Overview

  • Product name

    Anti-RPGRIP1L/FTM antibody
    See all RPGRIP1L/FTM primary antibodies
  • Description

    Rabbit polyclonal to RPGRIP1L/FTM
  • Host species

    Rabbit
  • Tested applications

    Suitable for: ICC/IFmore details
  • Species reactivity

    Reacts with: Human
    Predicted to work with: Mouse
  • Immunogen

    Recombinant fragment corresponding to Human RPGRIP1L/FTM aa 194-302.
    Sequence:

    TKYGNSLLEEARGEIRNLENVIQSQRGQIEELEHLAEILKTQLRRKENEI ELSLLQLREQQATDQRSNIRDNVEMIKLHKQLVEKSNALSAMEGKFIQLQ EKQRTLRIS


    Database link: Q68CZ1

  • Positive control

    • ICC/IF: A549 cells.
  • General notes

     This product was previously labelled as RPGRIP1L

     

Properties

  • Form

    Liquid
  • Storage instructions

    Shipped 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 buffer

    pH: 7.20
    Preservative: 0.02% Sodium azide
    Constituents: PBS, 40% Glycerol
  • Concentration information loading...
  • Purity

    Immunogen affinity purified
  • Clonality

    Polyclonal
  • Isotype

    IgG
  • Research areas

Applications

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.

Application Abreviews Notes
ICC/IF Use a concentration of 1 - 4 µg/ml.

Fixation/Permeabilization: PFA/Triton X-100

Target

  • 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.
  • Tissue specificity

    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.
  • Involvement in disease

    Ciliary 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
    COACH syndrome
  • Sequence similarities

    Belongs to the RPGRIP1 family.
    Contains 2 C2 domains.
  • Cellular localization

    Cytoplasm. 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
  • Database links

  • Alternative names

    • CORS 3 antibody
    • CORS3 antibody
    • Fantom antibody
    • FTM antibody
    • FTM_HUMAN antibody
    • JBTS 1 antibody
    • JBTS 7 antibody
    • JBTS1 antibody
    • JBTS7 antibody
    • Joubert syndrome 1 antibody
    • Joubert syndrome 7 antibody
    • Meckel syndrome, type 1 antibody
    • Meckel syndrome, type 5 antibody
    • MKS 5 antibody
    • MKS5 antibody
    • Nephrocystin-8 antibody
    • NPHP 8 antibody
    • NPHP8 antibody
    • Protein fantom antibody
    • Retinitis pigmentosa GTPase regulator interacting protein 1 like antibody
    • RPGR interacting protein 1 like protein antibody
    • RPGR-interacting protein 1-like protein antibody
    • RPGRIP1 like protein antibody
    • RPGRIP1-like protein antibody
    • Rpgrip1l antibody
    see all

Images

  • Immunofluorescent analysis of PFA-fixed, Triton X-100 permeabilized A549 cells labeling RPGRIP1L/FTM with ab220254 at 4 µg/mL (green).

References

ab220254 has not yet been referenced specifically in any publications.

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