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Editor's Notes

from literature on

Interphotoreceptor Retinoid-binding Protein (IRBP, RBP3)

Editor's Notes

Recent update from: 02.01.2000


RBP3, IRBP Notes

Species:
See also:
Chromosomal localisation in man:

in mouse:
Gene Data

Exons:
Primers:
Protein Data
Aminoacids:
Molecularweight on SDS-PAGE: 146 kDa

calculated:
Reference: (21)


RBP3, IRBP Notes

  • in space between photoreceptorouter segments, on the apical surface of RPE
Species:
See also:
Chromosomal localisation in man:

in mouse:
Gene Data

Exons:
Primers:
Protein Data
Aminoacids:
Molecularweight on SDS-PAGE:

calculated:
Reference: (6)


RBP3, IRBP Notes

  • negates involvement in RP
  • 526 patients, ADRP, ARRP, RP simplex
Species:
See also:
Chromosomal localisation in man:

in mouse:
Gene Data

Exons:
Primers:
Protein Data
Aminoacids:
Molecularweight on SDS-PAGE:

calculated:
Reference: (24)


RBP3, IRBP Notes

  • Arg267Gln, Val282Met, Ser291Arg, Asn785Lys, Arg833Cys in RAl Degenerations but no coseggregation
  • 9 silent changes
Species:
See also:
Chromosomal localisation in man:

in mouse:
Gene Data

Exons:
Primers:
Protein Data
Aminoacids:
Molecularweight on SDS-PAGE:

calculated:
Reference: (17)


RBP3, IRBP Notes

Species:
See also:
Chromosomal localisation in man:

in mouse:
Gene Data

Exons:
Primers:
Protein Data
Aminoacids:
Molecularweight on SDS-PAGE:

calculated:
Reference: (7)


RBP3, IRBP Notes

  • Only protein to remove efficient RAl from RPE membranes
  • IRBP lipid transfer is not RPE membrane specific
  • 2 Rol binding sites
Species:
See also:
Chromosomal localisation in man:

in mouse:
Gene Data

Exons:
Primers:
Protein Data
Aminoacids:
Molecularweight on SDS-PAGE:

calculated:
Reference: (1)


RBP3, IRBP Notes

  • Promoter: -156 -> +19
  • ATTAA -42 -> -76
  • retinoic acid responsive element -85 -> -105 AP2 sites
Species:
See also:
Chromosomal localisation in man:

in mouse:
Gene Data

Exons:
Primers:
Protein Data
Aminoacids:
Molecularweight on SDS-PAGE:

calculated:
Reference: (11)


RBP3, IRBP Notes

  • inhibitory cis element -1173 -> -350
  • enhancing cis element -350 -> -204
Species:
See also:
Chromosomal localisation in man:

in mouse:
Gene Data

Exons:
Primers:
Protein Data
Aminoacids:
Molecularweight on SDS-PAGE:

calculated:
Reference: (4)


RBP3, IRBP Notes

Species:
See also:
Chromosomal localisation in man:

in mouse:
Gene Data

Exons:
Primers:
Protein Data
Aminoacids:
Molecularweight on SDS-PAGE: 133 kDa

calculated:
Reference: (10)


RBP3, IRBP Notes

  • Shuttles retinoids between the retinal pigment epithelium and ROS
  • Binds cis- and trans isomers of Rol and Ral, RA, vitamin E, long-chain fatty acids
  • Delivers 11-cis-RAl
  • 2 binding sites
  • 70% of bound fatty acids = palmitic, oleic, and stearic acid
  • DHA showed only slight effect on atRol binding at site 1 and less efefct on 11-cis Ral binding
  • 11-cis Ral dissociation is profoundly affected by DHA
Species:
See also:
Chromosomal localisation in man:

in mouse:
Gene Data

Exons:
Primers:
Protein Data
Aminoacids:
Molecularweight on SDS-PAGE: 140 kDa

calculated:
Reference: (28)


RBP3, IRBP Notes

  • R1005Q doubles the amount of ligand necessary to attain saturation by increasing the number of binding sites
  • Kd was not changed
  • R1041G, R1073G, and R1122G have no effect on Kd and binding sites
  • Mutations do not change the ligand specificity
Species:
See also:
Chromosomal localisation in man:

in mouse:
Gene Data

Exons:
Primers:
Protein Data
Aminoacids:
Molecularweight on SDS-PAGE:

calculated:
Reference: (2)
Online Reference

RBP3, IRBP Notes

Species: Bos taurus
See also:
Chromosomal localisation in man:

in mouse:
Gene Data

Exons:
Primers:
Protein Data
Aminoacids:
Molecularweight on SDS-PAGE:

calculated:
Reference: (3)


