Key features and details
- Rabbit polyclonal to RPGRIP1L/FTM
- Suitable for: ICC/IF
- Reacts with: Human
- Isotype: IgG
Product nameAnti-RPGRIP1L/FTM antibody
See all RPGRIP1L/FTM primary antibodies
DescriptionRabbit polyclonal to RPGRIP1L/FTM
Tested applicationsSuitable for: ICC/IFmore details
Species reactivityReacts with: Human
Predicted to work with: Mouse
- ICC/IF: A549 cells.
This product was previously labelled as RPGRIP1L
Storage instructionsShipped at 4°C. Store at +4°C short term (1-2 weeks). Upon delivery aliquot. Store at -20°C long term. Avoid freeze / thaw cycle.
Storage bufferpH: 7.20
Preservative: 0.02% Sodium azide
Constituents: PBS, 40% Glycerol (glycerin, glycerine)
Concentration information loading...
PurityImmunogen affinity purified
Our Abpromise guarantee covers the use of ab220254 in the following tested applications.
The application notes include recommended starting dilutions; optimal dilutions/concentrations should be determined by the end user.
|ICC/IF||Use a concentration of 0.25 - 2 µg/ml.|
FunctionNegatively 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.
Tissue specificityUbiquitously expressed with relatively high level of expression in hypothalamus and islet. During early development, expressed in multiple organs including brain, eye, forelimb and kidney.
Involvement in diseaseCiliary dysfunction leads to a broad spectrum of disorders, collectively termed ciliopathies. Overlapping clinical features include retinal degeneration, renal cystic disease, skeletal abnormalities, fibrosis of various organ, and a complex range of anatomical and functional defects of the central and peripheral nervous system. The ciliopathy range of diseases includes Meckel-Gruber syndrome, Bardet-Biedl syndrome, Joubert syndrome, nephronophtisis, Senior-Loken syndrome, and Jeune asphyxiating thoracic dystrophy among others. Single-locus allelism is insufficient to explain the variable penetrance and expressivity of such disorders, leading to the suggestion that variations across multiple sites of the ciliary proteome, including RPGRIP1L, influence the clinical outcome.
Joubert syndrome 7
Meckel syndrome 5
Sequence similaritiesBelongs to the RPGRIP1 family.
Contains 2 C2 domains.
Cellular localizationCytoplasm. Cytoplasm > cytoskeleton > cilium basal body. Cytoplasm > cytoskeleton > cilium axoneme. Cytoplasm > cytoskeleton > microtubule organizing center > centrosome. Cell junction > tight junction. In cultured renal cells, it localizes diffusely in the cytoplasm but, as cells approach confluence, it accumulates to basolateral tight junctions.
- Information by UniProt
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ab220254 has not yet been referenced specifically in any publications.