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Rabbit Anti-NPHP1 Polyclonal Antibody#abs139198

Description

Catalog-specification	Delivery time	USD price
abs139198-100ug	1-2 Weeks	301.0
abs139198-50ug	1-2 Weeks	201.0

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Overview
catalog	abs139198
Other names	Nephronophthisis 1, also referred to as juvenile nephronophthisis 1 protein, is a genetic disorder that arises due to mutations and abnormalities in the NPHP1 gene. NPHP1 codes for a protein known as nephrocystin 1, which works to maintain the kidney's structure and functionality. Individuals affected by this disorder experience progressive kidney damage and ultimately develop end-stage renal disease, necessitating the need for dialysis or kidney transplantation. The symptoms of nephronophthisis include increased urination, excessive thirst, fatigue, anemia, and hypertension. The disorder usually becomes apparent in childhood or adolescence, but onset and severity can vary. Early detection and intervention are critical in managing this disease and preventing it from progressing to end-stage renal disease. Diagnosis is typically made through genetic testing and evaluation of the patient's clinical symptoms and medical history. Nephronophthisis is inherited in an autosomal recessive manner, meaning that both parents of the affected child must be carriers of the NPHP1 gene mutation. Carriers experience no symptoms, but their offspring have a 25% chance of being affected by the disease. Research efforts are ongoing in developing targeted therapies and treatments for nephronophthisis, and genetic counseling is advised for families with a history of the disorder.
Source	Rabbit
Specificity	The ability of the NPHP1 Antibody to detect total NPHP1 at its endogenous levels has been established. The content presented above has been rearranged to provide a highly similar description, ensuring it is derived from the original textual information.
Species Reactivity	Human;Mouse
Antigen	NPHP1
Application	WB 1:1000-3000, ELISA(peptide) 1:20000-1:40000
Immunogen	A synthesized peptide derived from human NPHP1.
MW	83 kDa
Properties
Concentration	1mg/ml
purification	The SulfoLink™ Coupling Resin was utilized for peptide affinity chromatography in order to purify the antiserum. Please rearrange the provided information to craft a highly similar content, ensuring that the generated content is based on the original text.
Clonality	Polyclonal Antibody
Stability & Storage	To maintain the quality of the product, it is recommended to store it in a freezer at a temperature of -20 °C for a period of one year. It is important to avoid subjecting the product to repeated cycles of freezing and thawing, which could have a negative impact on its stability and efficacy. By following these storage guidelines, you can be assured that the product will remain effective and safe for its intended use.
Storage buffer	This product contains Rabbit IgG in a solution of phosphate buffered saline with a pH level of 7.4, 150mM NaCl, 0.02% sodium azide, and 50% glycerol. It is recommended to store this product at a temperature of -20°C for optimal preservation. This solution will remain stable for up to 12 months from the date of receipt, ensuring its long-term viability for users. Order this product to achieve accurate and reliable results in your research endeavors.
Target
Background	Possible rearrangement: NPHP1, together with BCAR1, appears to regulate the polarity of epithelial cells. It contributes to the formation of apical junctions in kidney cells, and works with NPHP4 and RPGRIP1L/NPHP8 to achieve this. While not strictly necessary for ciliogenesis, it may assist in recruiting PTK2B/PYK2 to cell-matrix adhesions, thereby initiating signaling dependent on these proteins. In addition, NPHP1 may help regulate intraflagellar transport during cilia assembly. Its function is essential for proper development of the retina; in particular, it plays a role in connecting photoreceptor cilia and influencing the movement of IFT proteins such as IFT88 and WDR19. Finally, NPHP1 is also involved in spermatogenesis.
Tissue specificity	Widespread expression, with highest levels in pituitary gland, spinal cord, thyroid gland, testis, skeletal muscle, lymph node and trachea. Weakly expressed in heart, kidney and pancreas. Expressed in nasal epithelial cells (at protein level).
Posttranslational modification	Phosphorylation by CK2 is required for the interaction with PACS1 and the targeting to the base region of cilia.
Celluar localization	Cytoskeleton;Cytosol;
UniPort	O15259