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Millipore This Anti-Dysferlin/Dysf Antibody, Clone 10h3.1 Is Validated For Use In Western Blotting, Immunohistochemistry (Paraffin) For The Detection Of Dysferlin/Dysf. - Mill (Additional S&H or Hazmat Fees May Apply)
Dysferlin (UniProt O75923; also known as Dystrophy-associated fer-1-like 1 protein, Fer-1-like family member 1, Fer-1-like protein 1, Limb girdle muscular dystrophy 2B (autosomal recessive) is encoded by the DYSF (also known as FER1L1, LGMD2B, MMD1) gene (Gene ID 8291) in human. Dysferlin is a member of the ferlin family of calcium-dependent phospholipid-binding proteins involved in membrane vesicle fusion, membrane repair, T-tubule stability, and calcium homeostasis. Defects in dysferlin function/expression leads to muscular dystrophies (dysferlinopathies) that include Miyoshi myopathy (MM), limb-girdle muscular dystrophy type 2B (LGMD-2B), and distal myopathy with anterior tibial onset (DMAT).
Product Information
Format
Purified
Presentation
Purified mouse monoclonal IgG2bκ antibody in buffer containing 0.1 M Tris-Glycine (pH 7.4), 150 mM NaCl with 0.05% sodium azide.
Quality Level
MQ100
Applications
Application
This Anti-Dysferlin/DYSF Antibody, clone 10H3.1 is validated for use in Western Blotting, Immunohistochemistry (Paraffin) for the detection of Dysferlin/DYSF.
Key Applications
Western Blotting
Immunohistochemistry (Paraffin)
Application Notes
Immunohistochemistry Analysis: A 1:50-1,000 dilution from a representative lot detected Dysferlin/DYSF in human colon tissue and human skeletal muscle myocytes.
Biological Information
Immunogen
GST-tagged recombinant protein corresponding to the N-terminal of human Dysferlin/DYSF.
Epitope
N-terminal
Clone
10H3.1
Concentration
Please refer to lot specific datasheet.
Host
Mouse
Specificity
Expected to react with spliced isoforms 1, 3, 4, 6, 9, 10, 12, 14, and 15. Reactivity toward isoforms 2, 5, 7, 8, 11, and 13 has not been tested.
Isotype
IgG2bκ
Species Reactivity
Human
Antibody Type
Monoclonal Antibody
Entrez Gene Number
NP_003485
Gene Symbol
DYSF
FER1L1
Purification Method
Protein G Purified
UniProt Number
O75923
Molecular Weight
~237 kDa observed. Uncharacterized band(s) may appear in some lysates.
Product Usage Statements
Quality Assurance
Evaluated by Western Blotting in human fetal skeletal muscle tissue lysate.
Western Blotting Analysis: 0.5 µg/mL of this antibody detected Dysferlin/DYSF in 10 µg of human fetal skeletal muscle tissue lysate.
Usage Statement
Unless otherwise stated in our catalog or other company documentation accompanying the product(s), our products are intended for research use only and are not to be used for any other purpose, which includes but is not limited to, unauthorized commercial uses, in vitro diagnostic uses, ex vivo or in vivo therapeutic uses or any type of consumption or application to humans or animals.
