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折扣/货期| 货号：GTX01908 | 规格：500μl | 目录价：￥10400 |
产品详情
* 以下信息仅供参考，详情请以原厂网站为准
产品名称:
Dysferlin antibody [Ham1/7B6]
别名:
dysferlin , FER1L1 , LGMD2B , LGMDR2 , MMD1
反应种属:
Human
宿主来源:
Mouse
实验应用:
IHC-Fr, IHC-P
靶标/特异性:
Reactive with the dysferlin molecule in human skeletal muscle. Also present in many non-muscle tissues.
同种型:
IgG1
免疫原:
Synthetic peptide containing amino acids 1999-2016 of the human dysferlin molecule.
克隆性:
Monoclonal
克隆号:
Ham1/7B6
纯化方式:
Unpurified
偶联:
Unconjugated
产品浓度:
保存温度:
Store as concentrated solution. Centrifuge briefly prior to opening vial. For short-term storage (1-2 weeks), store at 4ºC. For long-term storage, aliquot and store at -20ºC or below. Avoid multiple freeze-thaw cycles.
运输温度:
4°C
产品形式:
Liquid
存储溶液:
Tissue culture supernatant, 0.09% Sodium azide.
生产商:
GeneTex
功能与背景:
The protein encoded by this gene belongs to the ferlin family and is a skeletal muscle protein found associated with the sarcolemma. It is involved in muscle contraction and contains C2 domains that play a role in calcium-mediated membrane fusion events, suggesting that it may be involved in membrane regeneration and repair. In addition, the protein encoded by this gene binds caveolin-3, a skeletal muscle membrane protein which is important in the formation of caveolae. Specific mutations in this gene have been shown to cause autosomal recessive limb girdle muscular dystrophy type 2B (LGMD2B) as well as Miyoshi myopathy. Alternative splicing results in multiple transcript variants. [provided by RefSeq, Aug 2008]
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