RPE65 Antibody BIOTIN-Conjugated | MyBioSource MBS544745 product information

product summary

company name :

MyBioSource

product type :

antibody

product name :

RPE65 Antibody BIOTIN-Conjugated

catalog :

MBS544745

quantity :

0.1 mg

price :

495 USD

clonality :

polyclonal

host :

rabbit

conjugate :

biotin

reactivity :

human, mouse, rat, cow

application :

western blot, ELISA, immunoprecipitation, enzyme immunoassay

more info or order :

MyBioSource product webpage

product information

catalog number :

MBS544745

products type :

Antibody

products full name :

RPE65 Antibody BIOTIN-Conjugated

products short name :

[RPE65]

products name syn :

[Rabbit Polyclonal RPE65 Antibody BIOTIN]

other names :

[RPE65; Retinoid isomerohydrolase; retinoid isomerohydrolase; retinal pigment epithelium 65; All-trans-retinyl-palmitate hydrolase; Retinal pigment epithelium-specific 65 kDa protein; Retinol isomerase]

products gene name :

[RPE65]

other gene names :

[Rpe65; Rpe65; LCA2; RP20; rd12; 65kDa; Mord1; A930029L06Rik]

uniprot entry name :

RPE65_MOUSE

clonality :

Polyclonal

host :

Rabbit

reactivity :

Bovine, Human, Mouse, Rat

sequence length :

533

form :

Biotin-Conjugated Immunoglobulins

concentration :

0.70 mg/ml IgG in antibody stabilization buffer

storage stability :

Store -20°C for long term storage

tested application :

ELISA (EIA), Immunoprecipitation (IP), Western Blot (WB)

app notes :

Western Blot: 1:500. IP: 1:200

other info1 :

Immunogen: Synthetic peptide corresponding to unique amino acid sequence on mouse RPE65 protein. Alternative Nomenclature: All-trans-retinyl-palmitate hydrolase antibody. LCA 2 antibody. Leber congenital amaurosis antibody. p63 antibody. rd 12 antibody. Retinal pigment epithelium specific 61 kDa protein antibody. Retinal pigment epithelium specific 65 kDa protein antibody. retinitis pigmentosa 20 antibody. Retinoid isomerohydrolase antibody. RP20 antibody

products description :

Biotin-Conjugated RE1-silencing transcription factor Antibody. 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.

ncbi gi num :

15488446

ncbi acc num :

AAL01119.1

uniprot acc num :

Q91ZQ5

ncbi mol weight :

61,085 Da

ncbi pathways :

Retinol Metabolism Pathway (198314); Retinol Metabolism Pathway (83218); Retinol Metabolism Pathway (406); Signal Transduction Pathway (1366876); The Canonical Retinoid Cycle In Rods (twilight Vision) Pathway (1367136); Visual Phototransduction Pathway (1367134)

uniprot summary :

RPE65: 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. Defects in RPE65 are a cause of autosomal dominant retinitis pigmentosa with choroidal involvement (PubMed:21654732). Affected individuals show reduction of central vision, constriction of visual fields, night blindness and chorioretinal atrophy. Belongs to the carotenoid oxygenase family. Protein type: EC 3.1.1.64; Cofactor and Vitamin Metabolism - retinol. Cellular Component: cytoplasm; endoplasmic reticulum; intracellular membrane-bound organelle; membrane; plasma membrane. Molecular Function: hydrolase activity; isomerase activity; metal ion binding; oxidoreductase activity, acting on single donors with incorporation of molecular oxygen, incorporation of two atoms of oxygen; retinal isomerase activity. Biological Process: detection of light stimulus involved in visual perception; insulin receptor signaling pathway; regulation of rhodopsin gene expression; response to stimulus; retina development in camera-type eye; retina morphogenesis in camera-type eye; retinal homeostasis; retinal metabolic process; retinol metabolic process; visual perception

size1 :

0.1 mg

price1 :

495 USD

more info or order :

MyBioSource product webpage