Dysferlin 抗体
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北京 101111
Quick Overview for Dysferlin 抗体 (ABIN265388)
抗原
See all Dysferlin (DYSF) 抗体适用
宿主
克隆类型
标记
应用范围
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特异性
- This antibody detects endogenous levels of DP-1/TFDP1 protein (region surrounding Pro2013).
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纯化方法
- The antibody was affinity-purified from rabbit antiserum by affinity chromatography using epitope-specific immunogen
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纯度
- > 95 % pure by SDS-PAGE
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anti-Dysferlin (DYSF) (AA 105-278) antibody
DYSF 适用: 人 ELISA, IHC, IF 宿主: 兔 Polyclonal unconjugated
anti-Dysferlin (DYSF) (C-Term) antibodyDYSF 适用: 人, 小鼠, 大鼠 WB, ELISA, IHC, IF, ICC 宿主: 兔 Polyclonal unconjugated
anti-Dysferlin (DYSF) antibodyDYSF 适用: 人 WB, ELISA 宿主: 兔 Polyclonal unconjugated
anti-Dysferlin (DYSF) antibodyDYSF 适用: 人 WB, IHC 宿主: 兔 Monoclonal unconjugated
anti-Dysferlin (DYSF) (AA 1584-1633) antibodyDYSF 适用: 人, 小鼠, 豚鼠, 兔, 猴 WB 宿主: 兔 Polyclonal unconjugated
anti-Dysferlin (DYSF) (AA 215-264) antibodyDYSF 适用: 人, Cow, 犬, 豚鼠, 兔, Pig, 猴 WB 宿主: 兔 Polyclonal unconjugated
anti-Dysferlin (DYSF) (AA 1999-2016) antibodyDYSF 适用: 人 IHC, IHC (p), IHC (fro) 宿主: 小鼠 Monoclonal Ham1-7B6 unconjugated
anti-Dysferlin (DYSF) (AA 1-479) antibodyDYSF 适用: 人 WB, IHC, ICC, IP 宿主: 兔 Polyclonal unconjugated
anti-Dysferlin (DYSF) (C-Term) antibodyDYSF 适用: 人, 小鼠, 大鼠, Cow, 犬, Pig WB, IHC 宿主: 兔 Polyclonal unconjugated
anti-Dysferlin (DYSF) (Middle Region) antibodyDYSF 适用: 人, 小鼠, 大鼠, Cow, 犬, 豚鼠, 兔, 马, 斑马鱼 WB 宿主: 兔 Polyclonal unconjugated
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应用备注
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ELISA: 1: 10000approx. 1: 20000. WB: 1: 500approx. 1: 1000.
Other applications not tested.
Optimal dilutions are dependent on conditions and should be determined by the user. -
限制
- 仅限研究用
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浓度
- 1.0 mg/mL
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缓冲液
- Phosphate buffered saline (PBS), pH 7.2., 0.05 % Sodium azide
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储存液
- Sodium azide
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注意事项
- This product contains sodium azide: a POISONOUS AND HAZARDOUS SUBSTANCE which should be handled by trained staff only.
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注意事项
- Avoid repeated freezing and thawing.
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储存条件
- 4 °C/-20 °C
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储存方法
- Store undiluted at 2-8 °C for one month or (in aliquots) at -20 °C for longer.
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- Dysferlin (DYSF)
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别名
- Dysferlin
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背景
- Dysferlin is a muscle-specific protein that is essential for normal muscle function and development. Mutations in the human dysferlin gene, DYSF, which maps to chromosome 2p13.3-p13.1, are associated with limb girdle muscular dystrophy-2B (LGMD-2B) and a related, adult-onset, distal dystrophy known as Miyoshi myopathy (MM). Dysferlin, a protein with a molecular mass of approximately 230 kDa, localizes to the muscle fiber membrane, but is absent in MM and LGMD-2B muscle. Dysferlin is detected in 5-6 week embryos, when limbs begin to form regional differentiation. Although it is not essential for initial myogenesis, dysferlin appears to be critical for sustained normal function in mature muscle. It has been suggested that the absence of dysferlin during development gives rise to the disease phenotype in adulthood. Identical mutations in the dysferlin gene can produce more than one myopathy phenotype, indicating that additional genes and/or other factors are also involved in the clinical phenotype. The DYSF gene has no homology to any other known mammalian gene, but the protein product is related to the spermatogenesis factor fer-1 of Caenorhabditis elegans. The name 'dysferlin' combines the role of the gene in producing muscular dystrophy with its homology to C. elegans.Synonyms: DYSF, Dystrophy-associated fer-1-like protein, FER1L1, Fer-1-like protein 1
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分子量
- approx. 240 kDa
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基因ID
- 8291
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NCBI登录号
- NP_001123927
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UniProt
- O75923
抗原
-