RBP3, IRBP Notes

  • Association of IRBP and metalloproteinases (gelatinase and caseinase)
  • Possibly contamination?
Species: Bos taurus
See also:
Chromosomal localisation in man:

in mouse:
Gene Data

Exons:
Primers:
Protein Data
Aminoacids:
Molecularweight on SDS-PAGE:

calculated:
Reference: (22)


RBP3, IRBP Notes

Species: Bos taurus See also:
Chromosomal localisation in man:

in mouse:
Gene Data
Exons:
Primers:
Protein Data Aminoacids:
Molecularweight on SDS-PAGE:

calculated:
Reference: (23)

RBP3, IRBP Notes

  • Half the size as mammalian RHO
  • cDNA of 6.2 kb
Species: Danio rerio
See also:
Chromosomal localisation in man:

in mouse:
Gene Data

Exons:
Primers:
Protein Data
Aminoacids:
Molecularweight on SDS-PAGE:

calculated:
Reference: (27)


RBP3, IRBP Notes

  • mRNA detected 24 hpf in pineal and 50 hpf in ventral retina
  • Cones express IRBP before the opsin, rods express rhodopsin before IRBP
  • Ventronasal quadrant is the first to express IRBP
Species: Danio rerio
See also:
Chromosomal localisation in man:

in mouse:
Gene Data

Exons:
Primers:
Protein Data
Aminoacids:
Molecularweight on SDS-PAGE:

calculated:
Reference: (26)
Online Reference

RBP3, IRBP Notes

Species: Gallus gallus
See also:
Chromosomal localisation in man:

in mouse:
Gene Data
mRNA: 1.3 kb
Exons:
Primers:
Protein Data
Aminoacids:
Molecularweight on SDS-PAGE: 140 kDa

calculated:
Reference: (19)


RBP3, IRBP Notes

  • Each repeat posses the ability to bind 9cRAl, atRol, atRA, DHA, a- and b-ionine, and trans-parinaic acid
  • Each repeat retains all functions of the whole protein
  • Repeats 1 and 2 correspond to a deep site, repeats 3 and 4 correspond to a shallow binding site
  • Binding occurs specific with all repeats and does not cooperativity having its own saturability and affinity
Species: Homo sapiens
See also:
Chromosomal localisation in man:

in mouse:
Gene Data

Exons:
Primers:
Protein Data
Aminoacids:
Molecularweight on SDS-PAGE:

calculated:
Reference: (18)


RBP3, IRBP Notes

Species: Homo sapiens
See also:
Chromosomal localisation in man:

in mouse:
Gene Data

Exons: 4
Primers:
Protein Data
Aminoacids:
Molecularweight on SDS-PAGE:

calculated:
Reference: (16)


RBP3, IRBP Notes

  • Binds hydrophobic ligands in the retina
  • 4 repeats of 300 AA
  • Each repeat posses the capability to bind Rol and 16-anthyloxy-palmitic acid
  • Binds and transports 11cRAl from stores in the RPE to the photoreceptor
  • Glycosylated in insect expression systems
  • Fatty acid analogs bind more tigthly to IRBP than retinoids
Species: Homo sapiens
See also:
Chromosomal localisation in man:

in mouse:
Gene Data

Exons: 4
Primers:
Protein Data
Aminoacids:
Molecularweight on SDS-PAGE:

calculated:
Reference: (13)


RBP3, IRBP Notes

  • 4 homologue segments, 300 AA each
  • Transports RAl and Rol from and to regeneration
  • Binds 11cRol, retinoic acid, cholesterol, fatty acids and tocopherol
  • single subunit
  • BglII und StyI RFLP
Species: Homo sapiens
See also:
Chromosomal localisation in man: 10p11.2-q11.2

in mouse:
Gene Data
cDNA:
Exons:
Primers:
Protein Data
Aminoacids: 1230 AA
Molecularweight on SDS-PAGE: 133 kDa

calculated:
Reference: (15)


