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KLHL3 Rabbit pAb (bs-8053R)  
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50ul/1180.00元
100ul/1980.00元
200ul/2800.00元
大包装/询价

产品编号 bs-8053R
英文名称 KLHL3 Rabbit pAb
中文名称 Kelch样蛋白3抗体
别    名 PHA2D;   
研究领域 细胞生物  免疫学  
抗体来源 Rabbit
克隆类型 Polyclonal
克 隆 号
交叉反应 Human (predicted: Mouse,Rat,Rabbit,Pig,Sheep,Cow,Chicken,Dog,Horse)
产品应用 WB=1:500-2000
not yet tested in other applications.
optimal dilutions/concentrations should be determined by the end user.
理论分子量 65 kDa
细胞定位 细胞浆 
性    状 Liquid
浓    度 1mg/ml
免 疫 原 KLH conjugated synthetic peptide derived from human KLHL3: 51-160/587 
亚    型 IgG
纯化方法 affinity purified by Protein A
缓 冲 液 0.01M TBS (pH7.4) with 1% BSA, 0.02% Proclin300 and 50% Glycerol.
保存条件 Shipped at 4℃. Store 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.
PubMed PubMed
产品介绍 KLHL3 protein contains a poxvirus and zinc finger domain at the N-terminus and six tandem repeats (kelch repeats) at the C-terminus. At the amino acid level, KLHL3 shares 77% similarity with Drosophila kelch and 89% similarity with Mayven (KLHL2), another human kelch homolog. At least three isoforms are produced and may be the result of alternative promoter usage. The KLHL3 maps within the smallest commonly deleted segment in myeloid leukemias characterized by a deletion of 5q; however, no inactivating mutations of KLHL3 could be detected in malignant myeloid disorders with loss of 5q.

Function:
Substrate-specific adapter of a BCR (BTB-CUL3-RBX1) E3 ubiquitin ligase complex that acts as a regulator of ion transport in the distal nephron. The BCR(KLHL3) complex may act by mediating ubiquitination of SLC12A3/NCC, thereby regulating SLC12A3/NCC subcellular location at the cell membrane.

Subunit:
Component of the BCR(KLHL3) E3 ubiquitin ligase complex, at least composed of CUL3 and KLHL3 and RBX1 (Probable). Interacts with SLC12A3.

Subcellular Location:
Cytoplasm, cytoskeleton. Cytoplasm, cytosol.

Tissue Specificity:
Widely expressed.

DISEASE:
Defects in KLHL3 are the cause of Pseudohypoaldosteronism type 2D (PHA2D) [MIM:614495]. A disorder characterized by severe hypertension, hyperkalemia, hyperchloremia, hyperchloremic metabolic acidosis, and correction of physiologic abnormalities by thiazide diuretics. PHA2D inheritance is autosomal dominant or recessive.

Similarity:
Contains 1 BACK (BTB/Kelch associated) domain.
Contains 1 BTB (POZ) domain.
Contains 6 Kelch repeats.

SWISS:
Q9UH77.2

Gene ID:
26249

Database links:
UniProtKB/Swiss-Prot: Q9UH77.2

产品图片
Sample: HL-60 Cell (Human) Lysate at 40 ug Primary: Anti-KLHL3 (bs-8053R) at 1/300 dilution Secondary: IRDye800CW Goat Anti-Rabbit IgG at 1/20000 dilution Predicted band size: 65 kD Observed band size: 60 kD
Sample: A549 Cell (Human) Lysate at 40 ug Primary: Anti-KLHL3 (bs-8053R) at 1/300 dilution Secondary: IRDye800CW Goat Anti-Rabbit IgG at 1/20000 dilution Predicted band size: 65 kD Observed band size: 60 kD
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