Rabbit anti-Human CLCN5 Polyclonal Antibody | anti-CLCN5 antibody

CLCN5, NT (CLCN5, CLCK2, H(+)/Cl(-) exchange transporter 5, Chloride channel protein 5, Chloride transporter ClC-5) (AP)

Cat. Num. AAA6286371

• Gene Names: CLCN5; XRN; CLC5; XLRH; CLCK2; ClC-5; DENTS; NPHL1; NPHL2; hCIC-K2
• Reactivity: Human
• Applications: Western Blot, ELISA
• Purity: Purified by Protein A Affinity Chromatography.
• Synonyms: CLCN5; Polyclonal Antibody; NT (CLCN5; CLCK2; H(+)/Cl(-) exchange transporter 5; Chloride channel protein 5; Chloride transporter ClC-5) (AP); Chloride transporter ClC-5; anti-CLCN5 antibody

• Host: Rabbit
• Reactivity: Human
• Clonality: Polyclonal
• Isotype: IgG
• Purity/Purification: Purified by Protein A Affinity Chromatography.
• Form/Format: Supplied as a liquid in PBS, pH 7.2. No preservative added. Labeled with Alkaline Phosphatase (AP).

• Applicable Applications for anti-CLCN5 antibody: Western Blot (WB), ELISA (EIA)
• Application Notes: WB: 1:100-500; ELISA: 1:1,000; Applications are based on unconjugated antibody.
• Immunogen: CLCN5 antibody is generated from rabbits immunized with a KLH conjugated synthetic peptide between 15-41 amino acids from the N-terminal region of human CLCN5.
• Conjugate: AP
• Note: Preservative Free
• Preparation and Storage: Store product at 4 degree C. DO NOT FREEZE! Stable at 4 degree C for 12 months after receipt as an undiluted liquid. Dilute required amount only prior to immediate use. Further dilutions can be made in assay buffer. For maximum recovery of product, centrifuge the original vial prior to removing the cap.

Western Blot (WB)

(CLCN5 Antibody (N-term) western blot analysis in K562 cell line lysates (35ug/lane).This demonstrates the CLCN5 antibody detected the CLCN5 protein (arrow).)

Related Product Information for anti-CLCN5 antibody

This gene encodes a member of the ClC family of chloride ion channels and ion transporters. Mutations in this gene have been found in Dent disease and renal tubular disorders complicated by nephrolithiasis. Alternatively spliced transcript variants encoding different isoforms have been found for this gene. [provided by RefSeq].

Product Categories/Family for anti-CLCN5 antibody

Antibodies; Transport Proteins

References

Smith, A.J., et al. FASEB J. 24(10):3696-3705(2010). Wellhauser, L., et al. Pflugers Arch. 460(2):543-557(2010). Smith, A.J., et al. J. Physiol. (Lond.) 588 (PT 12), 2033-2045 (2010): Picollo, A., et al. J. Gen. Physiol. 135(6):653-659(2010). Reed, A.A., et al. Am. J. Physiol. Renal Physiol. 298 (2), F365-F380 (2010).

NCBI and Uniprot Product Information

• NCBI GI #: 4557473
• NCBI GeneID: 1184
• NCBI Accession #: NP_000075.1
• NCBI GenBank Nucleotide #: NM_000084.4
• UniProt Accession #: P51795; Q5JQD5; Q7RTN8; A1L475; B3KPN6
• Molecular Weight: 83,147 Da
• NCBI Official Full Name: H(+)/Cl(-) exchange transporter 5 isoform b
• NCBI Official Synonym Full Names: chloride channel, voltage-sensitive 5
• NCBI Official Symbol: CLCN5
• NCBI Official Synonym Symbols: XRN; CLC5; XLRH; CLCK2; ClC-5; DENTS; NPHL1; NPHL2; hCIC-K2
• NCBI Protein Information: H(+)/Cl(-) exchange transporter 5; H(+)/Cl(-) exchange transporter 5; chloride transporter ClC-5; voltage-gated chloride ion channel CLCN5
• UniProt Protein Name: H(+)/Cl(-) exchange transporter 5
• Protein Family: H(+)/Cl(-) exchange transporter
• UniProt Gene Name: CLCN5
• UniProt Synonym Gene Names: CLCK2
• UniProt Entry Name: CLCN5_HUMAN

Uniprot Description

CLCN5 iso2: Proton-coupled chloride transporter. Functions as antiport system and exchanges chloride ions against protons. Important for normal acidification of the endosome lumen. May play an important role in renal tubular function. Defects in CLCN5 are a cause of hypophosphatemic rickets, X-linked recessive (XLRHR). XLRHR is a renal disease belonging to the 'Dent disease complex', a group of disorders characterized by proximal renal tubular defect, hypercalciuria, nephrocalcinosis, and renal insufficiency. The spectrum of phenotypic features is remarkably similar in the various disorders, except for differences in the severity of bone deformities and renal impairment. XLRH patients present with rickets or osteomalacia, hypophosphatemia due to decreased renal tubular phosphate reabsorption, hypercalciuria, and low molecular weight proteinuria. Patients develop nephrocalcinosis with progressive renal failure in adulthood. Female carriers may have asymptomatic hypercalciuria or hypophosphatemia only. Defects in CLCN5 are the cause of nephrolithiasis type 2 (NPHL2); also known as Dent disease 1. NPHL2 is an X- linked recessive renal disease belonging to the 'Dent disease complex'. NPHL2 patients manifest hypercalciuria, hypophosphatemia, aminoaciduria, nephrocalcinosis and nephrolithiasis, renal insufficiency leading to renal failure in adulthood, rickets (33% of patients) and osteomalacia. Defects in CLCN5 are the cause of nephrolithiasis type 1 (NPHL1); also designated XRN. NPHL1 is an X-linked recessive renal disease belonging to the 'Dent disease complex'. NPHL1 presents with hypercalciuria, nephrocalcinosis, renal stones and renal insufficiency. Patients lack urinary acidification defects, rickets, and osteomalacia. Defects in CLCN5 are the cause of low molecular weight proteinuria with hypercalciuria and nephrocalcinosis (LMWPHN). LMWPHN is an X-linked renal disease belonging to the 'Dent disease complex'. Patients tend to have hypercalciuric nephrocalcinosis without rickets or renal failure. Belongs to the chloride channel (TC 2.A.49) family. ClC-5/CLCN5 subfamily. 2 isoforms of the human protein are produced by alternative splicing.

Protein type: Transporter; Transporter, ion channel; Membrane protein, integral; Membrane protein, multi-pass

Chromosomal Location of Human Ortholog: Xp11.23-p11.22

Cellular Component: Golgi membrane; membrane; integral to plasma membrane; lysosomal membrane; apical part of cell; endosome membrane

Molecular Function: chloride channel activity; voltage-gated chloride channel activity; ATP binding; antiporter activity

Biological Process: transport; endocytosis; excretion; transmembrane transport

Disease: Proteinuria, Low Molecular Weight, With Hypercalciuria And Nephrocalcinosis; Nephrolithiasis, X-linked Recessive, With Renal Failure; Dent Disease 1; Hypophosphatemic Rickets, X-linked Recessive