NPHP1 Polyclonal Antibody - BT-AP11982

NPHP1 Polyclonal Antibody

Sizes: 20μL, 50μL, 100μL

Catalogue Numbers: BT-AP11982-20, BT-AP11982-50, BT-AP11982-100

Citations, Manuals and MSDS Available upon request.

Background: 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 diffe

Research Areas: Cell biology; Signal transduction

Target Protein: NPHP1

Applications: WB, ELISA

Reactivity: Human, Mouse

Clonality: Polyclonal

Host: Rabbit

Isotype: IgG

Full Product Name: Nephrocystin-1

Immunogen: The antiserum was produced against synthesized peptide derived from human AMPK alpha around the phosphorylation site of Thr172. AA range:140-189

Storage: -20°C for 1 year

Purification: The antibody was affinity-purified from rabbit antiserum by affinity-chromatography using epitope-specific immunogen.

Formulation: Liquid in PBS containing 50% glycerol, 0.5% BSA and 0.02% sodium azide.

Concentration: 1 mg/ml

UniProt Accession: Human: O15259; Mouse: Q9QY53

Synonyms: Nephrocystin-1;Juvenile nephronophthisis 1 protein

GeneID: Human: 4867

Research Use Only