phospho-Desmin (Thr76 + Thr77)抗体特异性结合抗原：抗体本身不能直接溶解或杀伤带有特异抗原的靶细胞，通常需要补体或吞噬细胞等共同发挥效应以**病原微生物或导致病理损伤。然而，抗体可通过与病毒或**的特异性结合，直接发挥中和病毒的作用。
产物编号xy- 5302R
英文名称phospho-Desmin (Thr76 + Thr77)
中文名称磷酸化结蛋白抗体
别 名Desmin (phospho T76/77); Desmin (phospho Thr76/Thr77); Desmin (phospho Thr76+Thr77); p-Desmin (Thr76/Thr77); CMD1I; CSM1; CSM2; DES; FLJ12025; FLJ39719; FLJ41013; FLJ41793; Intermediate filament protein; OTTHUMP00000064865; DESM_HUMAN; Desmin; FLJ12025; FLJ39719; FLJ41013; FLJ41793.
说 明 书100ul
产物类型磷酸化抗体
研究领域肿瘤 心血管 **学 信号转导
抗体来源搁补产产颈迟
克隆类型笔辞濒测肠濒辞苍补濒
phospho-Desmin (Thr76 + Thr77)抗体交叉反应 Human,
产物应用WB=1:500-2000 ELISA=1:500-1000 IHC-P=1:400-800 IHC-F=1:400-800 Flow-Cyt=1μg/Test IF=1:100-500 （石蜡切片需做抗原修复）
not yet tested in other applications.
optimal dilutions/concentrations should be determined by the end user.
分 子 量52kDa
细胞定位细胞浆
性 状Lyophilized or Liquid
浓 度1mg/1ml
免 疫 原KLH conjugated Synthesised phosphopeptide derived from human DES around the phosphorylation site of Thr76/Thr77:LG(p-T)(p-T)RT
亚 型IgG
纯化方法affinity purified by Protein A
储 存 液0.01M TBS(pH7.4) with 1% BSA, 0.03% Proclin300 and 50% Glycerol.
phospho-Desmin (Thr76 + Thr77)抗体保存条件Store at -20 °C for one year. Avoid repeated freeze/thaw cycles. The lyophilized antibody is stable at room temperature for at least one month and for greater than a year when kept at -20°C. When reconstituted in sterile pH 7.4 0.01M PBS or diluent of antibody the antibody is stable for at least two weeks at 2-4 °C.
PubMedPubMed
产物介绍产补肠办驳谤辞耻苍诲:
filaments found in muscle cells. In striated muscle they form a fibrous network connecting myofibrils to each other and to the plasma membrane from the periphery of the Z line structures. Defects in Desmin are the cause of desmin related cardio skeletal myopathy (CSM) also known as desmin related myopathy (DRM). CSM is characterized by skeletal muscle weakness associated with cardiac conduction blocks, arrhythmias, restrictive heart failure, and by intracytoplasmic accumulation of desmin reactive deposits in cardiac and skeletal muscle cells. A desmin related myopathy can have a distal onset, it is then known as hereditary distal myopathy (HDM). Defects in Desmin are also the cause of dilated cardiomyopathy type 1I (CMD1I). CMD1I is an autosomal form of dilated cardiomyopathy characterized by ventricular dilatation and impaired systolic function. Antidesmin antibodies are useful in identification of tumours of myogenic origin.
Function:
Desmin are class-III intermediate filaments found in muscle cells. In striated muscle they form a fibrous network connecting myofibrils to each other and to the plasma membrane from the periphery of the Z-line structures.
Subunit:
Homopolymer. Interacts with DST. Interacts with MTM1.
Subcellular Location:
Cytoplasm.
Post-translational modifications:
ADP-ribosylation prevents ability to form intermediate filaments.
DISEASE:
Defects in DES are the cause of myopathy myofibrillar type 1 (MFM1) [MIM:601419]. A neuromuscular disorder characterized by skeletal muscle weakness associated with cardiac conduction blocks, arrhythmias, restrictive heart failure, and by myofibrillar destruction with intracytoplasmic accumulation of desmin-reactive deposits in cardiac and skeletal muscle cells. Note=Mutations in the DES gene are associated with a variable clinical phenotype which encompasses isolated myopathies, pure cardiac phenotypes (including dilated cardiomyopathy, restrictive cardiomyopathy and arrhythmogenic right ventricular cardiomyopathy), cardiac conduction disease, and combinations of these disorders. If both cardiologic and neurologic features occur, they can manifest in any order, as cardiologic features can precede, occur simultaneously with, or follow manifestation of generalized neuromuscular disease (PubMed:19879535).
Defects in DES are the cause of cardiomyopathy dilated type 1I (CMD1I) [MIM:604765]. Dilated cardiomyopathy is a disorder characterized by ventricular dilation and impaired systolic function, resulting in congestive heart failure and arrhythmia. Patients are at risk of premature death.
Defects in DES are the cause of neurogenic scapuloperoneal syndrome Kaeser type (Kaeser syndrome) [MIM:181400]. Kaeser syndrome is an autosomal dominant disorder with a peculiar scapuloperoneal distribution of weakness and atrophy. A large clinical variability is observed ranging from scapuloperoneal, limb grindle and distal phenotypes with variable cardiac or respiratory involvement. Facial weakness, dysphagia and gynaecomastia are frequent additional symptoms. Affected men seemingly bear a higher risk of sudden, cardiac death as compared to affected women. Histological and immunohistochemical examination of muscle biopsy specimens reveal a wide spectrum of findings ranging from near normal or unspecific pathology to typical, myofibrillar changes with accumulation of desmin.
