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9.A.14.1.2
Rhodopsin. Photoreceptor required for image-forming vision at low light intensity. Light-induced isomerization of 11-cis to all-trans retinal triggers a conformational change leading to G-protein activation and release of all-trans retinal. The tetraspanning peripherin-2 (TC# 8.A.40.1.2) links rhodopsin to a cyclic nucleotide-dependent channel (TC# 1.A.1.5.3) in the outer segments of rod photoreceptors.  The G266D retinitis pigmentosa mutation in TMS4 of rhodopsin abolishes binding of peripherin-2 and prevents association with the CNGA1/CNGB1a subunits present in the complex (Becirovic et al. 2014).  A channel through opsin, responsible for the passage of retinal from and to its central site where it forms a Schiff's base with a lysine in TMS7 has been proposed (Hildebrand et al. 2009). Blankenship et al. 2015 presented the 2.3-A resolution structure of native source rhodopsin stabilized in a conformation competent for G protein binding. An extensive water-mediated hydrogen bond network linking the chromophore binding site to the site of G protein binding was observed, providing connections to conserved motifs essential for GPCR activation.  Both Opsin and Rhodopsin serve as phospholipid flippases (scramblases) and thus have been implicated in photoreceptor disc membrane homeostasis (Menon et al. 2011; Goren et al. 2014; Ernst and Menon 2015Wang et al. 2018). One particular conformation of rhodopsin is an open channel connecting the ligand binding site with the membrane and the intradiscal lumen of rod outer segments. Sufficient in size, the passageway permits the exchange of hydrophobic ligands such as retinal (Mattle et al. 2018).

Accession Number:P08100
Protein Name:Rhodopsin
Length:348
Molecular Weight:38893.00
Species:Homo sapiens (Human) [9606]
Number of TMSs:7
Location1 / Topology2 / Orientation3: Membrane1 / Multi-pass membrane protein2
Substrate H+

Cross database links:

Entrez Gene ID: 6010   
Pfam: PF00001    PF10413   
KEGG: hsa:6010    hsa:6010   

Gene Ontology

GO:0005794 C:Golgi apparatus
GO:0005887 C:integral to plasma membrane
GO:0060342 C:photoreceptor inner segment membrane
GO:0042622 C:photoreceptor outer segment membrane
GO:0004930 F:G-protein coupled receptor activity
GO:0046872 F:metal ion binding
GO:0009881 F:photoreceptor activity
GO:0005515 F:protein binding
GO:0018298 P:protein-chromophore linkage
GO:0016056 P:rhodopsin mediated signaling pathway

References (66)

