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 nephrocalcinL6060606osis, 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 (PCLN-1/Claudin-16) have been identified as the underlying genetic defect in FHHNC.
Null mutation of PCLN-1/Claudin-16 has been shown to produce chronic interstitial nephritis in cattle.
PCLN-1 gene codes for a protein of 305 aa (chromosome 3q27), with four TM domains and intracellular NH2- and COOH-termini.
PCLN-1 belongs to the Claudin family of proteins.
It is 10-18% related with claudins.
PCLN-1 is only expressed in tight junction of TAL implicated in Mg+2-reabsorption.
Applications:Suitable for use in ELISA.
Western Blot, though not tested, may potentially be used as an application.
Other applications not tested.
Recommended Dilution:Western Blot: 1:1000-:1:3000 using ECL technique
ELISA: 0.5-1ug/ml for affinity pure
Optimal dilutions to be determined by the researcher.
Storage and Stability:For long-term storage, aliquot and store at -20°C.
Aliquots are stable for at least 12 months at -20°C.
For maximum recovery of product, centrifuge the original vial after thawing and prior to removing the cap.
Further dilutions can be made in assay buffer.
仕様
Size：100ug
Host：rabbit
Source Antibody：human
Grade：Affinity Purified
Purity：Purified by immunoaffinity chromatography.
Form：Supplied as a liquid in 100mM Tris, pH 7.5, 0.2% BSA, 0.05% sodium azide, 40% glycerol.
Specificity：Recognizes human Paracellin-1. Species Sequence Homology: bovine (70%)