Similarity:
Belongs to the intermediate filament family.
Gene ID:
1674
phospho-Desmin (Thr76 + Thr77)抗体（antibody,
Ab）是由效应B细胞（效应**B细胞）分泌，机体用于抵御外来物质，如病毒，**等抗原，结构呈“驰”字型的球状蛋白质，仅仅存在于脊椎动物的血液和B**细胞膜表面。凡是能够跟抗体结合的物质，均被称作抗原，因此对于抗抗体（能够结合抗体的抗体）来说，抗体本身也是一种抗原物质。
phospho-Desmin (Thr76 + Thr77)抗体普通抗体重链和轻链的结构
重链结构：普通的**球蛋白具有2条重链（H链），分子量约为50kD,有μ、δ、γ、ε和α五种重链亚型，对应的**球蛋白名称分别为IgM、IgG、IgA、IgD和IgE。
轻链结构： 普通**球蛋白具有2条轻链（L链），分子质量约25kDa，有κ链和λ链两种亚型，这两种轻链决定了Ig的亚型类别（IgG1，IgG2，IgG3，IgG4）。一个天然的Ig分子两条轻链总是相同的，但在同一个体内可存在分别带有κ或λ链的抗体分子。不同种属生物体内两型轻链的比例不同，正常人血清**球蛋白κ链：λ链约为2：1，而在小鼠的比例为20：1。
2.2抗体Fab段和Fc段
滨驳骋经木瓜蛋白酶酶切后裂解为2个完全相同的Fab段和1个Fc段,每个Fab段都为单价,可与抗原结合但不会再发生凝集反应；经胃蛋白酶酶切后裂解为1个完整F(ab)2片段和碎片化的Fc片段,F(ab’)2片段为双价,可同时结合两个抗原表位。Fab段为抗原结合片段（fragment of antigen binding，Fab），相当于抗体分子的两个臂，由一个完整的轻链和重链的VH和CH1结构域组成。Fc段为可结晶段（fragment crystallizable，Fc）相当于Ig的CH2和CH3结构域，是Ig与效应分子或者细胞相互作用的部位。Fab段包含完整的可变区，以及恒定区的CH1区域。Fc段仅指Ig恒定区CH2和CH3的区域，相当于Y字结构下面那一部分。
合格 Nanog 胚胎干细胞关键蛋白抗体
合格 合格 GPI 糖磷脂酰肌醇抗体
合格 MEK1 + MEK2 丝裂原活化蛋白激酶激酶1抗体
合格 合格 Collagen I I型胶原蛋白/胶原蛋白1/1型胶原蛋白/I型胶原a1抗体
合格 C2a 补体C2a链多肽抗体
合格 beta Amyloid 1-28 β淀粉样肽1-28/β-Amyloid 1-28抗体
合格 CD90 CD90抗体
合格 CACNB3 L型电压依赖型钙通道β3(L-type Ca++ CPβ3)抗体
合格 NR0B1 肾上腺发育不全相关蛋白抗体
合格 Tex14 癌/睾丸抗原113抗体
合格 B3GALNT1 β1，3半乳糖转移酶3抗体
合格 phospho-MST4 + MST3 + STK25 (Thr178 + Thr190 + Thr174) 磷酸化丝氨酸/苏氨酸蛋白激酶MST4,MST3,STK25抗体
合格 合格 Caspase-1 p20 天冬氨酸-胱氨酸特异性蛋白酶家族抗体
合格 STOM 红细胞膜蛋白STOM抗体
合格 NSE 神经元特异性烯醇化酶/γ 烯醇化酶抗体
合格 HPV16 E7 人类**状瘤病毒16-E7抗体
合格 合格 phospho-HSP70 (Tyr611) 磷酸化热休克蛋白-70抗体
合格 phospho-HSP70 (Tyr41) 磷酸化热休克蛋白-70抗体
合格 Phospho-CD18 (Ser756) 磷酸化整合素β2/Integrin β2抗体
合格 合格 Phospho-CD18 (Thr758) 磷酸化整合素β2/Integrin β2抗体
合格 合格 ADRA1B alpha 1肾上腺素能受体B抗体
合格 DRAK2 DAP凋亡诱导蛋白激酶2抗体
合格 CCL4 巨噬细胞炎症因子1β /MIP-1β抗体
合格 DYNC1I1 胞浆动力蛋白中间链1抗体
合格 DYNC1I2 胞浆动力蛋白中间链2抗体
合格 合格 CATSPER2 阳离子通道精子相关蛋白2抗体
合格 合格 Serine racemase 丝氨酸消旋酶抗体
合格 Complement C5 beta chain 补体C5β链抗体
合格 MyD88 髓样分化蛋白抗体
合格 合格 Heparanase 乙酰肝素酶抗体
合格 Podoplanin 平足蛋白/**管内皮细胞蛋白抗体
合格 phospho-KMT6 (Ser21) 磷酸化抑癌蛋白EZH2抗体
合格 CD3 CD3抗体