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NPHP1 / Nephronophthisis Antibody

Rabbit Polyclonal Antibody

     
  • IHC-P - NPHP1 / Nephronophthisis Antibody ALS17155
    Human Tonsil: Formalin-Fixed, Paraffin-Embedded (FFPE)
  • IHC-P - NPHP1 / Nephronophthisis Antibody ALS17155
    Human Colon: Formalin-Fixed, Paraffin-Embedded (FFPE)
  • SPECIFICATION
  • CITATIONS
  • PROTOCOLS
  • BACKGROUND
Product Information
Application
  • Applications Legend:
  • WB=Western Blot
  • IHC=Immunohistochemistry
  • IHC-P=Immunohistochemistry (Paraffin-embedded Sections)
  • IHC-F=Immunohistochemistry (Frozen Sections)
  • IF=Immunofluorescence
  • FC=Flow Cytopmetry
  • IC=Immunochemistry
  • ICC=Immunocytochemistry
  • E=ELISA
  • IP=Immunoprecipitation
  • DB=Dot Blot
  • CHIP=Chromatin Immunoprecipitation
  • FA=Fluorescence Assay
  • IEM=Immunoelectronmicroscopy
  • EIA=Enzyme Immunoassay
WB, IHC-P, E
Primary Accession O15259
Other Accession 4867
Reactivity Human
Host Rabbit
Clonality Polyclonal
Isotype IgG
Calculated MW 83299 Da
Dilution IHC-P (10 µg/ml),
Additional Information
Gene ID 4867
Other Names NPHP1, JBTS4, Nephrocystin 1, Nephronophthisis, SLSN1, Nephrocystin-1, Nephronophthisis 1 (juvenile), NPH1
Target/Specificity Human NPHP1 / Nephronophthisis
Reconstitution & Storage PBS, pH 7.4, 0.03% Proclin 300, 50% glycerol. Long term: -20°C; Short term: +4°C. Avoid repeat freeze-thaw cycles.
PrecautionsNPHP1 / Nephronophthisis Antibody is for research use only and not for use in diagnostic or therapeutic procedures.
Protein Information
Name NPHP1
Synonyms NPH1
Function Together with BCAR1 it may play a role in the control of epithelial cell polarity. Involved in the organization of apical junctions in kidney cells together with NPHP4 and RPGRIP1L/NPHP8 (By similarity). Does not seem to be strictly required for ciliogenesis (By similarity). Seems to help to recruit PTK2B/PYK2 to cell matrix adhesions, thereby initiating phosphorylation of PTK2B/PYK2 and PTK2B/PYK2-dependent signaling. May play a role in the regulation of intraflagellar transport (IFT) during cilia assembly. Required for normal retina development. In connecting photoreceptor cilia influences the movement of some IFT proteins such as IFT88 and WDR19. Involved in spermatogenesis (By similarity).
Cellular Location Cell junction, adherens junction. Cell projection, cilium. Cytoplasm, cytoskeleton, cilium axoneme. Cell junction, tight junction. Note=Colocalizes with E-cadherin and BCAR1 at or near the cell-cell adherens junctions (By similarity). Localized to respiratory cilia axoneme Localized to the transition zone of respiratory cilia, photoreceptor-connecting cilia and renal monocilia. In cultured renal cells, it localizes diffusely in the cytoplasm but, as cells approach confluence, it accumulates to basolateral tight junctions.
Tissue Location 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).
Research Areas
Citations (0)

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Background

Together with BCAR1 it may play a role in the control of epithelial cell polarity. Involved in the organization of apical junctions in kidney cells together with NPHP4 and RPGRIP1L/NPHP8 (By similarity). Does not seem to be strictly required for ciliogenesis (By similarity). Seems to help to recruit PTK2B/PYK2 to cell matrix adhesions, thereby initiating phosphorylation of PTK2B/PYK2 and PTK2B/PYK2-dependent signaling. May play a role in the regulation of intraflagellar transport (IFT) during cilia assembly. Required for normal retina development. In connecting photoreceptor cilia influences the movement of some IFT proteins such as IFT88 and WDR19. Involved in spermatogenesis (By similarity).

References

Saunier S.,et al.Hum. Mol. Genet. 6:2317-2323(1997).
Hildebrandt F.,et al.Nat. Genet. 17:149-153(1997).
Hillier L.W.,et al.Nature 434:724-731(2005).
Caridi G.,et al.Am. J. Kidney Dis. 32:1059-1062(1998).
Mollet G.,et al.Nat. Genet. 32:300-305(2002).

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$ 395.00
Cat# ALS17155
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