Signal Transduction Antibodies 1
Anti-KLHL3 Antibody (CAB13771)
- SKU:
- CAB13771
- Product Type:
- Antibody
- Reactivity:
- Human
- Reactivity:
- Mouse
- Host Species:
- Rabbit
- Isotype:
- IgG
- Antibody Type:
- Polyclonal Antibody
- Research Area:
- Signal Transduction
Description
Antibody Name: | Anti-KLHL3 Antibody |
Antibody SKU: | CAB13771 |
Antibody Size: | 20uL, 50uL, 100uL |
Application: | WB |
Reactivity: | Human, Mouse |
Host Species: | Rabbit |
Immunogen: | Recombinant fusion protein containing a sequence corresponding to amino acids 1-110 of human KLHL3 (NP_059111.2). |
Application: | WB |
Recommended Dilution: | WB 1:500 - 1:2000 |
Reactivity: | Human, Mouse |
Positive Samples: | 293T, A-549, Mouse liver |
Immunogen: | Recombinant fusion protein containing a sequence corresponding to amino acids 1-110 of human KLHL3 (NP_059111.2). |
Purification Method: | Affinity purification |
Storage Buffer: | Store at -20'C. Avoid freeze / thaw cycles. Buffer: PBS with 0.02% sodium azide, 50% glycerol, pH7.3. |
Isotype: | IgG |
Sequence: | MEGE SVKL SSQT LIQA GDDE KNQR TITV NPAH MGKA FKVM NELR SKQL LCDV MIVA EDVE IEAH RVVL AACS PYFC AMFT GDMS ESKA KKIE IKDV DGQT LSKL IDYI YT |
Gene ID: | 26249 |
Uniprot: | Q9UH77 |
Cellular Location: | Cytoplasm, cytoskeleton, cytosol |
Calculated MW: | 55kDa/61kDa/64kDa |
Observed MW: | 75kDa |
Synonyms: | KLHL3, PHA2D |
Background: | 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. |
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: | KLHL3 |
NCBI Official Synonym Symbols: | PHA2D |
NCBI Protein Information: | kelch-like protein 3 |
UniProt Protein Name: | Kelch-like protein 3 |
Protein Family: | Kelch-like protein |
UniProt Gene Name: | KLHL3 |
UniProt Entry Name: | KLHL3_HUMAN |
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