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- bs-13183P
- 2025年10月16日
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- 技术资料
- 规格：
500ug
| 产品编号 | bs-13183P |
| 英文名称 | phospho-FLNC (2233) Antibody Blocking Peptide |
| 中文名称 | 磷酸化细丝蛋白2封闭多肽 |
| 英文别名 | 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. |
| 纯化方法 | HPLC |
| 亚基 | 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. |
| 亚细胞定位 | 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. |
| 组织特异性 | 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. |
| 翻译后修饰 | Ubiquitinated by FBXL22, leading to proteasomal degradation. |
| 相似性 | elongs to the filamin family. Contains 1 actin-binding domain. Contains 2 CH (calponin-homology) domains. Contains 24 filamin repeats. |
| 功能 | 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 adult 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. |
| 保存条件 | Shipped at 4℃. Stored at -20℃ for one year. Avoid repeated freeze/thaw cycles. |
| 注意事项 | This product as supplied is intended for research use only, not for use in human, therapeutic or diagnostic applications. |
| 背景资料 | 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. |
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phospho-FLNC (2233) Antibody Blocking Peptide(bs-13183P)-500ug
￥1080
