|Other Names||Protein fantom, Nephrocystin-8, RPGR-interacting protein 1-like protein, RPGRIP1-like protein, RPGRIP1L, FTM, KIAA1005, NPHP8|
|Target/Specificity||The synthetic peptide sequence used to generate the antibody AP8567a was selected from the N-term region of human RPGRIP1L. A 10 to 100 fold molar excess to antibody is recommended. Precise conditions should be optimized for a particular assay.|
|Format||Synthetic peptide was lyophilized with 100% acetonitrile and is supplied as a powder. Reconstitute with 0.1 ml DI water for a final concentration of 1 mg/ml.|
|Storage||Maintain refrigerated at 2-8°C for up to 6 months. For long term storage store at -20°C.|
|Precautions||This product is for research use only. Not for use in diagnostic or therapeutic procedures.|
|Synonyms||FTM, KIAA1005, NPHP8|
|Function||Negatively regulates signaling through the G-protein coupled thromboxane A2 receptor (TBXA2R). May be involved in mechanisms like programmed cell death, craniofacial development, patterning of the limbs, and formation of the left-right axis (By similarity). Involved in the organization of apical junctions in kidney cells together with NPHP1 and NPHP4 (By similarity). Does not seem to be strictly required for ciliogenesis (By similarity).|
|Cellular Location||Cytoplasm. Cytoplasm, cytoskeleton, cilium basal body. Cytoplasm, cytoskeleton, cilium axoneme. Cytoplasm, cytoskeleton, microtubule organizing center, centrosome. Cell junction, tight junction. Note=In cultured renal cells, it localizes diffusely in the cytoplasm but, as cells approach confluence, it accumulates to basolateral tight junctions|
|Tissue Location||Ubiquitously expressed with relatively high level of expression in hypothalamus and islet. During early development, expressed in multiple organs including brain, eye, forelimb and kidney.|
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Provided below are standard protocols that you may find useful for product applications.
RPGRIP1L can localize to the basal body-centrosome complex or to primary cilia and centrosomes in ciliated cells. This protein has been found to interact with nephrocystin-4. Defects in this gene are a cause of Joubert syndrome type 7 (JBTS7) and Meckel syndrome type 5 (MKS5).
Brancati,F., et.al., Clin. Genet. 74 (2), 164-170 (2008)Stratigopoulos,G., et.al., Am. J. Physiol. Regul. Integr. Comp. Physiol. 294 (4), R1185-R1196(2008)
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