Rabbit Polyclonal Antibody to NPHP1

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别名:NPH1; JBTS4; SLSN1
应用:WB,IHC
反应种属:Human, Mouse
规格：50μl/100μl

Description
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.

Description

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.

Specification
Aliases	NPH1; JBTS4; SLSN1
Swissprot	O15259
WB Predicted band size	83 kDa
Host/Isotype	Rabbit IgG
Storage	Store at 4°C short term. Aliquot and store at -20°C long term. Avoid freeze/thaw cycles.
Species Reactivity	Human, Mouse
Immunogen	Fusion protein of human NPHP1
Formulation	pH7.4 PBS, 0.05% NaN3, 40% Glycerol

Application
WB	1/500-1/2000
IHC	1/50-1/300
ELISA	1/5000-1/10000

	Gel: 6%SDS-PAGE, Lysate: 40 μg, Lane: RAW264.7 cell lysate, Primary antibody: P03231(NPHP1 Antibody) at dilution 1/800, Secondary antibody: Goat anti rabbit IgG at 1/5000 dilution, Exposure time: 20 seconds
	The image is immunohistochemistry of paraffin-embedded Human liver cancer tissue using P03231(NPHP1 Antibody) at dilution 1/60. (Original magnification: ×200)
	The image is immunohistochemistry of paraffin-embedded Human colorectal cancer tissue using P03231(NPHP1 Antibody) at dilution 1/60. (Original magnification: ×200)