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Mouse Anti-RPE65 Recombinant Antibody (401.8B11.3D9) (CBMAB-0776CQ)


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Summary

Host Animal
Mouse
Specificity
Cattle, Mouse, Rat, Chicken, Human, Frog, Monkey
Clone
401.8B11.3D9
Antibody Isotype
IgG1
Application
ELISA, FC, IF, IHC-Fr, IP, WB

Basic Information

Immunogen
Bovine RPE microsomal membranes
Specificity
Cattle, Mouse, Rat, Chicken, Human, Frog, Monkey
Antibody Isotype
IgG1
Clonality
Monoclonal
Application Notes
The COA includes recommended starting dilutions, optimal dilutions should be determined by the end user.

Formulations & Storage [For reference only, actual COA shall prevail!]

Purity
>95% as determined by analysis by SDS-PAGE
Storage
Store at +4°C short term (1-2 weeks). Aliquot and store at -20°C long term. Avoid repeated freeze/thaw cycles.

Target

Full Name
RPE65, Retinoid Isomerohydrolase
Introduction
This gene encodes a protein which is located in the retinal pigment epithelium and is involved in the production of 11-cis retinal and in visual pigment regeneration. 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.
Entrez Gene ID
Human6121
Mouse19892
Rat89826
Chicken395700
Frog100145692
Monkey701764
Cattle282043
UniProt ID
HumanQ16518
MouseQ91ZQ5
RatO70276
ChickenQ9YGX2
FrogB1WAQ0
MonkeyF7HIH7
CattleQ28175
Alternative Names
BCO3; LCA2; RP20; rd12; mRPE65; sRPE65
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 (By similarity).
Biological Process
Biological Process cellular response to electrical stimulusIEA:Ensembl
Biological Process circadian rhythmIEA:Ensembl
Biological Process detection of light stimulus involved in visual perceptionManual Assertion Based On ExperimentIMP:UniProtKB
Biological Process insulin receptor signaling pathwayIEA:Ensembl
Biological Process neural retina developmentIEA:Ensembl
Biological Process regulation of gene expressionIEA:Ensembl
Biological Process retina homeostasisManual Assertion Based On ExperimentIMP:UniProtKB
Biological Process retina morphogenesis in camera-type eyeIEA:Ensembl
Biological Process retinal metabolic processIEA:Ensembl
Biological Process retinoid metabolic processManual Assertion Based On ExperimentIDA:UniProtKB
Biological Process visual perceptionManual Assertion Based On ExperimentTAS:ProtInc
Biological Process vitamin A metabolic processManual Assertion Based On ExperimentTAS:ProtInc
Biological Process zeaxanthin biosynthetic processManual Assertion Based On ExperimentIDA:UniProtKB
Cellular Location
Cytoplasm
Cell membrane
Microsome membrane
Attached to the membrane by a lipid anchor when palmitoylated (membrane form), soluble when unpalmitoylated. Undergoes light-dependent intracellular transport to become more concentrated in the central region of the retina pigment epithelium cells.
Involvement in disease
Leber congenital amaurosis 2 (LCA2):
A severe dystrophy of the retina, typically becoming evident in the first years of life. Visual function is usually poor and often accompanied by nystagmus, sluggish or near-absent pupillary responses, photophobia, high hyperopia and keratoconus.
Retinitis pigmentosa 20 (RP20):
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.
Retinitis pigmentosa 87 with choroidal involvement (RP87):
A form of retinitis pigmentosa, 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. RP87 is an autosomal dominant form characterized by a slowly progressive visual disturbance accompanied by extensive choroid/retinal atrophy that mimics certain aspects of choroideremia. Disease severity and age of onset are variable, and some carriers are unaffected.
PTM
Palmitoylation by LRAT regulates ligand binding specificity; the palmitoylated form (membrane form) specifically binds all-trans-retinyl-palmitate, while the soluble unpalmitoylated form binds all-trans-retinol (vitamin A) (By similarity).
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For research use only. Not intended for any clinical use.

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