probed with the primary antibody (ET1702-45) at 1/200 dilution for 1 hour at room temperature. The detection was performed using an HRP conjugated compact polymer system. DAB was used as the chromogen. Tissues were counterstained with hematoxylin and mounted with DPX. -
Immunohistochemical analysis of paraffin-embedded mouse skeletal muscle tissue with Rabbit anti-Dysferlin antibody (ET1702-45) at 1/200 dilution.
The section was pre-treated using heat mediated antigen retrieval with Tris-EDTA buffer (pH 9.0) for 20 minutes. The tissues were blocked in 1% BSA for 20 minutes at room temperature, washed with ddH2O and PBS, and then probed with the primary antibody (ET1702-45) at 1/200 dilution for 1 hour at room temperature. The detection was performed using an HRP conjugated compact polymer system. DAB was used as the chromogen. Tissues were counterstained with hematoxylin and mounted with DPX. -
Application: Immunofluorescence (IHC-Fr)
Species: Mouse
Tissue: Skeletal muscle
Sample: Frozen section
Antigen retrieval: Not required
Wash buffer: 1× TBST
Blocking: 10% normal goat serum + 1% Triton X-100 + 0.3 M Glycine in TBST, 30 minutes at room temperature.
Primary antibody: ET1702-45, 1/200, overnight at 4℃.
Secondary antibody: Goat Anti-Rabbit IgG (iFluor™ 488, HA1121), 1.5 hours at room temperature.
Please note: All products are "FOR RESEARCH USE ONLY AND ARE NOT INTENDED FOR DIAGNOSTIC OR THERAPEUTIC USE"