SAB4200388
Anti-Dysferlin (N-terminal region) antibody produced in rabbit
~1.5 mg/mL, affinity isolated antibody
别名:
Anti-Dysferlin (N-terminal region) antibody produced in rabbit, Anti-DYSF, Anti-Limb girdle muscular dystrophy 2B
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关于此项目
NACRES:
NA.41
UNSPSC Code:
12352203
Conjugate:
unconjugated
Clone:
polyclonal
Application:
ICC, IP, WB
Citations:
5
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)
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
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
Anti-Dysferlin (N-terminal region) specifically recognizes human and mouse Dysferlin.
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.
Preparation Note
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.
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存储类别
10 - Combustible liquids
flash_point_f
Not applicable
flash_point_c
Not applicable
法规信息
新产品
此项目有
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
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
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)
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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