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产品中心-北京博奥森生物技术有限公司
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Rabbit Anti-alpha Actinin 4-Loading Control/PE-Cy7 Conjugated antibody (bs-1741R-PE-Cy7)
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说 明 书: 100ul  
100ul/2980.00元
大包装/询价
产品编号 bs-1741R-PE-Cy7
英文名称1 Rabbit Anti-alpha Actinin 4-Loading Control/PE-Cy7 Conjugated antibody
中文名称 PE-Cy7标记的α-辅肌动蛋白4(内参)抗体
别    名 actinin 4; Actinin alpha 4; Actinin alpha-4; Alpha-actinin-4; actinin4; ACTN 4; ACTN4; ACTN4_HUMAN; DKFZp686K23158; F actin cross linking protein; F-actin cross-linking protein; Focal segmental glomerulosclerosis 1; FSGS 1; FSGS; FSGS1; Non muscle alpha actinin 4; Non-muscle alpha-actinin 4.  
规格价格 100ul/2980元 购买        大包装/询价
说 明 书 100ul  
产品类型 内参抗体 
研究领域 免疫学  发育生物学  信号转导  干细胞  结合蛋白  细胞骨架  
抗体来源 Rabbit
克隆类型 Polyclonal
交叉反应 Human, Mouse, Rat,  (predicted: Chicken, Pig, Cow, Rabbit, )
产品应用 IF=1:50-200 
not yet tested in other applications.
optimal dilutions/concentrations should be determined by the end user.
分 子 量 105kDa
性    状 Lyophilized or Liquid
浓    度 1mg/ml
免 疫 原 KLH conjugated synthetic peptide derived from human Actinin alpha 4
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.
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.
产品介绍 background:
Loading Control

Alpha actinins belong to the spectrin gene superfamily which represents a diverse group of cytoskeletal proteins, including the alpha and beta spectrins and dystrophins. Alpha actinin is an actin-binding protein with multiple roles in different cell types. In nonmuscle cells, the cytoskeletal isoform is found along microfilament bundles and adherens-type junctions, where it is involved in binding actin to the membrane. In contrast, skeletal, cardiac, and smooth muscle isoforms are localized to the Z-disc and analogous dense bodies, where they help anchor the myofibrillar actin filaments. This gene encodes a nonmuscle, alpha actinin isoform which is concentrated in the cytoplasm, and thought to be involved in metastatic processes. Mutations in this gene have been associated with focal and segmental glomerulosclerosis. [provided by RefSeq, Jul 2008].

Function:
F-actin cross-linking protein which is thought to anchor actin to a variety of intracellular structures. This is a bundling protein. Probably involved in vesicular trafficking via its association with the CART complex. The CART complex is necessary for efficient transferrin receptor recycling but not for EGFR degradation.

Subunit:
Homodimer; antiparallel. Binds TRIM3 at the N-terminus. Identified in a complex with CASK, IQGAP1, MAGI2, NPHS1, SPTAN1 and SPTBN1. Identified in a mRNP granule complex, at least composed of ACTB, ACTN4, DHX9, ERG, HNRNPA1, HNRNPA2B1, HNRNPAB, HNRNPD, HNRNPL, HNRNPR, HNRNPU, HSPA1, HSPA8, IGF2BP1, ILF2, ILF3, NCBP1, NCL, PABPC1, PABPC4, PABPN1, RPLP0, RPS3, RPS3A, RPS4X, RPS8, RPS9, SYNCRIP, TROVE2, YBX1 and untranslated mRNAs. Component of the CART complex, at least composed of ACTN4, HGS/HRS, MYO5B and TRIM3. Interacts with BAIAP1 and PDLIM2.

Subcellular Location:
Nucleus. Cytoplasm. Note=Localized in cytoplasmic mRNP granules containing untranslated mRNAs. Colocalizes with actin stress fibers. Nuclear translocation can be induced by the PI3 kinase inhibitor wortmannin or by cytochalasin D. Exclusively localized in the nucleus in a limited number of cell lines (breast cancer cell line MCF-7, oral floor cancer IMC-2, and bladder cancer KU-7).

Tissue Specificity:
Widely expressed.

DISEASE:
Defects in ACTN4 are the cause of focal segmental glomerulosclerosis type 1 (FSGS1) [MIM:603278]. A renal pathology defined by the presence of segmental sclerosis in glomeruli and resulting in proteinuria, reduced glomerular filtration rate and edema. Renal insufficiency often progresses to end-stage renal disease, a highly morbid state requiring either dialysis therapy or kidney transplantation.

Similarity:
Belongs to the alpha-actinin family.
Contains 1 actin-binding domain.
Contains 2 CH (calponin-homology) domains.
Contains 2 EF-hand domains.
Contains 4 spectrin repeats.

Database links:

Entrez Gene: 81 Human

Entrez Gene: 60595 Mouse

Entrez Gene: 63836 Rat

Omim: 604638 Human

SwissProt: O43707 Human

SwissProt: P57780 Mouse

SwissProt: Q9QXQ0 Rat

Unigene: 270291 Human

Unigene: 81144 Mouse

Unigene: 15777 Rat



Important Note:
This product as supplied is intended for research use only, not for use in human, therapeutic or diagnostic applications.

α-Actinin-4;
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