Overview

  • Product name

  • Description

    Rabbit polyclonal to CRB1
  • Host species

    Rabbit
  • Tested applications

    Suitable for: IHC-P, ICC/IFmore details
  • Species reactivity

    Reacts with: Human
  • Immunogen

    Recombinant fragment corresponding to Human CRB1 aa 20-200.
    Database link: P82279

  • Positive control

    • IHC-P: Human eye tissue. ICC/IF: HeLa cells.

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: 7.30
    Preservative: 0.02% Sodium azide
    Constituents: PBS, 50% Glycerol
  • Concentration information loading...
  • Purity

    Immunogen affinity purified
  • Clonality

    Polyclonal
  • Isotype

    IgG
  • Research areas

Applications

Our Abpromise guarantee covers the use of ab238501 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
IHC-P 1/20 - 1/200.
ICC/IF 1/10 - 1/100.

Target

  • Function

    Plays a role in photoreceptor morphogenesis in the retina. May maintain cell polarization and adhesion.
  • Tissue specificity

    Preferential expression in retina, also expressed in brain, testis, fetal brain and fetal eye.
  • Involvement in disease

    Note=CRB1 mutations have been found in various retinal dystrophies, chronic and disabling disorders of visual function. They predominantly involve the posterior portion of the ocular fundus, due to degeneration in the sensory layer of the retina, retinal pigment epithelium, Bruch membrane, choroid, or a combination of these tissues. Onset of inherited retinal dystrophies is painless, bilateral and typically progressive. Most people experience gradual peripheral vision loss or tunnel vision, and difficulties with poor illumination and night vision. Central vision is usually unaffected, so the person may still be able to read. However, it can also deteriorate to cause total blindness. Examples of retinal dystrophies are retinitis pigmentosa, Leber congenital amaurosis, cone-rod dystrophy among others.
    Defects in CRB1 are the cause of retinitis pigmentosa type 12 (RP12) [MIM:600105]. A retinal dystrophy belonging to the group of pigmentary retinopathies. Retinitis pigmentosa is characterized by retinal pigment deposits visible on fundus examination and primary loss of rod photoreceptor cells, followed by secondary loss of cone photoreceptors. Patients typically have night vision blindness and loss of midperipheral visual field. As their condition progresses, they lose their far peripheral visual field and eventually central vision as well. RP12 is an autosomal recessive severe form oFTen manifesting in early childhood. Patients experiment progressive visual field loss with severe visual impairment before the age of twenty. Some patients have a preserved paraarteriolar retinal pigment epithelium (PPRPE) and hypermetropia.
    Defects in CRB1 are the cause of Leber congenital amaurosis type 8 (LCA8) [MIM:613835]. LCA designates a clinically and genetically heterogeneous group of childhood retinal degenerations, generally inherited in an autosomal recessive manner. Affected infants have little or no retinal photoreceptor function as tested by electroretinography. LCA represents the most common genetic cause of congenital visual impairment in infants and children.
    Defects in CRB1 are the cause of pigmented paravenous chorioretinal atrophy (PPCRA) [MIM:172870]. PPCRA is an unusual retinal degeneration characterized by accumulation of pigmentation along retinal veins. PPCRA is dominantly inherited, but exhibited variable expressivity. Males are more likely to exhibit a severe phenotype, whereas females may remain virtually asymptomatic even in later years. The PPCRA phenotype is associated with a mutation in CRB1 gene which is likely to affect the structure of the CRB1 protein.
  • Sequence similarities

    Belongs to the Crumbs protein family.
    Contains 19 EGF-like domains.
    Contains 3 laminin G-like domains.
  • Post-translational
    modifications

    Extensively glycosylated.
  • Cellular localization

    Secreted and Apical cell membrane. Distributed at the apical membrane of all retinal epithelial cells. Located in the apical membrane of the adherens junction in outer limiting membrane (OLM) of the retina.
  • Information by UniProt
  • Database links

  • Alternative names

    • CRB1 antibody
    • CRUM1_HUMAN antibody
    • Protein crumbs homolog 1 antibody

Images

  • Paraffin-embedded human eye tissue stained for CRB1 using ab238501 at 1/66 dilution in immunohistochemical analysis.

    After dewaxing and hydration, antigen retrieval was mediated by high pressure in a citrate buffer (pH 6.0). Section was blocked with 10% normal goat serum 30 minutes at RT. Then primary antibody (1% BSA) was incubated at 4°C overnight. The primary is detected by a biotinylated secondary antibody and visualized using an HRP conjugated SP system.

  • HeLa (Human epithelial cell line from cervix adenocarcinoma) cells labeling CRB1 (Green) using ab238501 at 1/22 dilution in ICC/IF, followed by Alexa Fluor®488-conjugated Goat Anti-Rabbit IgG (H+L) secondary antibody. The cells were fixed in 4% formaldehyde, permeabilized using 0.2% Triton X-100 and blocked in 10% normal goat serum. The cells were then incubated with the antibody overnight at 4°C. Counterstained with DAPI.

References

ab238501 has not yet been referenced specifically in any publications.

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