RBP3, IRBP Notes

  • IRBP receptor on cell surface (70 kDa)
Species: Monkey
See also:
Chromosomal localisation in man:

in mouse:
Gene Data

Exons:
Primers:
Protein Data
Aminoacids:
Molecularweight on SDS-PAGE:

calculated:
Reference: (9)


RBP3, IRBP Notes

Species: Mus musculus
See also:
Chromosomal localisation in man:

in mouse: 15
Gene Data

Exons:
Primers:
Protein Data
Aminoacids:
Molecularweight on SDS-PAGE:

calculated:
Reference: (8)


RBP3, IRBP Notes

  • IRBP promoter studies
  • DNAseI protection between -69 - -4, identical in bovine and mouse
Species: Mus musculus
See also:
Chromosomal localisation in man:

in mouse:
Gene Data

Exons:
Primers:
Protein Data
Aminoacids:
Molecularweight on SDS-PAGE:

calculated:
Reference: (14)


RBP3, IRBP Notes

  • Methylated promotor in mouse and cow at MspI site
Species: Mus musculus
See also:
Chromosomal localisation in man:

in mouse:
Gene Data

Exons:
Primers:
Protein Data
Aminoacids:
Molecularweight on SDS-PAGE:

calculated:
Reference: (5)


RBP3, IRBP Notes

  • IRBP promoter studies
  • Heterogenous activity throughout the retinae
  • Confined to photoreceptor cells
  • Activity in rod and cones
  • mRNA in photoreceptor and pineal gland
  • 1,3 kb 5 fragment sufficient to direct tissue specific expression to photoreceptor cells
  • IRBP promotor is photoreceptorspecific
  • Expression starts at embryonic day 17
Species: Mus musculus
See also:
Chromosomal localisation in man:

in mouse:
Gene Data

Exons:
Primers:
Protein Data
Aminoacids:
Molecularweight on SDS-PAGE:

calculated:
Reference: (29)


RBP3, IRBP Notes

  • IRBP promoter studies
  • Promotor segment -156 - +19 confers tissue specificity
  • Equals human -123 - +18 region
  • Only 70 bp 5' transcription start are required for promotor activity
  • -70 - -45 two photoreceptor specific elements RET-1/PCE1 and its reversed repeat(GATTAA-box)
  • GATTAA-box binds CRX-element
  • beyond -70 is an element that inhibits expression in neurons
Species: Mus musculus
See also:
Chromosomal localisation in man:

in mouse:
Gene Data

Exons:
Primers:
Protein Data
Aminoacids:
Molecularweight on SDS-PAGE:

calculated:
Reference: (29)


RBP3, IRBP Notes

  • Borst-box 90% homologue in man and bovine
Species: Mus musculus
See also:
Chromosomal localisation in man:

in mouse:
Gene Data

Exons:
Primers:
Protein Data
Aminoacids:
Molecularweight on SDS-PAGE:

calculated:
Reference: (18)
Online Reference

RBP3, IRBP Notes

  • in rd mice IRBP is mainly intracellular
  • IRBP is secreted by the RPE
  • Do endogenous factors regulate secretion?
  • No abnormal production in rd mice
Species: Mus musculus rd
See also:
Chromosomal localisation in man:

in mouse:
Gene Data

Exons:
Primers:
Protein Data
Aminoacids:
Molecularweight on SDS-PAGE: 140 kDa

calculated:
Reference: (25)


RBP3, IRBP Notes

  • IRBP is internalized by RPE via endocytosis and phagocytosis
  • Phagocytosis inhibition at constant light inhibited IRBP specific activity
  • Endocytosis plays an important role in removal of IRBP from IPM
Species: Xenopus laevis
See also:
Chromosomal localisation in man:

in mouse:
Gene Data

Exons:
Primers:
Protein Data
Aminoacids:
Molecularweight on SDS-PAGE:

calculated:
Reference: (20)


RBP3, IRBP Notes

  • Synthezsized by the neural retina and secreted into IPM by rods and cones
  • Facilitates the exchange of 11cRAl and atRAl
  • Major soluble component of IPM
  • May have arisen from quadruplication of a single gene
  • Identity to human 70%
Species: Xenopus laevis
See also:
Chromosomal localisation in man:

in mouse:
Gene Data
mRNA: 4.2 kb
Exons:
Primers:
Protein Data
Aminoacids: 293 AA
Molecularweight on SDS-PAGE: 124 kDa

calculated:
Reference: (12)


