UniProt Accession

 RPE65_HUMAN; Q16518; Q5T9U3 

Genbank Protein ID

 U18991; U20510; U20476; U20477; U20478; U20479; U20481; U20482; U20484; U20485; U20486; AF039868; AF039855; AF039856; AF039857; AF039858; AF039859; AF039860; AF039861; AF039862; AF039863; AF039864; AF039865; AF039866; AF039867; AL139413; CH471059; BC075035; BC075036 

Genbank Nucleotide ID

 AAA99012.1; AAC14586.1; AAC14586.1; AAC14586.1; AAC14586.1; AAC14586.1; AAC14586.1; AAC14586.1; AAC14586.1; AAC14586.1; AAC14586.1; AAC39660.1; AAC39660.1; AAC39660.1; AAC39660.1; AAC39660.1; AAC39660.1; AAC39660.1; AAC39660.1; AAC39660.1; AAC39660.1; AAC39660.1; AAC39660.1; AAC39660.1; AAC39660.1; CAI18957.1; EAX06478.1; AAH75035.1; AAH75036.1 

Protein Name

 Retinoid isomerohydrolase 

Protein Synonyms/Alias

 All-trans-retinyl-palmitate hydrolase; Retinal pigment epithelium-specific 65 kDa protein; Retinol isomerase 

Gene Name

 RPE65 

Gene Synonyms/Alias

  

Created Date

 July 27, 2013 

Organism

 Homo sapiens (Human) 

NCBI Taxa ID

 9606 

Lysine Modification

Position	Peptide	Type	References
376	VLPLNIDKADTGKNL	acetylation	[1]

Reference

 [1] Regulation of cellular metabolism by protein lysine acetylation.
 Zhao S, Xu W, Jiang W, Yu W, Lin Y, Zhang T, Yao J, Zhou L, Zeng Y, Li H, Li Y, Shi J, An W, Hancock SM, He F, Qin L, Chin J, Yang P, Chen X, Lei Q, Xiong Y, Guan KL.
 Science. 2010 Feb 19;327(5968):1000-4. [PMID: 20167786] 

Functional Description

 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. 

Sequence Annotation

 METAL 180 180 Iron; catalytic (By similarity).
 METAL 241 241 Iron; catalytic (By similarity).
 METAL 313 313 Iron; catalytic (By similarity).
 METAL 527 527 Iron; catalytic (By similarity).
 MOD_RES 2 2 N-acetylserine (By similarity).
 MOD_RES 101 101 Phosphothreonine.
 MOD_RES 105 105 Phosphothreonine.
 MOD_RES 113 113 N6-acetyllysine.
 MOD_RES 117 117 Phosphoserine.
 LIPID 112 112 S-palmitoyl cysteine; in membrane form.
 LIPID 231 231 S-palmitoyl cysteine; in membrane form
 LIPID 329 329 S-palmitoyl cysteine; in membrane form
 LIPID 330 330 S-palmitoyl cysteine; in membrane form  

Keyword

 Acetylation; Cell membrane; Complete proteome; Cytoplasm; Direct protein sequencing; Disease mutation; Endoplasmic reticulum; Hydrolase; Iron; Isomerase; Leber congenital amaurosis; Lipoprotein; Membrane; Metal-binding; Microsome; Palmitate; Phosphoprotein; Polymorphism; Reference proteome; Retinitis pigmentosa; Sensory transduction; Vision. 

Sequence Source

 UniProt (SWISSPROT/TrEMBL); GenBank; EMBL 

Protein Length

 533 AA 

Protein Sequence

Gene Ontology

 GO:0005783; C:endoplasmic reticulum; IEA:UniProtKB-KW.
 GO:0031090; C:organelle membrane; IEA:UniProtKB-SubCell.
 GO:0005886; C:plasma membrane; TAS:Reactome.
 GO:0052885; F:all-trans-retinyl-ester hydrolase, 11-cis retinol forming activity; IEA:EC.
 GO:0052884; F:all-trans-retinyl-palmitate hydrolase, 11-cis retinol forming activity; IEA:EC.
 GO:0046872; F:metal ion binding; IEA:UniProtKB-KW.
 GO:0004744; F:retinal isomerase activity; IEA:Compara.
 GO:0071257; P:cellular response to electrical stimulus; IEA:Compara.
 GO:0008286; P:insulin receptor signaling pathway; IEA:Compara.
 GO:0007603; P:phototransduction, visible light; TAS:Reactome.
 GO:0007468; P:regulation of rhodopsin gene expression; IEA:Compara.
 GO:0060042; P:retina morphogenesis in camera-type eye; IEA:Compara.
 GO:0042574; P:retinal metabolic process; IEA:Compara.
 GO:0007601; P:visual perception; TAS:ProtInc.
 GO:0006776; P:vitamin A metabolic process; TAS:ProtInc. 

Interpro

 IPR004294; Carotenoid_Oase. 

Pfam

 PF03055; RPE65 

SMART

  

PROSITE

  

PRINTS