KLHL3 Antibody (PACO23772)
- SKU:
- PACO23772
- Product Type:
- Antibody
- Reactivity:
- Human
- Host Species:
- Rabbit
- Isotype:
- IgG
- Applications:
- ELISA
- WB
- IHC
- IF
- Antibody Type:
- Polyclonal Antibody
- Conjugation:
- Unconjugated
Frequently bought together:
Description
| Antibody Name: | KLHL3 Antibody (PACO23772) |
| Antibody SKU: | PACO23772 |
| Size: | 100ul |
| Host Species: | Rabbit |
| Tested Applications: | ELISA, WB, IHC, IF |
| Recommended Dilutions: | ELISA:1:2000-1:10000, WB:1:500-1:3000, IHC:1:50-1:100, IF:1:100-1:500 |
| Species Reactivity: | Human |
| Immunogen: | Synthesized peptide derived from N-terminal of human KLHL3. |
| Form: | Liquid |
| Storage Buffer: | Rabbit IgG in phosphate buffered saline (without Mg2+ and Ca2+), pH 7.4, 150mM NaCl, 0.02% sodium azide and 50% glycerol. |
| Purification Method: | The antibody was affinity-purified from rabbit antiserum by affinity-chromatography using epitope-specific immunogen. |
| Clonality: | Polyclonal |
| Isotype: | IgG |
| Conjugate: | Non-conjugated |
 | Western blot analysis of extracts from RAW264.7 cells and 3T3 cells, using KLHL3 antibody. |
 | Immunofluorescence analysis of A549 cells, using KLHL3 antibody. |
 | Immunohistochemistry analysis of paraffin-embedded human brain tissue, using KLHL3 antibody. |
| Background: | 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. |
| Synonyms: | Kelch-like protein 3; KIAA1129; |
| UniProt Protein Function: | KLHL3: 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. Defects in KLHL3 are the cause of Pseudohypoaldosteronism type 2D (PHA2D). 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. 3 isoforms of the human protein are produced by alternative splicing. |
| UniProt Protein Details: | Protein type:Cytoskeletal; Actin-binding Chromosomal Location of Human Ortholog: 5q31 Cellular Component: cytosol Molecular Function:protein binding; structural molecule activity; ubiquitin-protein ligase activity Biological Process: ion homeostasis; protein ubiquitination; protein ubiquitination during ubiquitin-dependent protein catabolic process Disease: Pseudohypoaldosteronism, Type Iid |
| NCBI Summary: | This gene is ubiquitously expressed and encodes a full-length protein which has an N-terminal BTB domain followed by a BACK domain and six kelch-like repeats in the C-terminus. These kelch-like repeats promote substrate ubiquitination of bound proteins via interaction of the BTB domain with the CUL3 (cullin 3) component of a cullin-RING E3 ubiquitin ligase (CRL) complex. Muatations in this gene cause pseudohypoaldosteronism type IID (PHA2D); a rare Mendelian syndrome featuring hypertension, hyperkalaemia and metabolic acidosis. Alternative splicing results in multiple transcript variants encoding distinct isoforms. [provided by RefSeq, Mar 2012] |
| UniProt Code: | Q9UH77 |
| NCBI GenInfo Identifier: | 13431657 |
| NCBI Gene ID: | 26249 |
| NCBI Accession: | Q9UH77.2 |
| UniProt Secondary Accession: | Q9UH77,Q9UH75, Q9UH76, Q9ULU0, Q9Y6V6, B2RBK7, |
| UniProt Related Accession: | Q9UH77 |
| Molecular Weight: | 55,927 Da |
| NCBI Full Name: | Kelch-like protein 3 |
| NCBI Synonym Full Names: | kelch like family member 3 |
| NCBI Official Symbol: | KLHL3Â Â |
| NCBI Official Synonym Symbols: | PHA2DÂ Â |
| NCBI Protein Information: | kelch-like protein 3 |
| UniProt Protein Name: | Kelch-like protein 3 |
| Protein Family: | Kelch-like protein |
| UniProt Gene Name: | KLHL3Â Â |
| UniProt Entry Name: | KLHL3_HUMAN |
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