References:

1. Adler, A.J. and Edwards, R.B. IRBP Uniquely Removes 11 Retinal from Isolated RPE Membranes. 1995; Invest.Ophthalmol.Vis.Sci. 36: S6
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2. Baer, C.A., van Niel, E.E., Cronk, J.W., Kinter, M.T., Sherman, N.E., Braiman, M.S., and Gonzalez-Fernandez, F. Arginine to glutamine substitutions in the fourth module of Xenopus interphotoreceptor retinoid-binding protein. 1998; Mol.Vis. 4: 30
Goto Top Link to PudMed
Human Mutation Online

3. Barrett, D.J., Redmond, T.M., Wiggert, B., Oprian, D.D., Chader, G.J., and Nickerson, J.M. cDNA clones encoding bovine interphotoreceptor retinoid binding protein. 1985; Biochem.Biophys.Res.Comm. 131: 1086 - 1093.
Goto Top

4. Boatright, J.H., Borst, D.E., Lin, Z.Y., Si, J.S., Bhati, M., Patel, D., Bruno, J., and Nickerson, J.M. Functional Characterization of Cis Acting Regulatory Elements of the IRBP Gene Promotor Region. 1995; Invest.Ophthalmol.Vis.Sci. 36: S123
Goto Top

5. Borst, D.E. DNA methylation of the IRBP promotor. 1994; Invest.Ophthalmol.Vis.Sci. 34: 1703
Goto Top

6. Bunt Milam, A.H. and Saari, J.C. Immunocytochemical localization of two retinoid-binding proteins in vertebrate retina. 1983; J.Cell Biol. 97: 703 - 712.
Goto Top Link to PudMed

7. Chen, Y. and Noy, N. Retinoid specificity of interphotoreceptor retinoid-binding protein. 1994; Biochemistry. 33: 10658 - 10665.
Goto Top Link to PudMed

8. Danciger, M., Kozak, C.A., Nickerson, J., Redmond, T.M., and Farber, D.B. Localization of the gene for interphotoreceptor retinoid-binding protein to mouse chromosome 14 near Np-1. 1990; Genomics. 8: 727 - 731.
Goto Top

9. Duncan, T., Palmer, K., Chader, G.J., and Wiggert, B. A Putative Retinal Pigment Cell Surface Receptor for Interphotoreceptor Retinoid Binding Protein (IRBP). 1995; Invest.Ophthalmol.Vis.Sci. 36: S122
Goto Top

10. Edwards, J.F., Storts, R.W., Joyce, J.R., Shelton, J.M., and Menzies, C.S. Juvenile-onset neuronal ceroid-lipofuscinosis in Rambouillet sheep. 1994; Vet.Pathol. 31: 48 - 54.
Goto Top Link to PudMed

11. Fei, Y., Matragoon, S., and Liou, G.I. Characterization of the interphotoreceptor retinoid binding protein (IRBP) gene promotor. 1995; Invest.Ophthalmol.Vis.Sci. 36: S123
Goto Top

12. Gonzalez Fernandez, F., Kittredge, K.L., Rayborn, M.E., Hollyfield, J.G., Landers, R.A., Saha, M., and Grainger, R.M. Interphotoreceptor retinoid-binding protein (IRBP), a major 124 kDa glycoprotein in the interphotoreceptor matrix of Xenopus laevis. Characterization, molecular cloning and biosynthesis. 1993; Journal.of.Cell Science. 105: 7 - 21.
Goto Top

13. Lin, Z.Y., Li, G.R., Phong, M., Si, J.S., Takizawa, N., and Nickerson, J.M. Study Of Recombinant Human IRBP And Its Mutations. 1997; Invest.Ophthalmol.Vis.Sci. 38: A1 - A1000.
Goto Top

14. Liou, G.I. Localization and identification of a retina-specific cis-element in the gene encoding Interphotoreceptor Retinoid-binding Protein (IRBP). 1993; Invest.Ophthalmol.Vis.Sci. 34: 830
Goto Top

15. Liou, G.I., Ma, D.P., Yang, Y.W., Geng, L., Zhu, C., and Baehr, W. Human interstitial retinoid-binding protein. Gene structure and primary structure. 1989; J.Biol.Chem. 264: 8200 - 8206.
Goto Top Link to PudMed

