KLHL3 Antibody

Code CSB-PA890684HA01HU
Size US$166
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  • Immunohistochemistry of paraffin-embedded human adrenal gland tissue using CSB-PA890684HA01HU at dilution of 1:100

  • Immunohistochemistry of paraffin-embedded human pancreatic tissue using CSB-PA890684HA01HU at dilution of 1:100

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Product Details

Full Product Name
Rabbit anti-Homo sapiens (Human) KLHL3 Polyclonal antibody
Uniprot No.
Target Names
KLHL3
Alternative Names
KLHL3 antibody; KIAA1129 antibody; Kelch-like protein 3 antibody
Raised in
Rabbit
Species Reactivity
Human
Immunogen
Recombinant Human Kelch-like protein 3 protein (1-301AA)
Immunogen Species
Homo sapiens (Human)
Conjugate
Non-conjugated

The KLHL3 Antibody (Product code: CSB-PA890684HA01HU) is Non-conjugated. For KLHL3 Antibody with conjugates, please check the following table.

Available Conjugates
Conjugate Product Code Product Name Application
HRP CSB-PA890684HB01HU KLHL3 Antibody, HRP conjugated ELISA
FITC CSB-PA890684HC01HU KLHL3 Antibody, FITC conjugated
Biotin CSB-PA890684HD01HU KLHL3 Antibody, Biotin conjugated ELISA
Clonality
Polyclonal
Isotype
IgG
Purification Method
>95%, Protein G purified
Concentration
It differs from different batches. Please contact us to confirm it.
Buffer
Preservative: 0.03% Proclin 300
Constituents: 50% Glycerol, 0.01M PBS, PH 7.4
Form
Liquid
Tested Applications
ELISA, IHC
Recommended Dilution
Application Recommended Dilution
IHC 1:20-1:200
Troubleshooting and FAQs
Storage
Upon receipt, store at -20°C or -80°C. Avoid repeated freeze.
Lead Time
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.

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Target Background

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 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.
Gene References into Functions
  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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Involvement in disease
Pseudohypoaldosteronism 2D (PHA2D)
Subcellular Location
Cytoplasm, cytoskeleton. Cytoplasm, cytosol.
Tissue Specificity
Widely expressed.
Database Links

HGNC: 6354

OMIM: 605775

KEGG: hsa:26249

STRING: 9606.ENSP00000312397

UniGene: Hs.655084

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