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Dysferlin 抗体 (Middle Region)

DYSF 适用: 人, 小鼠, 大鼠, 犬, Cow, 兔, 豚鼠, 马, 斑马鱼 WB 宿主: 兔 Polyclonal unconjugated
产品编号 ABIN2782235
发货至: 中国
  • 抗原 See all Dysferlin (DYSF) 抗体
    Dysferlin (DYSF)
    抗原表位
    • 16
    • 5
    • 4
    • 2
    • 1
    • 1
    • 1
    • 1
    • 1
    • 1
    • 1
    Middle Region
    适用
    • 53
    • 23
    • 4
    • 4
    • 4
    • 3
    • 3
    • 3
    • 2
    • 1
    • 1
    • 1
    人, 小鼠, 大鼠, 犬, Cow, 兔, 豚鼠, 马, 斑马鱼
    宿主
    • 49
    • 3
    • 1
    克隆类型
    • 40
    • 13
    多克隆
    标记
    • 20
    • 4
    • 3
    • 3
    • 3
    • 3
    • 3
    • 3
    • 3
    • 2
    • 1
    • 1
    • 1
    • 1
    • 1
    • 1
    This Dysferlin antibody is un-conjugated
    应用范围
    • 14
    • 14
    • 14
    • 9
    • 6
    • 3
    • 1
    • 1
    • 1
    • 1
    • 1
    • 1
    Western Blotting (WB)
    序列
    SRILDESEDT DLPYPPPQRE ANIYMVPQNI KPALQRTAIE ILAWGLRNMK
    预测反应
    Cow: 100%, Dog: 93%, Guinea Pig: 100%, Horse: 100%, Human: 100%, Mouse: 93%, Rabbit: 93%, Rat: 100%, Zebrafish: 92%
    产品特性
    This is a rabbit polyclonal antibody against DYSF. It was validated on Western Blot using a cell lysate as a positive control.
    纯化方法
    Affinity Purified
    免疫原
    The immunogen is a synthetic peptide directed towards the middle region of human DYSF
    Top Product
    Discover our top product DYSF Primary Antibody
  • 应用备注
    Optimal working dilutions should be determined experimentally by the investigator.
    说明

    Antigen size: 2080 AA

    限制
    仅限研究用
  • 状态
    Liquid
    浓度
    Lot specific
    缓冲液
    Liquid. Purified antibody supplied in 1x PBS buffer with 0.09 % (w/v) sodium azide and 2 % sucrose.
    储存液
    Sodium azide
    注意事项
    This product contains Sodium azide: a POISONOUS AND HAZARDOUS SUBSTANCE which should be handled by trained staff only.
    注意事项
    Avoid repeated freeze-thaw cycles.
    储存条件
    -20 °C
    储存方法
    For short term use, store at 2-8°C up to 1 week. For long term storage, store at -20°C in small aliquots to prevent freeze-thaw cycles.
  • Leshinsky-Silver, Argov, Rozenboim, Cohen, Tzofi, Cohen, Wirguin, Dabby, Lev, Sadeh: "Dysferlinopathy in the Jews of the Caucasus: a frequent mutation in the dysferlin gene." in: Neuromuscular disorders : NMD, Vol. 17, Issue 11-12, pp. 950-4, (2007) (PubMed).

  • 抗原
    Dysferlin (DYSF)
    别名
    DYSF (DYSF 产品)
    别名
    DYSF antibody, fb73b05 antibody, wu:fb73b05 antibody, si:rp71-50c18.1 antibody, DKFZp459E1226 antibody, 2310004N10Rik antibody, AI604795 antibody, D6Pas3 antibody, mFLJ00175 antibody, FER1L1 antibody, LGMD2B antibody, MMD1 antibody, dysferlin antibody, dysferlin, limb girdle muscular dystrophy 2B (autosomal recessive) antibody, myoferlin antibody, DYSF antibody, dysf antibody, LOC589501 antibody, Dysf antibody
    背景
    DYSF 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, DYSF 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.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. Publication Note: This RefSeq record includes a subset of the publications that are available for this gene. Please see the Entrez Gene record to access additional publications.
    Alias Symbols: FER1L1, FLJ00175, FLJ90168, LGMD2B, MMD1
    Protein Interaction Partner: UBC, HECW2, HDAC6, CAV3, ANXA2, ANXA1, CAPN3,
    Protein Size: 2080
    分子量
    237 kDa
    基因ID
    8291
    NCBI登录号
    NM_003494, NP_003485
    UniProt
    O75923
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