phospho-FLNC (2233)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。
产品编号xy- 13183R
英文名称phospho-FLNC (2233)
中文名称磷酸化细丝蛋白2抗体
别 名FLNC (phospho S2233); p-Filamin 2 (phospho S2233); p-FLNC (phospho S2233); ABP 280; ABP L; ABPL; Actin binding like protein; Actin binding protein 280; Filamin 2; Filamin2; Filamin-2; Filamin C; Filamin C gamma; FLJ10186; FLN 2; FLN2; FLNC; Gamma actin binding protein; Gamma filamin; Protein FLNc; FLNC_HUMAN; Filamin-C; FLN-C; ABP-280-like protein; ABP-L; Actin-binding-like protein.
说 明 书100ul
产品类型磷酸化抗体
研究领域细胞生物 神经生物学 信号转导 细胞骨架 新陈代谢
抗体来源Rabbit
克隆类型Polyclonal
phospho-FLNC (2233)抗体交叉反应 Human, Mouse, Rat, Dog, Pig, Cow, Horse, Sheep,
产品应用IHC-P=1:400-800 IHC-F=1:400-800 ICC=1:100-500 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300kDa
细胞定位细胞浆 细胞膜
性 状Lyophilized or Liquid
浓 度1mg/1ml
免 疫 原KLH conjugated synthesised phosphopeptide derived from human FLNC around the phosphorylation site of Ser2233:LG(p-S)FG
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-FLNC (2233)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
产品介绍background:
Filamins are Actin-binding proteins which contain an N-terminal Actin-binding domain, a membrane glycoprotein domain and a C-terminal self-association domain. Filamins help reshape the cytoskeleton by forming flexible cross-links between two Actin filaments, which maintain membrane integrity during force application. Filamins also participate in signal transduction pathways associated with cell motility, adhesion, differentiation and survival, and force transduction. The filamin family is comprised of Filamin 1, Filamin 2 and Filamin 3. Filamin 2, also designated Filamin C, is a skeletal- and cardiac-muscle specific form of Filamin, which binds ©-sarcoglycan and ∂-sarcoglycan, but not å-sarcoglycan or ∫-sarcoglycan. Muscular dystrophy, an inherited group of disorders resulting in progressive weakness of muscles in the body, is associated with irregular subcellular localization of Filamin 2 caused by a deficiency in KY, a protein that interacts with Filamin 2.
Function:
FLNC is a muscle-specific filamin, which plays a central role in muscle cells, probably by functioning as a large actin-cross-linking protein. May be involved in reorganizing the actin cytoskeleton in response to signaling events, and may also display structural functions at the Z-disks in muscle cells. Defects in FLNC are the cause of autosomal dominant filaminopathy. Myofibrillar myopathy (MFM) is a neuromuscular disorder, usually with an ***** onset, characterized by focal myofibrillar destruction and pathological cytoplasmic protein aggregations. Autosomal dominant filaminopathy is a form of MFM characterized by morphological features of MFM and clinical features of a limb-girdle myopathy. A heterozygous nonsense mutation which segregates with the disease, has been identified in the FLNC gene.
Subunit:
Homodimer. Interacts with KY. Interacts with IGFN1. Interacts with FLNB, KCND2, ITGB1A, INPPL1, MYOT, MYOZ1 and MYOZ3. Interacts with sarcoglycans SGCD and SGCG. Interacts (via filament repeats 17-18, 20-21 and 24) with USP25 (isoform USP25m only). Interacts with FBLIM1.
Subcellular Location:
Cytoplasm. Membrane; Peripheral membrane protein. Cytoplasm, cytoskeleton. Cytoplasm, myofibril, sarcomere, Z line. Note=A small amount localizes at membranes. In striated muscle cells, it predominantly localizes in myofibrillar Z lines, while a minor fraction localizes with subsarcolemme.
Tissue Specificity:
Highly expressed in striated muscles. Weakly expressed in thyroid, fetal brain, fetal lung, retina, spinal cord and bone marrow. Not expressed in testis, pancreas, adrenal gland, placenta, liver and kidney.
Post-translational modifications:
Ubiquitinated by FBXL22, leading to proteasomal degradation.
DISEASE:
Defects in FLNC are the cause of myopathy myofibrillar type 5 (MFM5) [MIM:609524]. A neuromuscular disorder, usually with an ***** onset, characterized by focal myofibrillar destruction and pathological cytoplasmic protein aggregations, and clinical features of a limb-girdle myopathy.
Defects in FLNC are the cause of myopathy distal type 4 (MPD4) [MIM:614065]. MPD4 is a slowly progressive muscular disorder characterized by distal muscle weakness and atrophy affecting the upper and lower limbs. Onset occurs around the third to fourth decades of life, and patients remain ambulatory even after long disease duration. Muscle biopsy shows non-specific changes with no evidence of rods, necrosis, or inflammation.
Similarity:
elongs to the filamin family.
Contains 1 actin-binding domain.
Contains 2 CH (calponin-homology) domains.
Contains 24 filamin repeats.
SWISS:
Q14315
Gene ID:
2318
phospho-FLNC (2233)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“Y”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-FLNC (2233)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
IgG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。
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合格 CYP4A1 细胞色素P450 4A1抗体
合格 CYP4A22 细胞色素P4504A22抗体（脂肪酸Ω羟化酶）
合格 DHCR7 脱氢胆固醇还原酶7抗体
合格 合格 HMGCS1 三羟基**基辅酶A合成酶1
合格 LASS5 长寿相关基因lASS5抗体
合格 TNFSF15 肿瘤坏死因子配体超家族成员15抗体
合格 TECK 胸腺表达趋化因子抗体
合格 IGFBP7 胰岛素样生长因子结合蛋白7抗体
合格 WISP2 结缔组织生长因子样蛋白抗体
合格 CLIC1 氯离子通道蛋白27抗体
合格 Cystatin A 胱抑素A/半胱氨酸蛋白酶抑制剂A抗体
合格 Hsc70 热休克蛋白71抗体
合格 合格 TUBA1C 微管相关蛋白α6抗体
合格 Laminin B2 gamma 1 层粘连蛋白B2γ1抗体
合格 ITLN1 内皮细胞凝集素HL1抗体
合格 ALDH1B1 乙醛脱氢酶5抗体
合格 BAG2 Bcl-2结合抑凋亡蛋白2抗体
合格 CREM 环磷酸腺苷反应元件调节蛋白抗体
合格 合格 TFEB T**细胞转录调节因子TFEB抗体
合格 TAF12 TATA框结合蛋白关联因子12抗体
合格 DP1 转录因子DP1抗体
合格 合格 AKT3 蛋白激酶B3
合格 合格 Cdc7 细胞分裂周期蛋白7抗体
合格 phospho-LSP1 (Ser252) 磷酸化白细胞F肌动蛋白结合蛋白抗体