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Gene Review

RHO  -  rhodopsin

Sus scrofa

 
 
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Disease relevance of RHO

 

High impact information on RHO

  • In transgenic (Tg) swine with a rod-specific (rhodopsin) gene mutation, cone photoreceptor physiology was normal for months but later declined, consistent with delayed cone cell death [3].
  • The monomeric model of rhodopsin-like G protein-coupled receptors (GPCRs) has progressively yielded the floor to the concept of GPCRs being oligo(di)mers, but the functional correlates of dimerization remain unclear [4].
  • G protein beta gamma subunits increased the Vmax of the phosphorylation of rhodopsin 12-fold [5].
  • CONCLUSIONS: The rhodopsin transgenic pig retina shares many cytologic features with human retinas with retinitis pigmentosa and provides an opportunity to examine the earliest stages in photoreceptor degeneration, about which little is known in humans [6].
  • RESULTS: At birth, rod numbers were normal in the transgenic retinas, but their outer segments were short and disorganized and their inner segments contained stacks of rhodopsin-positive membranes [6].
 

Biological context of RHO

  • A model consistent with our observations introduces the idea of a binding site for the carboxy terminus of rhodopsin on rhodopsin kinase [1].
  • Therefore, methylation does not affect the intrinsic ability of T beta gamma to functionally interact with T alpha and R*. However, in disk membranes an approximate 2-fold effect was observed, with the methylated T beta gamma being more efficient [7].
  • The presence and localization of autoantibodies was determined in strain 13 guinea pigs with experimental retinal autoimmunity (ERA) induced by immunization with rhodopsin and rod outer segments (ROS) [8].
  • An antigenic peptide obtained by a cyanogene bromide cleavage of rhodopsin was purified and determined as residues 2-39 in the amino acid sequence [9].
 

Anatomical context of RHO

 

Associations of RHO with chemical compounds

 

Analytical, diagnostic and therapeutic context of RHO

References

  1. Altered light responses of single rod photoreceptors in transgenic pigs expressing P347L or P347S rhodopsin. Kraft, T.W., Allen, D., Petters, R.M., Hao, Y., Peng, Y.W., Wong, F. Mol. Vis. (2005) [Pubmed]
  2. Sequence variability in the retinal-attachment domain of mammalian rhodopsins. Pappin, D.J., Findlay, J.B. Biochem. J. (1984) [Pubmed]
  3. Retinal rod photoreceptor-specific gene mutation perturbs cone pathway development. Banin, E., Cideciyan, A.V., Alemán, T.S., Petters, R.M., Wong, F., Milam, A.H., Jacobson, S.G. Neuron (1999) [Pubmed]
  4. Glycoprotein hormone receptors: link between receptor homodimerization and negative cooperativity. Urizar, E., Montanelli, L., Loy, T., Bonomi, M., Swillens, S., Gales, C., Bouvier, M., Smits, G., Vassart, G., Costagliola, S. EMBO J. (2005) [Pubmed]
  5. Activation by G protein beta gamma subunits of agonist- or light-dependent phosphorylation of muscarinic acetylcholine receptors and rhodopsin. Haga, K., Haga, T. J. Biol. Chem. (1992) [Pubmed]
  6. Rhodopsin transgenic pigs as a model for human retinitis pigmentosa. Li, Z.Y., Wong, F., Chang, J.H., Possin, D.E., Hao, Y., Petters, R.M., Milam, A.H. Invest. Ophthalmol. Vis. Sci. (1998) [Pubmed]
  7. Functional significance of G protein carboxymethylation. Parish, C.A., Rando, R.R. Biochemistry (1994) [Pubmed]
  8. Localization of specific autoantibodies in the retinal photoreceptor cell layer in experimental retinal autoimmunity. Meyers-Elliott, R.H., Jacobs, D.R., Gammon, R.A. J. Neuroimmunol. (1983) [Pubmed]
  9. Anti-bovine rhodopsin monoclonal antibody recognizing light-dependent structural change. Takao, M., Iwasa, T., Yamamoto, H., Takeuchi, T., Tokunaga, F. Zool. Sci. (2002) [Pubmed]
  10. Stimulation of rhodopsin phosphorylation by guanine nucleotides in rod outer segments. Swarup, G., Garbers, D.L. Biochemistry (1983) [Pubmed]
  11. Experimental autoimmune uveo-retinitis and specificity of retinal antigens. Faure, J.P., Dorey, C., Van Tuyen, V., de Kozak, Y. Modern problems in ophthalmology. (1976) [Pubmed]
  12. Optical coherence tomography (OCT) abnormalities in rhodopsin mutant transgenic swine with retinal degeneration. Huang, Y., Cideciyan, A.V., Alemán, T.S., Banin, E., Huang, J., Syed, N.A., Petters, R.M., Wong, F., Milam, A.H., Jacobson, S.G. Exp. Eye Res. (2000) [Pubmed]
  13. Lensectomy and vitrectomy decrease the rate of photoreceptor loss in rhodopsin P347L transgenic pigs. Mahmoud, T.H., McCuen, B.W., Hao, Y., Moon, S.J., Tatebayashi, M., Stinnett, S., Petters, R.M., Wong, F. Graefes Arch. Clin. Exp. Ophthalmol. (2003) [Pubmed]
  14. Transplantation of full-thickness retina in the rhodopsin transgenic pig. Ghosh, F., Wong, F., Johansson, K., Bruun, A., Petters, R.M. Retina (Philadelphia, Pa.) (2004) [Pubmed]
 
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