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生物来源
rabbit
质量水平
偶联物
unconjugated
抗体形式
affinity isolated antibody
抗体产品类型
primary antibodies
克隆
polyclonal
形式
buffered aqueous solution
分子量
~250 kDa
种属反应性
human, mouse
增强验证
recombinant expression
Learn more about Antibody Enhanced Validation
浓度
~1.5 mg/mL
技术
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登记号
运输
dry ice
储存温度
−20°C
靶向翻译后修饰
unmodified
基因信息
human ... DYSF(8291)
mouse ... Dysf(26903)
相关类别
一般描述
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
特异性
Anti-Dysferlin (N-terminal region) specifically recognizes human and mouse Dysferlin.
免疫原
synthetic peptide corresponding to a sequence in the N-terminal region of human dysferlin (GeneID: 8291), conjugated to KLH.
应用
Anti-Dysferlin (N-terminal region) antibody produced in rabbit has been used in:
- immunoblotting
- immunoprecipitation
- immunofluorescence
生化/生理作用
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.
外形
solution in 0.01 M phosphate buffered saline, pH 7.4, containing 15 mM sodium azide.
储存及稳定性
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.
免责声明
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.
闪点(°F)
Not applicable
闪点(°C)
Not applicable
法规信息
新产品
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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