KLHL3,Kelch样蛋白3抗体

产品编号HZ-8053R
英文名称KLHL3
中文名称Kelch样蛋白3抗体
别 名KLHL 3; FLJ40871; kelch (Drosophila) like 3; kelch like 3 (Drosophila); kelch like 3; Kelch like protein 3; KIAA1129; MGC44594; KLHL3_HUMAN.
说 明 书0.1ml 0.2ml
研究领域细胞生物 免疫学
抗体来源Rabbit
克隆类型Polyclonal
交叉反应 Human, Mouse, Rat, Chicken, Dog, Pig, Cow, Horse, Rabbit, Sheep,
KLHL3,Kelch样蛋白3抗体产品应用WB=1:100-500 ELISA=1:500-1000 IHC-P=1:100-500 IHC-F=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65kDa
细胞定位细胞浆
性 状Lyophilized or Liquid
浓 度1mg/1ml
免 疫 原KLH conjugated synthetic peptide derived from human KLHL3
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.
KLHL3,Kelch样蛋白3抗体PubMedPubMed
产品介绍background:
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.

KLHL3,Kelch样蛋白3抗体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.

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

KLHL3,Kelch样蛋白3抗体Important Note:
This product as supplied is intended for research use only, not for use in human, therapeutic or diagnostic applications. 

产品图片	Lane 1: (human/cell line)A549lysatesprobed withKLHL3Polyclonal Antibody, Unconjugated (Catalog #HZ-8053R) at 1:300 overnight at 4ⅹC. Followed by a conjugated secondary antibody (Secondary Catalog #926-32211) at 1:10000 for 60 min at 37ⅹC.