C5838-90A

Claudin 16, Human, Control Peptide (Paracellin 1, CLDN16)

Size

£416.00

SKU:: C5838-90A
Purity:: Chromatographically purified
Storage Conditions:: -20°C
Supplier:: United States Biological
Format:: Supplied as a liquid in PBS, pH 7.2
Extra Details:: A 17 aa peptide sequence within the cytoplasmic, C-terminus of human Paracellin 1. Magnesium as a cofactor is required in many cellular activities. Mg+2 reabsorption in the kidney is mediated primarily be a poorly understood paracellular pathway (passage of solutes between the cells) in the thick ascending limb of Henle (TAL). Tight junctions constitute the barrier to paracellular conductance. Familial hypomagnesaemia with hypercalciuria and nephrocalcinosis (FHHNC, MIM 248250) is a complex renal tubular disorder characterized by hypomagnesaemia, hypercalciuria, advanced nephrocalcinosis, hyposthenuria and progressive renal failure. The mode of inheritance is autosomal recessive. A primary defect in the reabsorption of magnesium in the TAL has been proposed to be essential in FHHNC pathophysiology. Recently, mutations in the gene paracellin-1 (Paracellin 1)/Claudin-16) have been identified as the underlying genetic defect in FHHNC. Null mutation of Paracellin 1)/Claudin-16 has been shown to produce chronic interstitial nephritis in cattle. Paracellin 1) gene codes for a protein of 305aa (chromosome 3q27), with four TM domains and intracellular NH2- and COOH-termini. Paracellin 1) belongs to the Claudin family of proteins. It is 10-18% related with claudins. Paracellin 1) is only expressed in tight junction of TAL implicated in Mg+2-reabsorption. Applications: ELISA: 1:10-50K for neat serum and 0.5-1ug/ml for affinity pure.

Shipping Conditions:: Blue Ice