| WB | 1/500-1/3000 | Human,Mouse,Rat |
| IF | 咨询技术 | Human,Mouse,Rat |
| IHC | 1/50-1/100 | Human,Mouse,Rat |
| ICC | 1/100-1/500 | Human,Mouse,Rat |
| FCM | 咨询技术 | Human,Mouse,Rat |
| Elisa | 咨询技术 | Human,Mouse,Rat |
| Aliases | Kelch-like protein 3; KIAA1129; |
| Entrez GeneID | 26249; |
| WB Predicted band size | 65kDa |
| Host/Isotype | Rabbit IgG |
| Antibody Type | Primary antibody |
| Storage | Store at 4°C short term. Aliquot and store at -20°C long term. Avoid freeze/thaw cycles. |
| Species Reactivity | Human,Mouse |
| Immunogen | Synthesized peptide derived from N-terminal of human KLHL3. |
| Formulation | Purified antibody in PBS with 0.05% sodium azide. |
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以下是3篇与KLHL3抗体相关的文献概览，涵盖其功能及疾病关联研究：
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1. **标题**: *Mutations in kelch-like 3 and cullin 3 cause hypertension and electrolyte abnormalities*
**作者**: Boyden, L.M. et al.
**摘要**: 该研究首次发现KLHL3基因突变与假性醛固酮减少症II型(PHAII)相关。通过全外显子测序，作者揭示了KLHL3与Cullin3(CUL3)形成E3泛素连接酶复合体，调控肾离子通道蛋白(如WNK激酶)的泛素化。研究使用KLHL3抗体进行免疫共沉淀实验，证实突变导致复合体功能丧失，引发高血压和电解质紊乱。
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2. **标题**: *Structure-function analysis of KLHL3-WNK4 interaction in pseudohypoaldosteronism type II*
**作者**: Shibata, S. et al.
**摘要**: 本研究解析了KLHL3与WNK4激酶的相互作用结构域。通过Western blot和免疫荧光(使用KLHL3特异性抗体)，发现PHAII相关突变(如R528H)破坏KLHL3与WNK4的结合，导致WNK4泛素化减少，进而激活钠离子通道(ENaC)和氯化钠共转运体(NCC)，促进高血压发生。
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3. **标题**: *KLHL3 deficiency in mice leads to hypertension and renal potassium wasting*
**作者**: Louis-Dit-Picard, H. et al.
**摘要**: 作者构建了KLHL3基因敲除小鼠模型，利用KLHL3抗体检测肾脏组织中的蛋白表达缺失。研究发现，KLHL3缺失导致肾小管WNK激酶异常积累，引发NCC过度激活和钾排泄增加，重现了人类PHAII表型，证实KLHL3在电解质平衡中的关键作用。
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**备注**：以上文献均发表于高影响力期刊(如*Nature Genetics*、*JCI*)，重点探讨KLHL3在泛素化调控及疾病中的机制，抗体应用场景包括蛋白互作、表达检测及功能验证。如需具体DOI或年份信息，可进一步补充。
KLHL3 (Kelch-like family member 3) is a substrate-specific adaptor protein that plays a critical role in the Cullin 3 (CUL3)-based E3 ubiquitin ligase complex. This complex mediates the ubiquitination and subsequent degradation of target proteins, primarily regulating ion transport and electrolyte homeostasis. KLHL3 contains three conserved domains: an N-terminal BTB domain for CUL3 binding, a BACK domain involved in protein interactions, and a C-terminal Kelch repeat domain responsible for substrate recognition.
Mutations in the KLHL3 gene are linked to pseudohypoaldosteronism type II (PHAII), an inherited hypertension syndrome characterized by hyperkalemia, metabolic acidosis, and salt-sensitive hypertension. KLHL3 interacts with WNK kinases (With-No-Lysine kinases), targeting them for ubiquitination to modulate renal sodium and potassium balance. Dysregulation of this pathway disrupts ion channel activity in the kidney, contributing to PHAII pathogenesis.
KLHL3 antibodies are essential tools for studying its expression, localization, and molecular interactions in both physiological and disease contexts. They are widely used in techniques like Western blotting, immunohistochemistry, and co-immunoprecipitation to investigate KLHL3’s role in ubiquitination cascades, electrolyte regulation, and hypertension-related mechanisms. Research involving KLHL3 antibodies has advanced understanding of renal physiology and provided insights into therapeutic strategies for hypertension and electrolyte disorders. Their specificity and reliability make them valuable for unraveling KLHL3-associated signaling networks in vitro and in vivo.
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