SAB4503260
Anti-Dysferlin, antibody produced in rabbit
affinity isolated antibody
Synonym(s):
dysferlin, limb girdle muscular dystrophy 2B (autosomal recessive)
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About This Item
NACRES:
NA.41
UNSPSC Code:
12352203
Product Name
Anti-Dysferlin, antibody produced in rabbit, affinity isolated antibody
biological source
rabbit
conjugate
unconjugated
antibody form
affinity isolated antibody
antibody product type
primary antibodies
clone
polyclonal
form
buffered aqueous solution
mol wt
antigen 237 kDa
species reactivity
mouse, human
concentration
~1 mg/mL
technique(s)
ELISA: 1:10000
western blot: 1:500-1:1000
NCBI accession no.
UniProt accession no.
shipped in
wet ice
storage temp.
−20°C
target post-translational modification
unmodified
Quality Level
Gene Information
human ... DYSF(8291)
Application
Anti-Dysferlin, antibody produced in rabbit has been used in western blotting.
Biochem/physiol Actions
Dysferlin (DYSF) plays a key role in membrane repair, vesicle trafficking, and Ttubule structure. Lack of DYSF protein expression in skeletal muscles leads to dysferlinopathy. Mutations in the DYSF gene are associated with Miyoshi myopathy, limb-girdle muscular dystrophy type 2B (LGMD2B), and distal myopathy with anterior tibial onset (DMAT).
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.
Features and Benefits
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General description
Dysferlin (DYSF) is a large transmembrane protein that belongs to the ferlin family of Ca2+-dependent phospholipid-binding proteins. It is expressed in skeletal and cardiac muscles. DYSF gene is mapped to human chromosome 2p13. Anti-Dysferlin antibody detects endogenous levels of total dysferlin protein.
Immunogen
The antiserum was produced against synthesized peptide derived from human Dysferlin.
Immunogen Range: 1981-2030
Immunogen Range: 1981-2030
Physical form
Rabbit IgG in phosphate buffered saline (without Mg2+ and Ca2+), pH 7.4, 150mM NaCl, 0.02% sodium azide and 50% glycerol.
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Storage Class
10 - Combustible liquids
wgk
nwg
flash_point_f
Not applicable
flash_point_c
Not applicable
Regulatory Information
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Jin Tang et al.
Neuropathology : official journal of the Japanese Society of Neuropathology (2018-05-26)
Dysferlinopathy, a progressive muscular dystrophy, results from mutations in the Dysferlin gene (DYSF, MIM*603009). Traditional diagnosis relies on the reduction or absence of dysferlin. However, altered dysferlin has been observed in other myopathies, leading to a precise diagnosis through molecular
Functional recovery of a novel knock-in mouse model of dysferlinopathy by readthrough of nonsense mutation
Seo K, et al.
Molecular therapy. Methods & clinical development (2021)
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
Janice A Dominov et al.
Annals of clinical and translational neurology, 6(4), 642-654 (2019-04-26)
Dysferlin is a large transmembrane protein that functions in critical processes of membrane repair and vesicle fusion. Dysferlin-deficiency due to mutations in the dysferlin gene leads to muscular dystrophy (Miyoshi myopathy (MM), limb girdle muscular dystrophy type 2B (LGMD2B), distal
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