RPE65 Antibody (YA7182)
(Synonyms: LCA2; mRPE65; p63; rd12; Retinal pigment epithelium specific 61 kDa protein; RP20; RPE65; sRPE65)Based on 1 Customer Validation
RPE65 Antibody (YA7182) is a Rabbit-derived and non-conjugated IgG monoclonal antibody, targeting to RPE65.
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Host:
Rabbit
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Isotype:
IgG
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Application:
WB, IHC-P, IP, IF-Tissue
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Reactivity :
Human, Mouse, Rat
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Formulation:
Supplied in 1*TBS (pH7.4), 0.05% BSA, 40% Glycerol. Preservative: 0.05% Sodium Azide.
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Conjugation:
Non-conjugated
Applications
| Application |
WB
WB: Western Blot
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IP
IP: Immunoprecipitation
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IHC-P
IHC-P: Immunohistochemistry-Paraffin
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IF-Tissue
IF-Tissue: Immunofluorescence-Tissue
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|---|---|---|---|---|
| Dilution Ratio | 1:2000 | 1:10-1:50 | 1:1000 | 1:500 |
Product Details
RPE65 Antibody (YA7182) is a Rabbit-derived and non-conjugated IgG monoclonal antibody, targeting to RPE65.
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Host Rabbit
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Clonality Monoclonal,Recombinant
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Species ReactivityHuman, Mouse, Rat
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Observed Molecular WeightObserved band size: 61 kDaNote: Due to possible protein modifications or aggregation, the molecular weight should be confirmed by actual measurement, and the predicted value is for reference only.
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Calculated Molecular Weight Predicted band size: 61 kDa
SwissProt: Q16518 Human ; Q91ZQ5 Mouse ; O70276 Rat
Synthetic peptide within Human RPE65 aa 441-484 / 533.
affinity purified.
Non-conjugated
Unmodified
IgG
Product Properties
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Appearance
Solution
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Formulation
Supplied in 1*TBS (pH7.4), 0.05% BSA, 40% Glycerol. Preservative: 0.05% Sodium Azide.
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Concentration
Batch-dependent, Please check the COA for the concentration of each lot. Check Lot Concentration
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Storage & Stability
Stored at -20°C for 1 year. Avoid repeated freeze / thaw cycles.
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Shipping
Shipping with blue ice.
Background
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Function
RPE65 (Retinal pigment epithelium 65 kDa protein) is a critical enzyme in the visual cycle that is specifically expressed in retinal pigment epithelial (RPE) cells. As a retinoid isomerohydrolase, RPE65 catalyzes the conversion of all-trans-retinyl esters to 11-cis-retinol, a vital process for visual phototransduction [1].RPE65 facilitates retinoid isomerization through its iron-binding site, maintaining the continuous supply of 11-cis-retinal in the retina - the essential precursor for rhodopsin regeneration [2]. Deficiencies in RPE65 function disrupt the visual cycle and are associated with various inherited retinal disorders, including Leber congenital amaurosis type 2 (LCA2) and retinitis pigmentosa [3].
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Subcellular Localization
Lipid droplet; Endoplasmic reticulum; Nucleus
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Expression
Tissue_specificity:It is mainly expressed in adipose tissue, small intestine, heart, colon and stomach, with lower expression levels in brain, kidney and liver.
Positive sample: Mouse eyeball tissue lysate, Rat eyeball tissue lysate, rat eyeball tissue, mouse eyeball tissue. -
Isoforms & Post-Translational Modification
Q96AQ7 has 4 isomers: Q96AQ7-1: 26754 Da (predicted); Q96AQ7-2: 18439 Da (predicted); Q96AQ7-3: 14013 Da (predicted); Q96AQ7-4: 27923 Da (predicted).
Ubiquitinated and targeted to proteasomal degradation, resulting in a short half-life (about 15 minutes in 3T3-L1 cells). Protein stability depends on triaclyglycerol synthesis, fatty acid availability and lipid droplet formation -
Subunit
Homodimer (By similarity). Homooligomer; undergoes liquid-liquid phase separation (LLPS) via its N-terminus, facilitating lipid droplet fusion, occurs at the lipid droplet contact sites (By similarity). Interacts with CIDEA (PubMed:19843876). Interacts with PLIN1 (PubMed:23399566). Interacts with NFAT5; this interaction is direct and retains NFAT5 in the cytoplasm (By similarity). Interacts with CEBPB (By similarity). Interacts with isoform CLSTN3beta of CLSTN3; inhibiting the lipid transferase activity of CIDEC (By similarity)
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SwissProt ID
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Synonyms
LCA2; mRPE65; p63; rd12; Retinal pigment epithelium specific 61 kDa protein; RP20; RPE65; sRPE65
Documentation
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Data Sheet (261 KB)
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SDS (252 KB)
- English - EN (252 KB)
- Français - FR (252 KB)
- Deutsch - DE (252 KB)
- Norwegian - NO (252 KB)
- Español - ES (252 KB)
- Swedish - SV (252 KB)
- Italian - IT (252 KB)
- Korean - KR (252 KB)
- Portuguese - PT (252 KB)
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User Guide for Antibodies (1077 KB)
References
[1]. Gu SM, et al. Mutations in RPE65 cause autosomal recessive childhood-onset severe retinal dystrophy. Nat Genet. 1997 Oct;17(2):194-7. [Content Brief]
[2]. Moiseyev G, et al. RPE65 is the isomerohydrolase in the retinoid visual cycle. Proc Natl Acad Sci U S A. 2005 Aug 30;102(35):12413-8. [Content Brief]
[3]. Russell S, et al. Efficacy and safety of voretigene neparvovec (AAV2-hRPE65v2) in patients with RPE65-mediated inherited retinal dystrophy: a randomised, controlled, open-label, phase 3 trial. Lancet. 2017 Aug 26;390(10097):849-860. [Content Brief]