Anti-RPE65 Rabbit pAb
- 100ug (NP22580) Please inquiry
Antibody type:Primary antibody
Label:Unconjugated
Modification:Unmodification
Isotype:Rabbit IgG
Host:Rabbit
Application:WB
Purify method:Affinity purified
Species:Human,Mouse,Rat
Gene Name:RPE65
Synonyms:p63; BCO3; LCA2; RP20; rd12; mRPE65; sRPE65
Gene Synonyms:
Gene Full Name:RPE65, retinoid isomerohydrolase
Gene Infomation:The protein encoded by this gene is a component of the vitamin A visual cycle of the retina which supplies the 11-cis retinal chromophore of the photoreceptors opsin visual pigments. It is a member of the carotenoid cleavage oxygenase superfamily. All members of this superfamily are non-heme iron oxygenases with a seven-bladed propeller fold and oxidatively cleave carotenoid carbon:carbon double bonds. However, the protein encoded by this gene has acquired a divergent function that involves the concerted O-alkyl ester cleavage of its all-trans retinyl ester substrate and all-trans to 11-cis double bond isomerization of the retinyl moiety. As such, it performs the essential enzymatic isomerization step in the synthesis of 11-cis retinal. Mutations in this gene are associated with early-onset severe blinding disorders such as Leber congenital. [provided by RefSeq, Oct 2017]
Antigen:Recombinant fusion protein containing a sequence corresponding to amino acids 274-533 of human RPE65 (NP_000320.1).
Antigen Synonyms:Retinoid isomerohydrolase
Clonality:Polyclonal antibody
Source:Human
Reaction:
Form:Liquid
Tested Applications:
- Western blot (1:100 to 1:500)
- Immunofluorescence (1:50 to 1:400)
- Immunohistochemistry (1:200 to 1:500)
- Flow cytometry analysis (1:200 to 1:500)
- Enzyme-linked Immunosorbent Assay (1:100-1:5000)
Clone:
Dilution:WB1:500 -1:2000(Optimal dilutions should be determined by the end user)
Mole Mass:61kDa
Location:Cell membrane, Cytoplasm, Endoplasmic reticulum, Membrane, Microsome
Concentration:
Sequence Similarity:
Gene Id:
SwissProt ID:Q16518
Unigene:6121
Nucleotide Accession:
Tissue specificity:Retina (at protein level). Retinal pigment epithelium specific.
Storage:Store at -20°C. Avoid freeze / thaw cycles.
Buffer condition:PBS with 0.02% sodium azide, 50% glycerol, pH7.3.
Background:
Molar Function:Critical isomerohydrolase in the retinoid cycle involved in regeneration of 11-cis-retinal, the chromophore of rod and cone opsins. Catalyzes the cleavage and isomerization of all-trans-retinyl fatty acid esters to 11-cis-retinol which is further oxidized by 11-cis retinol dehydrogenase to 11-cis-retinal for use as visual chromophore (PubMed:16116091). Essential for the production of 11-cis retinal for both rod and cone photoreceptors (PubMed:17848510). Also capable of catalyzing the isomerization of lutein to meso-zeaxanthin an eye-specific carotenoid (PubMed:28874556). 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 .
Western Blot:Stability: The thermal stability is described by the loss rate. The loss rate was determined by accelerated thermal degradation test, that is, incubate the protein at 37°C for 48h, and no obvious degradation and precipitation were observed. The loss rate is less than 5% within the expiration date under appropriate storage condition.
Storage: Store at 4°C for frequent use. Stored at -20°C in a manual defrost freezer for one year without detectable loss of activity. Avoid repeated freeze-thaw cycles.
Notes: For In vitro laboratory use only. Not for any clinical, therapeutic, or diagnostic use in humans or animals. Not for animal or human consumption.