KLHL3 Antibody

說明書

中文名稱：

KLHL3兔多克隆抗體
貨號：

CSB-PA012431GA01HU
規格：

￥3,900
其他：

產品詳情
產品評價
相關產品
靶點詳情

產品詳情

Uniprot No.：

Q9UH77
基因名：

KLHL3
別名：

KLHL3 antibody; KIAA1129 antibody; Kelch-like protein 3 antibody
宿主：

Rabbit
反應種屬：

Human,Mouse,Rat
免疫原：

Human KLHL3
免疫原種屬：

Homo sapiens (Human)
抗體亞型：

IgG
純化方式：

Antigen Affinity purified
濃度：

It differs from different batches. Please contact us to confirm it.
保存緩沖液：

PBS with 0.1% Sodium Azide, 50% Glycerol, pH 7.3. -20°C, Avoid freeze / thaw cycles.
產品提供形式：

Liquid
應用范圍：

ELISA,WB,IHC
Protocols：

ELISA Protocol
Western Blotting (WB) Protocol
Immunohistochemistry (IHC) Protocol
儲存條件：

Upon receipt, store at -20°C or -80°C. Avoid repeated freeze.
貨期：

Basically, we can dispatch the products out in 1-3 working days after receiving your orders. Delivery time maybe differs from different purchasing way or location, please kindly consult your local distributors for specific delivery time.
用途：

For Research Use Only. Not for use in diagnostic or therapeutic procedures.

產品評價

靶點詳情

功能：

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 acts by mediating ubiquitination of WNK4, an inhibitor of potassium channel KCNJ1, leading to WNK4 degradation. The BCR(KLHL3) complex also mediates ubiquitination and degradation of CLDN8, a tight-junction protein required for paracellular chloride transport in the kidney.
基因功能參考文獻：
1. A new recessive mutation in KLHL3 (S553L) was identified in familial hyperkalemia and hypertension. Increased urinary NCC was found in affected members (heterozygous) with dominant KLHL3 Q309R, and in affected members (homozygous) of the recessive form. PMID: 28511177
2. Mutation in the KLHL3 gene is associated with Gordon syndrome. PMID: 28222034
3. The results demonstrate that Hcy decreases the expression of cMyBP-C through a KLHL3-mediated ubiquitin-proteasome pathway, and thereby influences heart development. PMID: 28315668
4. This study provides substantial new insights into the role of phosphorylation of KLHL3 in regulating the interaction with WNK4 PMID: 27727489
5. Data indicate that WNK lysine deficient protein kinase 4 protein (WNK4) was degraded not only by proteasomes but also by atypical protein kinase C scaffold protein p62 (p62)-kelch-like 3 protein (KLHL3)-mediated selective autophagy. PMID: 26349538
6. Familial hyperkalemia and hypertension caused by KLHL3 mutations is accompanied by hypercalciuria as well as hyperkalemia and hypertension. PMID: 25925082
7. Akt and PKA phosphorylated KLHL3 at S433, and phosphorylation of KLHL3 by PKA inhibited WNK4 degradation. PMID: 26435498
8. KLHL3 is phosphorylated at serine 433 in the Kelch domain (a site frequently mutated in hypertension with hyperkalemia) by protein kinase C in cultured cells and that this phosphorylation prevents WNK4 binding and degradation. PMID: 25313067
9. Hyperkalemic hypertension-associated cul3 mutations depletes KLHL3, preventing WNK degradation, despite increased CUL3-mediated WNK ubiquitylation. PMID: 25250572
10. CUL3 and KLHL3 gene products are physiologically important regulators of thiazide-sensitive distal nephron sodium chloride reabsorption. PMID: 24266877
11. analysis of how mutations of KLHL3 show less ability to ubiquitinate WNK4 because of KLHL3's low stability and/or decreased binding to CUL3 or WNK4 PMID: 23962426
12. Disease causing mutations in human KLHL3 disrupt the interaction with CUL3, a crystallographic study. PMID: 23573258
13. KLHL3 is a substrate adaptor for WNK4 in a ubiquitin E3 ligase complex PMID: 23665031
14. CUL3 and KLHL3 have roles in in electrolyte homeostasis and in Pseudohypoaldosteronism type II PMID: 23576762
15. The CUL3-KLHL3 E3 ligase complex mutated in Gordon's hypertension syndrome interacts with and ubiquitylates WNK isoforms: disease-causing mutations in KLHL3 and WNK4 disrupt interaction. PMID: 23387299
16. identified KLHL3 as a third gene responsible for familial hyperkalemic hypertension; study establishes a role for KLHL3 as a new member of the complex signaling pathway regulating ion homeostasis in the distal nephron and indirectly blood pressure PMID: 22406640
17. fundamental role for KLHL3 and CUL3 in blood pressure, K(+) and pH homeostasis PMID: 22266938
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相關疾病：

Pseudohypoaldosteronism 2D (PHA2D)
亞細胞定位：

Cytoplasm, cytoskeleton. Cytoplasm, cytosol.
組織特異性：

Widely expressed.
數據庫鏈接：

HGNC: 6354

OMIM: 605775

KEGG: hsa:26249

STRING: 9606.ENSP00000312397

UniGene: Hs.655084

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