[1] “Isolation and nucleotide sequence of the gene encoding human rhodopsin.”  Nathans J.et.al.   6589631
[2] “The full-ORF clone resource of the German cDNA consortium.”  Bechtel S.et.al.   17974005
[3] “The status, quality, and expansion of the NIH full-length cDNA project: the Mammalian Gene Collection (MGC).”  The MGC Project Teamet.al.   15489334
[4] “Sequence analysis of the 5.34-kb 5' flanking region of the human rhodopsin-encoding gene.”  Bennett J.et.al.   8566799
[5] “Rhodopsin mutations in autosomal dominant retinitis pigmentosa.”  Al-Maghtheh M.et.al.   8401533
[6] “Autosomal dominant retinitis pigmentosa: absence of the rhodopsin proline-->histidine substitution (codon 23) in pedigrees from Europe.”  Farrar G.J.et.al.   2239971
[7] “A point mutation of the rhodopsin gene in one form of retinitis pigmentosa.”  Dryja T.P.et.al.   2137202
[8] “Mutations within the rhodopsin gene in patients with autosomal dominant retinitis pigmentosa.”  Dryja T.P.et.al.   2215617
[9] “A 3-bp deletion in the rhodopsin gene in a family with autosomal dominant retinitis pigmentosa.”  Inglehearn C.F.et.al.   1985460
[10] “Identification of novel rhodopsin mutations associated with retinitis pigmentosa by GC-clamped denaturing gradient gel electrophoresis.”  Sheffield V.C.et.al.   1897520
[11] “Pro-347-Arg mutation of the rhodopsin gene in autosomal dominant retinitis pigmentosa.”  Gal A.et.al.   1840561
[12] “Rhodopsin mutations in autosomal dominant retinitis pigmentosa.”  Sung C.H.et.al.   1862076
[13] “Mutation spectrum of the rhodopsin gene among patients with autosomal dominant retinitis pigmentosa.”  Dryja T.P.et.al.   1833777
[14] “Autosomal dominant retinitis pigmentosa: a novel mutation in the rhodopsin gene in the original 3q linked family.”  Farrar G.J.et.al.   1302614
[15] “Point mutations of rhodopsin gene found in Japanese families with autosomal dominant retinitis pigmentosa (ADRP).”  Fujiki K.et.al.   1391967
[16] “Identification of novel rhodopsin mutations responsible for retinitis pigmentosa: implications for the structure and function of rhodopsin.”  Macke J.P.et.al.   8317502
[17] “Autosomal dominant 'sector' retinitis pigmentosa due to a point mutation predicting an Asn-15-Ser substitution of rhodopsin.”  Kranich H.et.al.   8353500
[18] “Heterozygous missense mutation in the rhodopsin gene as a cause of congenital stationary night blindness.”  Dryja T.P.et.al.   8358437
[19] “Further screening of the rhodopsin gene in patients with autosomal dominant retinitis pigmentosa.”  Vaithinathan R.et.al.   8088850
[20] “Identification of a novel rhodopsin mutation (Met-44-Thr) in a simplex case of retinitis pigmentosa.”  Reig C.et.al.   8076945
[21] “Three novel rhodopsin mutations (C110F, L131P, A164V) in patients with autosomal dominant retinitis pigmentosa.”  Fuchs S.et.al.   7981701
[22] “Identification of a new mutation at codon 171 of rhodopsin gene causing autosomal dominant retinitis pigmentosa.”  Antinolo G.et.al.   7987326
[23] “Five novel missense mutations of the rhodopsin gene in autosomal dominant retinitis pigmentosa.”  Souied E.et.al.   7987331
[24] “Two new rhodopsin transversion mutations (L40R; M216K) in families with autosomal dominant retinitis pigmentosa.”  Al-Maghtheh M.et.al.   8081400
[25] “Autosomal dominant retinitis pigmentosa in a large family: a clinical and molecular genetic study.”  Rosas D.J.et.al.   8045708
[26] “Missense rhodopsin mutation in a family with recessive RP.”  Kumaramanickavel G.et.al.   7987385
[27] “Rhodopsin mutation proline347-to-alanine in a family with autosomal dominant retinitis pigmentosa indicates an important role for proline at position 347.”  Macke J.P.et.al.   7633434
[28] “Dark-light: model for nightblindness from the human rhodopsin Gly-90-->Asp mutation.”  Sieving P.A.et.al.   7846071
[29] “Retinitis punctata albescens associated with the Arg135Trp mutation in the rhodopsin gene.”  Souied E.et.al.   8554077
[30] “Rhodopsin mutation G109R in a family with autosomal dominant retinitis pigmentosa.”  Goliath R.et.al.   9452035
[31] “A novel mutation within the rhodopsin gene (Thr-94-Ile) causing autosomal dominant congenital stationary night blindness.”  Al-Jandal N.et.al.   9888392
[32] “A dual role for EDEM1 in the processing of rod opsin.”  Kosmaoglou M.et.al.   19934218
[33] “A homozygous p.Glu150Lys mutation in the opsin gene of two Pakistani families with autosomal recessive retinitis pigmentosa.”  Azam M.et.al.   19960070
[34] “Isolation and nucleotide sequence of the gene encoding human rhodopsin.”  Nathans J.et.al.   6589631
[35] “The full-ORF clone resource of the German cDNA consortium.”  Bechtel S.et.al.   17974005
[36] “The status, quality, and expansion of the NIH full-length cDNA project: the Mammalian Gene Collection (MGC).”  The MGC Project Teamet.al.   15489334
