Goat dysferlin Polyclonal Antibody | anti-DYSF antibody
Goat anti-dysferlin Antibody
Western Blot: Preliminary experiments in Human and rodent Heart and Skeletal Muscle lysates gave no specific signal but low background (at antibody concentration up to 1ug/ml).
NCBI and Uniprot Product Information
NCBI Description
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]
Uniprot Description
DYSF: Key calcium ion sensor involved in the Ca(2+)-triggered synaptic vesicle-plasma membrane fusion. Plays a role in the sarcolemma repair mechanism of both skeletal muscle and cardiomyocytes that permits rapid resealing of membranes disrupted by mechanical stress. Defects in DYSF are the cause of limb-girdle muscular dystrophy type 2B (LGMD2B). LGMD2B is an autosomal recessive degenerative myopathy characterized by weakness and atrophy starting in the proximal pelvifemoral muscles, with onset in the late teens or later, massive elevation of serum creatine kinase levels and slow progression. Scapular muscle involvement is minor and not present at onset. Upper limb girdle involvement follows some years after the onset in lower limbs. Defects in DYSF are the cause of Miyoshi muscular dystrophy type 1 (MMD1). MMD1 is a late-onset muscular dystrophy involving the distal lower limb musculature. It is characterized by weakness that initially affects the gastrocnemius muscle during early adulthood. Otherwise the phenotype overlaps with LGMD2B, especially in age at onset and creatine kinase elevation. Defects in DYSF are the cause of distal myopathy with anterior tibial onset (DMAT). Onset of the disorder is between 14 and 28 years of age and the anterior tibial muscles are the first muscle group to be involved. Inheritance is autosomal recessive. Belongs to the ferlin family. 15 isoforms of the human protein are produced by alternative splicing.
Protein type: Vesicle; Membrane protein, integral; Calcium-binding
Chromosomal Location of Human Ortholog: 2p13.3
Cellular Component: cytoplasmic vesicle membrane; early endosome; endocytic vesicle; endosome; integral to membrane; lamellipodium; late endosome; plasma membrane; sarcolemma; T-tubule
Molecular Function: calcium ion binding; calcium-dependent phospholipid binding; phospholipid binding; protein binding
Biological Process: muscle contraction; plasma membrane repair; vesicle fusion
Disease: Miyoshi Muscular Dystrophy 1; Muscular Dystrophy, Limb-girdle, Type 2b; Myopathy, Distal, With Anterior Tibial Onset
Research Articles on DYSF
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Product Notes
The DYSF dysf (Catalog #AAA423540) is an Antibody produced from Goat and is intended for research purposes only. The product is available for immediate purchase. The Goat anti-dysferlin Antibody reacts with Expected from sequence similarity: Human, Rat, Pig, Cow and may cross-react with other species as described in the data sheet. AAA Biotech's dysferlin can be used in a range of immunoassay formats including, but not limited to, Peptide ELISA (EIA). Peptide ELISA: Antibody detection limit dilution 1: 128000. Western Blot: Preliminary experiments in Human and rodent Heart and Skeletal Muscle lysates gave no specific signal but low background (at antibody concentration up to 1ug/ml). Researchers should empirically determine the suitability of the DYSF dysf for an application not listed in the data sheet. Researchers commonly develop new applications and it is an integral, important part of the investigative research process. The amino acid sequence is listed below: HLFCQQHRVK AP. It is sometimes possible for the material contained within the vial of "dysferlin, Polyclonal Antibody" to become dispersed throughout the inside of the vial, particularly around the seal of said vial, during shipment and storage. We always suggest centrifuging these vials to consolidate all of the liquid away from the lid and to the bottom of the vial prior to opening. Please be advised that certain products may require dry ice for shipping and that, if this is the case, an additional dry ice fee may also be required.Precautions
All products in the AAA Biotech catalog are strictly for research-use only, and are absolutely not suitable for use in any sort of medical, therapeutic, prophylactic, in-vivo, or diagnostic capacity. By purchasing a product from AAA Biotech, you are explicitly certifying that said products will be properly tested and used in line with industry standard. AAA Biotech and its authorized distribution partners reserve the right to refuse to fulfill any order if we have any indication that a purchaser may be intending to use a product outside of our accepted criteria.Disclaimer
Though we do strive to guarantee the information represented in this datasheet, AAA Biotech cannot be held responsible for any oversights or imprecisions. AAA Biotech reserves the right to adjust any aspect of this datasheet at any time and without notice. It is the responsibility of the customer to inform AAA Biotech of any product performance issues observed or experienced within 30 days of receipt of said product. To see additional details on this or any of our other policies, please see our Terms & Conditions page.Item has been added to Shopping Cart
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