Skip to content

Basket

You currently have no items in your basket.

Total (excl. vat) £0.00
View basket & checkout
Antibodies

NPHP1 Polyclonal Antibody

Product Sizes
20 ul
£118.00
E-AB-53023-20UL
60 ul
£183.00
E-AB-53023-60UL
120 ul
£272.00
E-AB-53023-120UL
200 ul
£420.00
E-AB-53023-200UL
About this Product
SKU:
E-AB-53023
Additional Names:
JBTS4,Juvenile nephronophthisis 1 protein,NPH1,NPHP1,Nephrocystin 1,Nephronophthisis,SLSN1,nephronophthisis 1 (juvenile)
Application:
Immunohistochemistry, Western Blot
Buffer:
Phosphate buffered solution, pH 7.4, containing 0.05% stabilizer and 50% glycerol.
Clonality:
Polyclonal
Concentration:
1.02 mg/ml
Extra Details:
This gene encodes a protein with src homology domain 3 (SH3) patterns. This protein interacts with Crk-associated substrate, and it appears to function in the control of cell division, as well as in cell-cell and cell-matrix adhesion signaling, likely as part of a multifunctional complex localized in actin- and microtubule-based structures. Mutations in this gene cause familial juvenile nephronophthisis type 1, a kidney disorder involving both tubules and glomeruli. Defects in this gene are also associated with Senior-Loken syndrome type 1, also referred to as juvenile nephronophthisis with Leber amaurosis, which is characterized by kidney and eye disease, and with Joubert syndrome type 4, which is characterized by cerebellar ataxia, oculomotor apraxia, psychomotor delay and neonatal breathing abnormalities, sometimes including retinal dystrophy and renal disease. Multiple transcript variants encoding different isoforms have been found for this gene.
Host:
Rabbit
Immunogen:
Fusion protein of human NPHP1
Isotype:
IgG
Purification:
Affinity Purified
Reactivities:
Human, Mouse
Shipping Conditions:
Blue Ice
Storage Conditions:
-20[o]C Avoid freeze/thaw cycles.
Supplier:
Elabscience
Type:
Antibody: Polyclonal Antibody