Merck

SAB4200388

Sigma-Aldrich

Anti-Dysferlin (N-terminal region) antibody produced in rabbit

enhanced validation

~1.5 mg/mL, affinity isolated antibody

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Sinónimos:
Anti-Dysferlin (N-terminal region) antibody produced in rabbit, Anti-DYSF, Anti-Limb girdle muscular dystrophy 2B
NACRES:
NA.41

biological source

rabbit

conjugate

unconjugated

antibody form

affinity isolated antibody

antibody product type

primary antibodies

clone

polyclonal

form

buffered aqueous solution

mol wt

~250 kDa

species reactivity

human, mouse

enhanced validation

recombinant expression
Learn more about Antibody Enhanced Validation

concentration

~1.5 mg/mL

technique(s)

immunocytochemistry: 7-14 μg/mL using differentiated C2C12 myoblasts
immunoprecipitation (IP): 5-10 μg using HEK-293T cells over-expressing human dysferlin
western blot: 0.5-1.0 μg/mL using HEK-293T cells over-expressing human dysferlin

UniProt accession no.

shipped in

dry ice

storage temp.

−20°C

target post-translational modification

unmodified

Gene Information

human ... DYSF(8291)
mouse ... Dysf(26903)

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Este artículo
D6694SAB4200383SAB4200101
biological source

rabbit

biological source

rabbit

biological source

rabbit

biological source

rabbit

conjugate

unconjugated

conjugate

unconjugated

conjugate

unconjugated

conjugate

unconjugated

Quality Level

200

Quality Level

200

Quality Level

200

Quality Level

-

form

buffered aqueous solution

form

buffered aqueous solution

form

buffered aqueous solution

form

buffered aqueous solution

enhanced validation

recombinant expression
Learn more about Antibody Enhanced Validation

enhanced validation

-

enhanced validation

-

enhanced validation

recombinant expression
Learn more about Antibody Enhanced Validation

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General description

Dysferlin is a transmembrane protein, that belongs to the ferlin-1 family of proteins including myoferlin and otoferlin. It is homologous to the C. elegans fer-1 protein. Dysferlin is expressed in kidney cells, skeletal and cardiac muscle.
Dysferlin is encoded by the gene mapped to human chromosome 2p13.2

Specificity

Anti-Dysferlin (N-terminal region) specifically recognizes human and mouse Dysferlin.

Immunogen

synthetic peptide corresponding to a sequence in the N-terminal region of human dysferlin (GeneID: 8291), conjugated to KLH.

Application

Anti-Dysferlin (N-terminal region) antibody produced in rabbit has been used in:
  • immunoblotting
  • immunoprecipitation
  • immunofluorescence

Biochem/physiol Actions

Dysferlin is implicated in membrane fusion events. It plays a role in membrane repair processes, such as the ability to reseal the sarcolemma upon muscle injury. Dysferlin localization in the membrane and trafficking is impaired by mutations in caveolin-1 and -3. Mutations in the dysferlin gene are associated with limb-girdle muscle dystrophy type 2B (LGMD2B), distal anterior compartment myopathy and related Miyoshi myopathy.

Physical form

solution in 0.01 M phos­phate buffered saline, pH 7.4, containing 15 mM sodium azide.

Storage and Stability

For continuous use, store at 2-8 °C for up to one month. For extended storage, freeze in working aliquots. Repeated freezing and thawing, or storage in “frost-free” freezers,is not recommended. If slight turbidity occurs upon prolonged storage, clarify the solution by centrifugation before use. Working dilutions should be discarded if not used within 12 hours.

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.

Storage Class

10 - Combustible liquids

flash_point_f

Not applicable

flash_point_c

Not applicable


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Florian Barthélémy et al.
Journal of neuromuscular diseases, 5(1), 21-28 (2018-02-27)
Skeletal muscle undergoes many micro-membrane lesions at physiological state. Based on their sizes and magnitude these lesions are repaired via different complexes on a specific spatio-temporal manner. One of the major repair complex is a dysferlin-dependent mechanism. Accordingly, mutations in
Aberrant dysferlin trafficking in cells lacking caveolin or expressing dystrophy mutants of caveolin-3
Hernandez-Deviez DJ, et al.
Human Molecular Genetics, 15(1), 129-142 (2005)
Kyowon Seo et al.
Molecular therapy. Methods & clinical development, 21, 702-709 (2021-06-19)
Biallelic mutations in the dysferlin gene cause limb-girdle muscular dystrophy 2B or Miyoshi distal myopathy. We found that nonsense mutations are the most common mutation type among Korean patients with dysferlinopathy; more than half of the patients have at least
S H Laval et al.
Neuropathology and applied neurobiology, 30(2), 91-105 (2004-03-27)
The limb-girdle muscular dystrophies are a diverse group of muscle-wasting disorders characteristically affecting the large muscles of the pelvic and shoulder girdles. Molecular genetic analyses have demonstrated causative mutations in the genes encoding a disparate collection of proteins involved in
Louise Glover et al.
Traffic (Copenhagen, Denmark), 8(7), 785-794 (2007-06-06)
The muscular dystrophies are a heterogeneous group of inherited disorders, defined by progressive muscle weakness and atrophy. Following the discovery of dystrophin, remarkable progress has been made in defining the molecular properties of proteins involved in the various dystrophies. This

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