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 (Claudin 16) have been identified as the underlying genetic defect in FHHNC.
Null mutation of 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.
Claudin-16 is only expressed in tight junction of TAL implicated in Mg+2-reabsorption.
Applications:Suitable for use in ELISA, Western Blotting.
Other applications not tested.
Recommended Dilution:Western Blot: 1:1-:1:3K) using ECL technique
ELISA: 1:10,000-50,000 for neat serum and 0.5-1ug/ml for affinity pure.
Control peptide can be used to coat ELISA plates at 1ug/ml.
Optimal dilutions to be determined by the researcher.
Storage and Stability:May be stored at 4°C for short-term only.
For long-term storage and to avoid repeated freezing and thawing, aliquot and add glycerol (40-50%).
Freeze 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:100ul
Host:rabbit
Source Antibody:human
Grade:Serum
Purity:Serum
Form:Supplied as a liquid, neat serum, 40% glycerol.
Specificity:Human Claudin 16 (Paracellin 1) is 70% conserved in bovine.
Isotype:IgG
Calc Applications Abbrev:E WB
Calc Crossreactivity:Hu
Immunogen:A 17 aa peptide sequence within the cytoplasmic, C-terminus of human Claudin 16 (Paracellin 1)