Name: KLHL3 Antibody (PACO23772)
SKU: PACO23772
Availability: OutOfStock

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:	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