Anti-RPE65 Antibody, clone KPSA1,100UG,MABN2438-100UG,Sigma

销售价: ¥ 3346.40 / 件
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订货号 0BM1428
品牌型号 Sigma MABN2438-100UG
货期 询货期
最小订货量 1件
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产品介绍 Product Description

General description

Retinoid isomerohydrolase (UniProt: Q16518; also known as EC:3.1.1.64, All-trans-retinyl-palmitate hydrolase, Lutein isomerase, Meso-zeaxanthin isomerase, Retinal pigment epithelium-specific 65 kDa protein, Retinol isomerase) is encoded by the RPE65 gene (Gene ID: 6121) in human. Retinol isomerase is a lipid-anchor protein that is attached to the membrane when palmitoylated and is present in soluble form when unpalmitoylated. It is shown to be critical for retinoid cycle and is involved in regeneration of 11-cis-retinal, the chromophore of rod and cone opsins. It catalyzes the cleavage and isomerization of all-trans-retinyl fatty acid esters to 11-cis-retinol that is further oxidized by 11-cis retinol dehydrogenase to 11-cis-retinal for use as visual chromophore. It can also catalyze the isomerization of lutein to meso-zeaxanthin an eye-specific carotenoid. The soluble form is reported to binds vitamin A (all-trans-retinol), making it available for lecithin retinol acyltransferase (LRAT) processing to all-trans-retinyl ester. The membrane form, palmitoylated by LRAT, binds all-trans-retinyl esters, making them available for isomerohydrolase processing to all-cis-retinol. The soluble form can be regenerated by transferring its palmitoyl groups onto 11-cis-retinol, a reaction catalyzed by LRAT. It undergoes light-dependent intracellular transport to become more concentrated in the central region of the retina pigment epithelium cells. Mutations in RPE65 gene are known to cause Retinitis pigmentosa that is characterized by retinal pigment deposits visible on fundus examination and primary loss of rod photoreceptor cells followed by secondary loss of cone photoreceptors. (Ref.: Golczak, M., et al. (2010). J. Biol. Chem. 285(13); 9667-9682; Moiseyev, G., et al. (2005). Proc. Natl. Acad. Sci. USA. 102(35):12413-12418).


Immunogen

Recombinant full-length Human RPE65 expressed in Sf9 cells.


Application

Quality Control Testing

Evaluated by Western Blotting in Bovine retina microsomal preparation.

Western Blotting Analysis: A 1:10,000 dilution of this antibody detected RPE65 in Bovine retina microsomal preparation.

Tested Applications

Western Blotting Analysis: A representative lot detected RPE65 in Western Blotting applications (Golczak, M., et al. (2010). J Biol Chem. 285(13):9667-9682; Banskota, S., et al. (2022). Cell. 185(2):250-265.e16).

Immunoaffinity Purification: A representative lot was used for purification of crossed-linked RPE65.(Golczak, M., et al. (2010). J Biol Chem. 285(13):9667-9682).

Immunohistochemistry Applications: A representative lot detected RPE65 in Immunohistochemistry applications (Amengual, J., et al. (2014). Hum Mol Genet. 23(20):5402-17).

ELISA Analysis: A representative lot detected RPE65 in ELISA applications (Golczak, M., et al. (2010). J Biol Chem. 285(13):9667-9682).

Note: Actual optimal working dilutions must be determined by end user as specimens, and experimental conditions may vary with the end user.


Biochem/physiol Actions

Clone KPSA1 is a mouse monoclonal antibody that detects RPE65. It targets an epitope within 23 amino acid from the C-terminal region.


Physical form

Purified mouse monoclonal antibody IgG1 in buffer containing 0.1 M Tris-Glycine (pH 7.4), 150 mM NaCl and 0.05% sodium azide.


Preparation Note

1.0 mg/mL. Please refer to guidance on suggested starting dilutions and/or titers per application and sample type.
Recommended storage: +2°C to +8°C.


Other Notes

Concentration: Please refer to the Certificate of Analysis for the lot-specific concentration.


Disclaimer

Unless otherwise stated in our catalog or other company documentation accompanying the product(s), our products are intended for research use only and are not to be used for any other purpose, which includes but is not limited to, unauthorized commercial uses, in vitro diagnostic uses, ex vivo or in vivo therapeutic uses or any type of consumption or application to humans or animals.


技术参数 Specifications
biological sourcemouse
Quality Level200
conjugateunconjugated
antibody formpurified antibody
antibody product typeprimary antibodies
cloneKPSA1, monoclonal
species reactivitybovine, human, mouse
packagingantibody small pack of 100
technique(s)direct ELISA: suitable,immunohistochemistry (formalin-fixed, paraffin-embedded sections): suitable,western blot: suitable
isotypeIgG1κ
epitope sequenceC-terminal
Protein ID accession no.NP_000320
UniProt accession no.Q16518
target post-translational modificationunmodified
Gene Informationhuman ... RPE65(6121)
长度(mm)
宽度(mm)
高度(mm)
重量(kg)
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