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Western Blot (WB) (Western blot analysis NPHP1 using HepG2 whole cell lysates)

Rabbit anti-Human, Mouse NPHP1 Polyclonal Antibody | anti-NPHP1 antibody

NPHP1 Antibody

Gene Names
NPHP1; NPH1; JBTS4; SLSN1
Reactivity
Human, Mouse
Applications
Western Blot, ELISA
Purity
The antiserum was purified by peptide affinity chromatography using SulfoLink Coupling Resin.
Synonyms
NPHP1; Polyclonal Antibody; NPHP1 Antibody; JBTS4; Juvenile nephronophthisis 1 protein; Nephrocystin 1; nephronophthisis 1 (juvenile); Nephronophthisis; NPH1; SLSN1; anti-NPHP1 antibody
Ordering
For Research Use Only!
Host
Rabbit
Reactivity
Human, Mouse
Clonality
Polyclonal
Isotype
IgG
Specificity
NPHP1 antibody detects endogenous levels of total NPHP1
Purity/Purification
The antiserum was purified by peptide affinity chromatography using SulfoLink Coupling Resin.
Form/Format
Liquid
Phosphate buffered saline, pH 7.4, 150mM NaCl, 0.02% sodium azide and 50% glycerol.
Concentration
1mg/ml (varies by lot)
Sequence Length
733
Applicable Applications for anti-NPHP1 antibody
Western Blot (WB), ELISA (EIA)
Application Notes
WB: 1:1000-3000
Immunogen
A synthesized peptide derived from human NPHP1
Subcellular Location
Cell Junction, Adherens Junction. Cell Projection, Cilium. Cytoplasm, Cytoskeleton, Cilium Axoneme. Cell Junction, Tight Junction. 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 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).
Conjugation
Unconjugated
Preparation and Storage
Store at -20 degree C. Stable for 12 months from date of receipt.

Western Blot (WB)

(Western blot analysis NPHP1 using HepG2 whole cell lysates)

Western Blot (WB) (Western blot analysis NPHP1 using HepG2 whole cell lysates)
Related Product Information for anti-NPHP1 antibody
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).
Subunit Structure: Interacts with BCAR1, PTK2B/PYK2 and tensin. Interacts with INVS and NPHP3. Interacts with PACS1; the interaction is dependent on NPHP1 phosphorylation by CK2. Interacts with KIF7. Interacts with AHI1 and TNK2. Interacts with NPHP4 in a complex containing NPHP1, NPHP4 and RPGRIP1L. Interacts with IQCB1; the interaction likely requires additional interactors. Interacts with ANKS3 (By similarity).
Post-translational Modifications: Phosphorylation by CK2 is required for the interaction with PACS1 and the targeting to the base region of cilia.
Similarity: The SH3 domain mediates the stable interaction with Cas. Belongs to the nephrocystin-1 family.

NCBI and Uniprot Product Information

NCBI GI #
NCBI GeneID
NCBI Accession #
NCBI GenBank Nucleotide #
UniProt Accession #
Molecular Weight
Observed: 83 kDa
Predicted: 84 kDa
NCBI Official Full Name
nephrocystin-1 isoform 1
NCBI Official Synonym Full Names
nephrocystin 1
NCBI Official Symbol
NPHP1
NCBI Official Synonym Symbols
NPH1; JBTS4; SLSN1
NCBI Protein Information
nephrocystin-1
UniProt Protein Name
Nephrocystin-1
Protein Family
UniProt Gene Name
NPHP1
UniProt Synonym Gene Names
NPH1

NCBI 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. [provided by RefSeq, Jul 2008]

Uniprot Description

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 (). Does not seem to be strictly required for ciliogenesis (). 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 ().

Research Articles on NPHP1

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Product Notes

The NPHP1 nphp1 (Catalog #AAA9609995) is an Antibody produced from Rabbit and is intended for research purposes only. The product is available for immediate purchase. The NPHP1 Antibody reacts with Human, Mouse and may cross-react with other species as described in the data sheet. AAA Biotech's NPHP1 can be used in a range of immunoassay formats including, but not limited to, Western Blot (WB), ELISA (EIA). WB: 1:1000-3000. Researchers should empirically determine the suitability of the NPHP1 nphp1 for an application not listed in the data sheet. Researchers commonly develop new applications and it is an integral, important part of the investigative research process. It is sometimes possible for the material contained within the vial of "NPHP1, Polyclonal Antibody" to become dispersed throughout the inside of the vial, particularly around the seal of said vial, during shipment and storage. We always suggest centrifuging these vials to consolidate all of the liquid away from the lid and to the bottom of the vial prior to opening. Please be advised that certain products may require dry ice for shipping and that, if this is the case, an additional dry ice fee may also be required.

Precautions

All products in the AAA Biotech catalog are strictly for research-use only, and are absolutely not suitable for use in any sort of medical, therapeutic, prophylactic, in-vivo, or diagnostic capacity. By purchasing a product from AAA Biotech, you are explicitly certifying that said products will be properly tested and used in line with industry standard. AAA Biotech and its authorized distribution partners reserve the right to refuse to fulfill any order if we have any indication that a purchaser may be intending to use a product outside of our accepted criteria.

Disclaimer

Though we do strive to guarantee the information represented in this datasheet, AAA Biotech cannot be held responsible for any oversights or imprecisions. AAA Biotech reserves the right to adjust any aspect of this datasheet at any time and without notice. It is the responsibility of the customer to inform AAA Biotech of any product performance issues observed or experienced within 30 days of receipt of said product. To see additional details on this or any of our other policies, please see our Terms & Conditions page.

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