[37] “Sequence analysis of the 5.34-kb 5' flanking region of the human rhodopsin-encoding gene.”  Bennett J.et.al.   8566799
[38] “Rhodopsin mutations in autosomal dominant retinitis pigmentosa.”  Al-Maghtheh M.et.al.   8401533
[39] “Autosomal dominant retinitis pigmentosa: absence of the rhodopsin proline-->histidine substitution (codon 23) in pedigrees from Europe.”  Farrar G.J.et.al.   2239971
[40] “A point mutation of the rhodopsin gene in one form of retinitis pigmentosa.”  Dryja T.P.et.al.   2137202
[41] “Mutations within the rhodopsin gene in patients with autosomal dominant retinitis pigmentosa.”  Dryja T.P.et.al.   2215617
[42] “A 3-bp deletion in the rhodopsin gene in a family with autosomal dominant retinitis pigmentosa.”  Inglehearn C.F.et.al.   1985460
[43] “Identification of novel rhodopsin mutations associated with retinitis pigmentosa by GC-clamped denaturing gradient gel electrophoresis.”  Sheffield V.C.et.al.   1897520
[44] “Pro-347-Arg mutation of the rhodopsin gene in autosomal dominant retinitis pigmentosa.”  Gal A.et.al.   1840561
[45] “Rhodopsin mutations in autosomal dominant retinitis pigmentosa.”  Sung C.H.et.al.   1862076
[46] “Mutation spectrum of the rhodopsin gene among patients with autosomal dominant retinitis pigmentosa.”  Dryja T.P.et.al.   1833777
[47] “Autosomal dominant retinitis pigmentosa: a novel mutation in the rhodopsin gene in the original 3q linked family.”  Farrar G.J.et.al.   1302614
[48] “Point mutations of rhodopsin gene found in Japanese families with autosomal dominant retinitis pigmentosa (ADRP).”  Fujiki K.et.al.   1391967
[49] “Identification of novel rhodopsin mutations responsible for retinitis pigmentosa: implications for the structure and function of rhodopsin.”  Macke J.P.et.al.   8317502
[50] “Autosomal dominant 'sector' retinitis pigmentosa due to a point mutation predicting an Asn-15-Ser substitution of rhodopsin.”  Kranich H.et.al.   8353500
[51] “Heterozygous missense mutation in the rhodopsin gene as a cause of congenital stationary night blindness.”  Dryja T.P.et.al.   8358437
[52] “Further screening of the rhodopsin gene in patients with autosomal dominant retinitis pigmentosa.”  Vaithinathan R.et.al.   8088850
[53] “Identification of a novel rhodopsin mutation (Met-44-Thr) in a simplex case of retinitis pigmentosa.”  Reig C.et.al.   8076945
[54] “Three novel rhodopsin mutations (C110F, L131P, A164V) in patients with autosomal dominant retinitis pigmentosa.”  Fuchs S.et.al.   7981701
[55] “Identification of a new mutation at codon 171 of rhodopsin gene causing autosomal dominant retinitis pigmentosa.”  Antinolo G.et.al.   7987326
[56] “Five novel missense mutations of the rhodopsin gene in autosomal dominant retinitis pigmentosa.”  Souied E.et.al.   7987331
[57] “Two new rhodopsin transversion mutations (L40R; M216K) in families with autosomal dominant retinitis pigmentosa.”  Al-Maghtheh M.et.al.   8081400
[58] “Autosomal dominant retinitis pigmentosa in a large family: a clinical and molecular genetic study.”  Rosas D.J.et.al.   8045708
[59] “Missense rhodopsin mutation in a family with recessive RP.”  Kumaramanickavel G.et.al.   7987385
[60] “Rhodopsin mutation proline347-to-alanine in a family with autosomal dominant retinitis pigmentosa indicates an important role for proline at position 347.”  Macke J.P.et.al.   7633434
[61] “Dark-light: model for nightblindness from the human rhodopsin Gly-90-->Asp mutation.”  Sieving P.A.et.al.   7846071
[62] “Retinitis punctata albescens associated with the Arg135Trp mutation in the rhodopsin gene.”  Souied E.et.al.   8554077
[63] “Rhodopsin mutation G109R in a family with autosomal dominant retinitis pigmentosa.”  Goliath R.et.al.   9452035
[64] “A novel mutation within the rhodopsin gene (Thr-94-Ile) causing autosomal dominant congenital stationary night blindness.”  Al-Jandal N.et.al.   9888392
[65] “A dual role for EDEM1 in the processing of rod opsin.”  Kosmaoglou M.et.al.   19934218
[66] “A homozygous p.Glu150Lys mutation in the opsin gene of two Pakistani families with autosomal recessive retinitis pigmentosa.”  Azam M.et.al.   19960070
Structure:
4ZWJ   5DGY   5W0P   6CMO     

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Predict TMSs (Predict number of transmembrane segments)
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FASTA formatted sequence
1:	MNGTEGPNFY VPFSNATGVV RSPFEYPQYY LAEPWQFSML AAYMFLLIVL GFPINFLTLY 
61:	VTVQHKKLRT PLNYILLNLA VADLFMVLGG FTSTLYTSLH GYFVFGPTGC NLEGFFATLG 
121:	GEIALWSLVV LAIERYVVVC KPMSNFRFGE NHAIMGVAFT WVMALACAAP PLAGWSRYIP 
181:	EGLQCSCGID YYTLKPEVNN ESFVIYMFVV HFTIPMIIIF FCYGQLVFTV KEAAAQQQES 
241:	ATTQKAEKEV TRMVIIMVIA FLICWVPYAS VAFYIFTHQG SNFGPIFMTI PAFFAKSAAI 
301:	YNPVIYIMMN KQFRNCMLTT ICCGKNPLGD DEASATVSKT ETSQVAPA