Mouse H (+) /Cl (-) exchange transporter 5 (CLCN5) ELISA kit; CLC5; CLCK2; DENTS; NPHL1; NPHL2; XLRH; XRN; hCIC-K2; hClC-K2; OTTHUMP00000023279; OTTHUMP00000023281; chloride channel 5

For Research Use Only. Not for use in diagnostic procedures.

Store all reagents at 2-8 degree C

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Small volumes of CLCN5 elisa kit vial(s) may occasionally become entrapped in the seal of the product vial during shipment and storage. If necessary, briefly centrifuge the vial on a tabletop centrifuge to dislodge any liquid in the container`s cap. Certain products may require to ship with dry ice and additional dry ice fee may apply.

MBS9331253 is a ready-to-use microwell, strip plate ELISA (enzyme-linked immunosorbent assay) Kit for analyzing the presence of the chloride channel 5 (CLCN5) ELISA Kit target analytes in biological samples. The concentration gradients of the kit standards or positive controls render a theoretical kit detection range in biological research samples containing CLCN5. The ELISA analytical biochemical technique of the MBS9331253 kit is based on CLCN5 antibody-CLCN5 antigen interactions (immunosorbency) and an HRP colorimetric detection system to detect CLCN5 antigen targets in samples. The ELISA Kit is designed to detect native, not recombinant, CLCN5. Appropriate sample types may include undiluted body fluids and/or tissue homogenates, secretions. Quality control assays assessing reproducibility identified the intra-assay CV (%) and inter-assay CV(%).

83,101 Da

chloride channel 5

H(+)/Cl(-) exchange transporter 5; H(+)/Cl(-) exchange transporter 5; chloride channel protein 5; chloride transporter ClC-5

H(+)/Cl(-) exchange transporter 5

Clc5; ClC-5??[Similar Products]

CLCN5_MOUSE

CLCN5: 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 *****hood. 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 *****hood, 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: Membrane protein, integral; Transporter, ion channel; Membrane protein, multi-pass; Transporter

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

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

Biological Process: transport; endocytosis; chloride transport; ion transport; transmembrane transport

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