Rb2246-271213-WS

A synthetic peptide from aa region 490-533 of mouse RPE65 conjugated to blue carrier protein was used as the antigen. The peptide is homologous in rat and shares 88% identity with human sequence.

RPE65_MOUSE
RPE65_RAT
RPE65_HUMAN

All-trans-retinyl-palmitate hydrolase, Retinal pigment epithelium specific 65 kDa protein, Retinol isomerase, RPE65

FUNCTION: Plays important roles in the production of 11-cis retinal and in visual pigment regeneration. The soluble form binds vitamin A (all-trans-retinol), making it available for LRAT processing to all-trans-retinyl ester. The membrane form, palmitoylated by LRAT, binds all-trans-retinyl esters, making them available for IMH (isomerohydrolase) processing to all-cis-retinol. The soluble form is regenerated by transferring its palmitoyl groups onto 11-cis-retinol, a reaction catalyzed by LRAT. The enzymatic activity is linearly dependent of the expression levels and membrane association.
Defects in RPE65 are the cause of Leber congenital amaurosis type 2 (LCA2). 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 RPE65 are the cause of retinitis pigmentosa type 20 (RP20). RP leads to degeneration of retinal photoreceptor cells. 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. RP20 inheritance is autosomal dominant.
Subcellular location: Cytoplasm. Cell membrane; Lipid-anchor. Note: Attached to the membrane by a lipid anchor when palmitoylated (membrane form), soluble when unpalmitoylated
TISSUE SPECIFICITY: Retinal pigment epithelium specific.

Maintain the lyophilised/reconstituted antibodies frozen at -20C for long term storage and refrigerated at 2-8C for a shorter term. When reconstituting, glycerol (1:1) may be added for an additional stability. Avoid freeze and thaw cycles.

12 months after reconstitution

This item will be shipped to you at ambient temperature in a lyophilised form.

1. Boulanger A, et al. Mol. Vis. 7:283-287(2001)

For research use only

100 ul

Unconjugated antibody

NZ white rabbit

Whole serum

Polyclonal

Polyclonal, whole serum

IHC, WB (confirmed by recombinant protein). A dilution of 1 : 300 to 1 : 2000 is recommended. The optimal dilution should be determined by the end user. Not yet tested in other applications.

Specific for RPE65.

Mouse, rat. Other species not yet tested but likely to work in human.

Lyophilised

Reconstitute in 100 ul of sterile water. Centrifuge to remove any insoluble material.

Control peptide is available at $120 per 50 μg. Please enquire sales@osenses.com

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