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MABC974

Sigma-Aldrich

Anti-Dysferlin/DYSF Antibody, clone 10H3.1

clone 10H3.1, from mouse

Synonym(s):

Dysferlin, Dystrophy-associated fer-1-like protein, Fer-1-like protein 1, Dysferlin/DYSF

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About This Item

UNSPSC Code:
12352203
eCl@ss:
32160702
NACRES:
NA.43

biological source

mouse

Quality Level

100

antibody form

purified immunoglobulin

antibody product type

primary antibodies

clone

10H3.1, monoclonal

species reactivity

human

technique(s)

immunohistochemistry: suitable (paraffin)
western blot: suitable

isotype

IgG2bκ

NCBI accession no.

NP_003485

UniProt accession no.

O75923

shipped in

wet ice

target post-translational modification

unmodified

Gene Information

human ... DYSF(8291)

General description

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).

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.

Immunogen

Epitope: N-terminal
GST-tagged recombinant protein corresponding to the N-terminal of human Dysferlin/DYSF.

Application

Immunohistochemistry Analysis: A 1:50-1,000 dilution from a representative lot detected Dysferlin/DYSF in human colon tissue and human skeletal muscle myocytes.
Research Category
Apoptosis & Cancer
Research Sub Category
Apoptosis - Additional
This Anti-Dysferlin/DYSF Antibody, clone 10H3.1 is validated for use in Western Blotting, Immunohistochemistry (Paraffin) for the detection of Dysferlin/DYSF.

Quality

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.

Target description

~237 kDa observed. Uncharacterized band(s) may appear in some lysates.

Physical form

Format: Purified
Protein G Purified
Purified mouse monoclonal IgG2bκ antibody in buffer containing 0.1 M Tris-Glycine (pH 7.4), 150 mM NaCl with 0.05% sodium azide.

Storage and Stability

Stable for 1 year at 2-8°C from date of receipt.

Other Notes

Concentration: Please refer to lot specific datasheet.

Disclaimer

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.

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wgk_germany

WGK 1

flash_point_f

Not applicable

flash_point_c

Not applicable

Certificates of Analysis (COA)

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