16. McGee, T.L., Berson, E.L., and Dryja, T.P. Search for point mutations in the interstitial retinol-binding protein gene in patients with hereditary retinal degenerations. 1992; Invest.Ophthalmol.Vis.Sci. 33 (Suppl.): 1396
Goto Top

17. McGee, T.L., DeStefano, J.D., Berson, E.L., and Dryja, T.P. A complete screen of the entire coding sequence of the IRBP gene for mutations in patients with hereditary degenerations. 1993; Invest.Ophthalmol.Vis.Sci. 34: 1460
Goto Top

18. Nickerson, J.M., Li, G.R., Lin, Z.Y., Takizawa, N., Si, J.S., and Gross, E.A. Structure-function relationships in the four repeats of human interphotoreceptor retinoid-binding protein (IRBP). 1998; Mol.Vis. 4:33: 33
Goto Top Link to PudMed

19. Oliver, E.R., Cunningham, L.L., van Niel, E.E., Youssri, A.I., and Gonzalez Fernandez, F. Molecular cloning and characterization of chicken Interphotoreceptor Retinoid-binding Protein. 1997; Invest.Ophthalmol.Vis.Sci. 38: S304
Goto Top

20. Payman, R. and Gonzalez Fernandez, F. Turnover of IRBP in the Xenopus interphotoreceptor matrix. 1995; Invest.Ophthalmol.Vis.Sci. Suppl. S6
Goto Top

21. Pfeffer, B., Wiggert, B., Lee, L., Zonnenberg, B., Newsome, D., and Chader, G. The presence of a soluble interphotoreceptor retinol-binding protein (IRBP) in the retinal interphotoreceptor space. 1983; J.Cell Physiol. 117: 333 - 341.
Goto Top Link to PudMed

22. Plantner, J.J., Quinn, T.A., and Luo, X. Metalloproteinases Associated With Interphotoreceptor Retinoid Binding Protein. 1996; Invest.Ophthalmol.Vis.Sci. S909
Goto Top

23. Redmond, T.M. and Nickerson, J.M. Retinoid binding to a recombinant central two-repeat segment of IRBP. 1992; Invest.Ophthalmol.Vis.Sci. 33: 1181
Goto Top

24. Ringens, P.J., Fang, M., Shinohara, T., Bridges, C.D., Lerea, C.L., Berson, E.L., and Dryja, T.P. Analysis of genes coding for S-antigen, interstitial retinol binding protein, and the alpha-subunit of cone transducin in patients with retinitis pigmentosa. 1990; Invest.Ophthalmol.Vis.Sci. 31: 1421 - 1426.
Goto Top Link to PudMed

25. Smith, S.B., Lee, L., Nickerson, J., Si, J.S., Chader, G.J., and Wiggert, B. Synthesis and secretion of interphotoreceptor retinoid-binding protein (IRBP) and developmental expression of IRBP mRNA in normal and rd mouse retinas. 1992; Exp.Eye Res. 54: 957 - 963.
Goto Top

26. Stenkamp, D.L., Cunningham, L.L., Raymond, P.A., and Gonzalez-Fernandez, F. Novel expression pattern of interphotoreceptor retinoid-binding protein (IRBP) in the adult and developing zebrafish retina and RPE. 1998; Mol.Vis. 4:26: 26
Goto Top Link to PudMed

27. van Niel, E.E., Rajendran, R.R., and Gonzalez Fernandez, F. Zebafish IRBP gene. 1995; Invest.Ophthalmol.Vis.Sci. 36: S122
Goto Top

28. Wolf, G. Transport of retinoids by the interphotoreceptor retinoid-binding protein. 1998; Nutr.Rev. 56: 156 - 158.
Goto Top Link to PudMed

29. Yokoyama, T., Liou, G.I., Caldwell, R.B., and Overbeek, P.A. Photoreceptor-specific activity of the human interphotoreceptor retinoid-binding protein (IRBP) promoter in transgenic mice. 1992; Exp.Eye Res. 55: 225 - 233.
Goto Top Link to PudMed


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Dr. rer. medic. Markus Preising, Dipl.Biol.
Molecular Genetics Laboratory
Department of Paediatric Ophthalmology, Strabismology and Ophthalmogenetics
University of Regensburg
Head: Prof. Dr. med. Birgit Lorenz