84-1048-21　PHEX (PEX, Phosphate-regulating Gene with Homologies to Endopeptidases on the X Chromosome) 100ul　P4070-68B

特徴

• The amyloid beta-peptide (Ab) of 39-43aa (Ab40, Ab42, Ab43) is constitutively produced in brain upon proteolysis of the b-amyloid precursor protein (APP).
• In the young and healthy humans, Ab is rapidly catabolized before it can be deposited in the brain.
• However, upon aging or the onset of familial Alzheimer's Disease, alterations in either synthesis or degradation/clearance of Ab may contribute to amyloid depositions in the brain.
• Ab is susceptible to a number of in vivo and in vitro proteases like cathepsin-D and M-13 metalloproteases.
• The M-13 family includes several members of zinc-dependent proteases like damage-induced neuronal endopeptidase (DINE), product of phosphate regulating gene with homologies to endopeptidases on the X chromosome (PHEX), neutral endopeptidase 24.11 or neprilysin (NEP) and neprilysin-like proteases (NEPLs).
• NEPLs (alpha, beta, gamma) arise from the alternative splicing of a single NELPS gene and show ~54% sequence homology.
• M13 members are generally type II transmembrane proteins consisting of a single polypeptide chain with Zinc binding HEXXH motif, a short cytoplasmic tail, a transmembrane segment and an extra-cytoplasmic domain containing the enzyme active site.
• Phosphate regulating gene with homologies to endopeptidases on the X chromosome (PHEX) (formerly PEX) is a zinc-containing, type II integral membrane glycoprotein (~110kD; mouse/rat/human: 749aa; chromosome xp22.2-p22.1) with structural resemblance to M13 NEP proteases.
• In contrast to NEP, PHEX has very narrow substrate specificity and hydrolyzes parathroid hormone related peptide (PTHrP107-139).
• Compared to NEP, PHEX can proteolyze Ab40 (but not Ab42), at a very low rate and to a very poor extent and therefore is not a major in vivo peptidase for Ab.
• However, it might degrade Ab in Alzheimer patients where Ab is accumulated in excess and an Ab-degradative pathway, alternative to NEP, exists.
• PHEX is expressed lymphocytes and fetal brain but not in adult brain, placenta, skeletal muscle, pancreas, heart, liver and lung.
• Defects in PHEX are a cause of X-linked hypophosphatemic rickets (HYP).
• PHEX is implicated in bone and dentin mineralization and renal phosphate 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: 1:1000-1:5000 using ECL.
• ELISA: 1:10,000-1:100,000 using 50-100ng of control peptide/well
• Optimal dilutions to be determined by the researcher.
• Control Peptide:P4070-68C: PHEX, Rat, Control Peptide (PEX, Phosphate-regulating Gene with Homologies to Endopeptidases on the X Chromosome)
• Storage and Stability:May be stored at 4°C for short-term only.
• For long-term storage, 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.