4BEY
Night blindness causing G90D rhodopsin in complex with GaCT2 peptide
Summary for 4BEY
| Entry DOI | 10.2210/pdb4bey/pdb |
| Related | 4BEZ |
| Descriptor | Rhodopsin, Guanine nucleotide-binding protein G(t) subunit alpha-1, alpha-D-mannopyranose-(1-3)-beta-D-mannopyranose-(1-4)-2-acetamido-2-deoxy-beta-D-glucopyranose-(1-4)-2-acetamido-2-deoxy-beta-D-glucopyranose, ... (8 entities in total) |
| Functional Keywords | membrane protein, gpcr, disease mutant, congential stationary night blindness, active state |
| Biological source | Bos taurus (Bovine) More |
| Total number of polymer chains | 2 |
| Total formula weight | 41810.70 |
| Authors | Singhal, A.,Ostermaier, M.K.,Vishnivetskiy, S.A.,Panneels, V.,Homan, K.T.,Tesmer, J.J.G.,Veprintsev, D.,Deupi, X.,Gurevich, V.V.,Schertler, G.F.X.,Standfuss, J. (deposition date: 2013-03-12, release date: 2013-05-08, Last modification date: 2023-12-20) |
| Primary citation | Singhal, A.,Ostermaier, M.K.,Vishnivetskiy, S.A.,Panneels, V.,Homan, K.T.,Tesmer, J.J.,Veprintsev, D.,Deupi, X.,Gurevich, V.V.,Schertler, G.F.,Standfuss, J. Insights Into Congenital Stationary Night Blindness Based on the Structure of G90D Rhodopsin. Embo Rep., 14:520-, 2013 Cited by PubMed Abstract: We present active-state structures of the G protein-coupled receptor (GPCRs) rhodopsin carrying the disease-causing mutation G90D. Mutations of G90 cause either retinitis pigmentosa (RP) or congenital stationary night blindness (CSNB), a milder, non-progressive form of RP. Our analysis shows that the CSNB-causing G90D mutation introduces a salt bridge with K296. The mutant thus interferes with the E113Q-K296 activation switch and the covalent binding of the inverse agonist 11-cis-retinal, two interactions that are crucial for the deactivation of rhodopsin. Other mutations, including G90V causing RP, cannot promote similar interactions. We discuss our findings in context of a model in which CSNB is caused by constitutive activation of the visual signalling cascade. PubMed: 23579341DOI: 10.1038/EMBOR.2013.44 PDB entries with the same primary citation |
| Experimental method | X-RAY DIFFRACTION (2.9 Å) |
Structure validation
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