84-2491-22　NPHP1 (Nephrocystin-1, Juvenile Nephronophthisis 1 Protein, NPH1) (Biotin) 100ul　130498-Biotin

特徴

• Juvenile nephronophthsis, an autosomal recessive cystic kidney disease, is the primary cause for chronic renal failure in children.
• The gene for nephronophthsis type 1 (NPHP1) has recently been identified.
• Nephronophthisis together with Cas it may play a role in the control of epithelial cell polarity.
• Seems to help to recruit protein tyrosine kinase 2 beta (PTK2B) to cell matrix adhesions, thereby initiating phosphorylation of PTK2B and PTK2B-dependent signaling .
• NPHP1 expression has been studied in mouse and human systems.
• Expression in the human adult is widespread but relatively weak.
• In contrast, expression in adult mouse is strong and localized to the testis.
• In situ hybridization to whole mouse embryo demonstrates widespread and consistently strong expression throughout all developmental stages.
• 4 isoforms exist, produced by alternative splicing.
• Applications:Suitable for use in Western Blot.
• Other applications not tested.
• Recommended Dilution:Optimal dilutions to be determined by the researcher.
• AA Sequence:MLARRQRDPLQALRRRNQELKQQVDSLLSESQLKEALEPNKRQHIYQRCIQLKQAIDENKNALQKLSKADESAPVANYNQRKEEEHTLLDKLTQQLQGLAVTISRENITEYASFLPFFFLF
• Storage and Stability:Store product at 4°C if to be used immediately within two weeks.
• For long-term storage, aliquot to avoid repeated freezing and thawing and store at -20°C.
• Aliquots are stable at -20°C for 12 months after receipt.
• Dilute required amount only prior to immediate use.
• Further dilutions can be made in assay buffer.  For maximum recovery of product, centrifuge the original vial after thawing and prior to removing the cap. 
• Note: Applications are based on unconjugated antibody.