CRB1 sirna

CRB1 siRNA (Human)

Cat. Num. AAA8202608

• Gene Names: CRB1; LCA8; RP12
• Reactivity: Human
• Applications: RNA Interference (RNAi)
• Purity: > 97%
• Synonyms: CRB1; CRB1 siRNA (Human); Protein crumbs homolog 1; CRB1 sirna

• Host: Synthetic
• Reactivity: Human
• Specificity: CRB1 siRNA (Human) is a target-specific 19-23 nt siRNA oligo duplexes designed to knock down gene expression.
• Purity/Purification: > 97%
• Form/Format: Lyophilized powder
• Sequence Length: 1294

• Applicable Applications for CRB1 sirna: RNA Interference (RNAi)
• Quality Control: Oligonucleotide synthesis is monitored base by base through trityl analysis to ensure appropriate coupling efficiency. The oligo is subsequently purified by affinity-solid phase extraction. The annealed RNA duplex is further analyzed by mass spectrometry to verify the exact composition of the duplex. Each lot is compared to the previous lot by mass spectrometry to ensure maximum lot-to-lot consistency.
• Directions for Use: We recommends transfection with 100 nM siRNA 48 to 72 hours prior to cell lysis. Before resuspending, briefly centrifuge the tube to ensure the lyophilized siRNA is at the bottom of the tube. Resuspend the siRNA oligos to an appropriate concentration with DEPC water. For each vial, suitable for 250 transfections in 24 well plate (20 pmol for each well).
• Components: We offer pre-designed sets of 3 different target-specific siRNA oligo duplexes of human CRB1 gene. Each vial contains 5 nmol of lyophilized siRNA. The duplexes can be transfected individually or pooled together to achieve knockdown of the target gene, which is most commonly assessed by qPCR or western blot. Our siRNA oligos are also chemically modified (2'-OMe) at no extra charge for increased stability and enhanced knockdown in vitro and in vivo.
• Preparation and Storage: Shipped at 4 degree C. Store at -20 degree C for one year.

Related Product Information for CRB1 sirna

siRNA to inhibit CRB1 expression using RNA interference

NCBI and Uniprot Product Information

• NCBI GI #: 302370926
• NCBI GeneID: 23418
• NCBI Accession #: NP_001180569.1
• NCBI GenBank Nucleotide #: NM_001193640.1
• UniProt Accession #: P82279; Q5K3A6; Q5TC28; Q5VUT1; Q6N027; Q8WWY0; Q8WWY1; A2A308; B7Z5T2; B9EG71
• Molecular Weight: 95,046 Da
• NCBI Official Full Name: protein crumbs homolog 1 isoform 2
• NCBI Official Synonym Full Names: crumbs family member 1, photoreceptor morphogenesis associated
• NCBI Official Symbol: CRB1
• NCBI Official Synonym Symbols: LCA8; RP12
• NCBI Protein Information: protein crumbs homolog 1
• UniProt Protein Name: Protein crumbs homolog 1
• Protein Family: Protein crumbs
• UniProt Gene Name: CRB1
• UniProt Entry Name: CRUM1_HUMAN

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: Membrane protein, integral; Cell development/differentiation; Motility/polarity/chemotaxis

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: Retinitis Pigmentosa 12; Leber Congenital Amaurosis 8; Pigmented Paravenous Chorioretinal Atrophy