Anti-NPHP1 Rabbit pAb

Antibody type:Primary antibody

Label:Unconjugated

Modification:Unmodification

Isotype:Rabbit IgG

Host:Rabbit

Application:WB,IF

Purify method:Affinity purified

Species:Human,Mouse,Rat

Gene Name:NPHP1

Synonyms:NPH1; JBTS4; SLSN1

Gene Synonyms:

Gene Full Name:nephrocystin 1

Gene Infomation: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]

Antigen:Recombinant fusion protein containing a sequence corresponding to amino acids 345-614 of human NPHP1 (NP_001121651.1).

Antigen Synonyms:Nephrocystin-1

Clonality:Polyclonal antibody

Source:Human

Reaction:

Form:Liquid

Tested Applications:

• Western blot (1:100 to 1:500)
• Immunofluorescence (1:50 to 1:400)
• Immunohistochemistry (1:200 to 1:500)
• Flow cytometry analysis (1:200 to 1:500)
• Enzyme-linked Immunosorbent Assay (1:100-1:5000)

Note: Users are strongly advised to determine the optimal dilution of antibody to use for their specific applications.

Clone:

Dilution:WB1:500 -1:2000; IF1:50 -1:100(Optimal dilutions should be determined by the end user)

Mole Mass:80kDa

Location:Cell junction, Cell projection, Cilium, Cytoplasm, Cytoskeleton, Tight junction

Concentration:

Sequence Similarity:

Gene Id:

SwissProt ID:O15259

Unigene:4867

Nucleotide Accession:

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).

Storage:Store at -20°C. Avoid freeze / thaw cycles.

Buffer condition:PBS with 0.02% sodium azide, 50% glycerol, pH7.3.

Background:

Molar 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 . 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 .

Western Blot: 

Stability: The thermal stability is described by the loss rate. The loss rate was determined by accelerated thermal degradation test, that is, incubate the protein at 37°C for 48h, and no obvious degradation and precipitation were observed. The loss rate is less than 5% within the expiration date under appropriate storage condition.

Storage: Store at 4°C for frequent use. Stored at -20°C in a manual defrost freezer for one year without detectable loss of activity. Avoid repeated freeze-thaw cycles.

Notes: For In vitro laboratory use only. Not for any clinical, therapeutic, or diagnostic use in humans or animals. Not for animal or human consumption.