Western Blot (WB)
(Western blot analysis of CRB1 in human small intestine Tissue lysate with CRB1 antibody at 1 μg/mL.)
Rabbit anti-Human CRB1 Polyclonal Antibody | anti-CRB1 antibody
CRB1 Antibody
Western Blot (WB)
(Western blot analysis of CRB1 in human small intestine Tissue lysate with CRB1 antibody at 1 μg/mL.)
Western Blot (WB)
(Western blot analysis of CRB1 in human small intestine Tissue lysate with CRB1 antibody at 1 μg/mL.)
NCBI and Uniprot Product Information
NCBI Description
This gene encodes a protein which is similar to the Drosophila crumbs protein and localizes to the inner segment of mammalian photoreceptors. In Drosophila crumbs localizes to the stalk of the fly photoreceptor and may be a component of the molecular scaffold that controls proper development of polarity in the eye. Mutations in this gene are associated with a severe form of retinitis pigmentosa, RP12, and with Leber congenital amaurosis. Alternate splicing results in multiple transcript variants, some protein coding and some non-protein coding.[provided by RefSeq, Apr 2012]
Uniprot Description
CRB1: Plays a role in photoreceptor morphogenesis in the retina. May maintain cell polarization and adhesion. 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). 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). 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). 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. Belongs to the Crumbs protein family. 4 isoforms of the human protein are produced by alternative splicing.
Protein type: Motility/polarity/chemotaxis; Cell development/differentiation; Membrane protein, integral
Chromosomal Location of Human Ortholog: 1q31.3
Cellular Component: microvillus; apical plasma membrane; extracellular region; integral to membrane
Molecular Function: protein binding; calcium ion binding
Biological Process: eye photoreceptor cell development; plasma membrane organization and biogenesis; cell-cell signaling; establishment and/or maintenance of cell polarity
Disease: Leber Congenital Amaurosis 8; Retinitis Pigmentosa 12; Pigmented Paravenous Chorioretinal Atrophy
Research Articles on CRB1
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Product Notes
The CRB1 crb1 (Catalog #AAA153460) is an Antibody produced from Rabbit and is intended for research purposes only. The product is available for immediate purchase. The CRB1 Antibody reacts with Human and may cross-react with other species as described in the data sheet. AAA Biotech's CRB1 can be used in a range of immunoassay formats including, but not limited to, ELISA (EIA), Western Blot (WB). CRB1 antibody can be used for detection of CRB1 by Western blot at 1 - 2 mug/mL. Researchers should empirically determine the suitability of the CRB1 crb1 for an application not listed in the data sheet. Researchers commonly develop new applications and it is an integral, important part of the investigative research process. It is sometimes possible for the material contained within the vial of "CRB1, Polyclonal Antibody" to become dispersed throughout the inside of the vial, particularly around the seal of said vial, during shipment and storage. We always suggest centrifuging these vials to consolidate all of the liquid away from the lid and to the bottom of the vial prior to opening. Please be advised that certain products may require dry ice for shipping and that, if this is the case, an additional dry ice fee may also be required.Precautions
All products in the AAA Biotech catalog are strictly for research-use only, and are absolutely not suitable for use in any sort of medical, therapeutic, prophylactic, in-vivo, or diagnostic capacity. By purchasing a product from AAA Biotech, you are explicitly certifying that said products will be properly tested and used in line with industry standard. AAA Biotech and its authorized distribution partners reserve the right to refuse to fulfill any order if we have any indication that a purchaser may be intending to use a product outside of our accepted criteria.Disclaimer
Though we do strive to guarantee the information represented in this datasheet, AAA Biotech cannot be held responsible for any oversights or imprecisions. AAA Biotech reserves the right to adjust any aspect of this datasheet at any time and without notice. It is the responsibility of the customer to inform AAA Biotech of any product performance issues observed or experienced within 30 days of receipt of said product. To see additional details on this or any of our other policies, please see our Terms & Conditions page.Item has been added to Shopping Cart
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