4cj6: Difference between revisions

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~~'''Unreleased~~ structure~~'''~~

===Crystal structure of the complex of the Cellular Retinal Binding Protein Mutant R234W with 9-cis-retinal===

~~The~~ entry ~~4cj6~~ is ~~ON HOLD~~

==Disease==

[[http://www.uniprot.org/uniprot/RLBP1_HUMAN RLBP1_HUMAN]] Defects in RLBP1 are a cause of retinitis pigmentosa autosomal recessive (ARRP) [MIM:[http://omim.org/entry/268000 268000]]. RP leads to degeneration of retinal photoreceptor cells. Patients typically have night vision blindness and loss of midperipheral visual field. As their condition progresses, they lose their far peripheral visual field and eventually central vision as well.<ref>PMID:9326942</ref> Defects in RLBP1 are the cause of Bothnia retinal dystrophy (BRD) [MIM:[http://omim.org/entry/607475 607475]]; also known as Vasterbotten dystrophy. Affected individuals show night blindness from early childhood with features consistent with retinitis punctata albescens and macular degeneration.<ref>PMID:10102298</ref> Defects in RLBP1 are the cause of rod-cone dystrophy Newfoundland (NFRCD) [MIM:[http://omim.org/entry/607476 607476]]. NFRCD is a retinal dystrophy reminiscent of retinitis punctata albescens but with a substantially lower age at onset and more-rapid and distinctive progression. Rod-cone dystrophies results from initial loss of rod photoreceptors, later followed by cone photoreceptors loss.<ref>PMID:11868161</ref> Defects in RLBP1 are a cause of retinitis punctata albescens (RPA) [MIM:[http://omim.org/entry/136880 136880]]. A rare form of stationary night blindness characterized by a delay in the regeneration of cone and rod photopigments.

~~Authors~~: ~~Bolze, C~~.S.~~, Helbling, R.E~~., ~~Owen, R~~.~~L., Pearson, A.R., Pompidor, G., Dworkowski, F., Fuchs, M.R., Furrer, J., Golczak, M., Palczewski, K., Cascella, M., Stocker, A~~.

==Function==

[[http://www.uniprot.org/uniprot/RLBP1_HUMAN RLBP1_HUMAN]] Soluble retinoid carrier essential the proper function of both rod and cone photoreceptors. Participates in the regeneration of active 11-cis-retinol and 11-cis-retinaldehyde, from the inactive 11-trans products of the rhodopsin photocycle and in the de novo synthesis of these retinoids from 11-trans metabolic precursors. The cycling of retinoids between photoreceptor and adjacent pigment epithelium cells is known as the 'visual cycle'.<ref>PMID:19846785</ref>

~~Description~~: ~~Crystal structure of the complex of the Cellular Retinal Binding Protein Mutant R234W with~~ 9-cis-retinal

==About this Structure==

[[4cj6]] is a 1 chain structure. Full crystallographic information is available from [http://oca.weizmann.ac.il/oca-bin/ocashort?id=4CJ6 OCA].

==Reference==

[[Category: Bolze, C S.]]

[[Category: Cascella, M.]]

[[Category: Dworkowski, F.]]

[[Category: Fuchs, M R.]]

[[Category: Furrer, J.]]

[[Category: Golczak, M.]]

[[Category: Helbling, R E.]]

[[Category: Owen, R L.]]

[[Category: Palczewski, K.]]

[[Category: Pearson, A R.]]

[[Category: Pompidor, G.]]

[[Category: Stocker, A.]]

[[Category: 9-cis-retinal]]

[[Category: Isomerase]]

[[Category: Visual cycle]]

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@@ Line 1: / Line 1: @@
-'''Unreleased structure'''
+{{STRUCTURE_4cj6|  PDB=4cj6  |  SCENE=  }}
+===Crystal structure of the complex of the Cellular Retinal Binding Protein Mutant R234W with 9-cis-retinal===
+{{ABSTRACT_PUBMED_24328211}}
-The entry 4cj6 is ON HOLD
+==Disease==
+[[http://www.uniprot.org/uniprot/RLBP1_HUMAN RLBP1_HUMAN]] Defects in RLBP1 are a cause of retinitis pigmentosa autosomal recessive (ARRP) [MIM:[http://omim.org/entry/268000 268000]]. RP leads to degeneration of retinal photoreceptor cells. Patients typically have night vision blindness and loss of midperipheral visual field. As their condition progresses, they lose their far peripheral visual field and eventually central vision as well.<ref>PMID:9326942</ref>   Defects in RLBP1 are the cause of Bothnia retinal dystrophy (BRD) [MIM:[http://omim.org/entry/607475 607475]]; also known as Vasterbotten dystrophy. Affected individuals show night blindness from early childhood with features consistent with retinitis punctata albescens and macular degeneration.<ref>PMID:10102298</ref>   Defects in RLBP1 are the cause of rod-cone dystrophy Newfoundland (NFRCD) [MIM:[http://omim.org/entry/607476 607476]]. NFRCD is a retinal dystrophy reminiscent of retinitis punctata albescens but with a substantially lower age at onset and more-rapid and distinctive progression. Rod-cone dystrophies results from initial loss of rod photoreceptors, later followed by cone photoreceptors loss.<ref>PMID:11868161</ref>   Defects in RLBP1 are a cause of retinitis punctata albescens (RPA) [MIM:[http://omim.org/entry/136880 136880]]. A rare form of stationary night blindness characterized by a delay in the regeneration of cone and rod photopigments.
-Authors: Bolze, C.S., Helbling, R.E., Owen, R.L., Pearson, A.R., Pompidor, G., Dworkowski, F., Fuchs, M.R., Furrer, J., Golczak, M., Palczewski, K., Cascella, M., Stocker, A.
+==Function==
+[[http://www.uniprot.org/uniprot/RLBP1_HUMAN RLBP1_HUMAN]] Soluble retinoid carrier essential the proper function of both rod and cone photoreceptors. Participates in the regeneration of active 11-cis-retinol and 11-cis-retinaldehyde, from the inactive 11-trans products of the rhodopsin photocycle and in the de novo synthesis of these retinoids from 11-trans metabolic precursors. The cycling of retinoids between photoreceptor and adjacent pigment epithelium cells is known as the 'visual cycle'.<ref>PMID:19846785</ref>
-Description: Crystal structure of the complex of the Cellular Retinal Binding Protein Mutant R234W with 9-cis-retinal
+==About this Structure==
+[[4cj6]] is a 1 chain structure. Full crystallographic information is available from [http://oca.weizmann.ac.il/oca-bin/ocashort?id=4CJ6 OCA].
+==Reference==
+<ref group="xtra">PMID:024328211</ref><references group="xtra"/><references/>
+[[Category: Bolze, C S.]]
+[[Category: Cascella, M.]]
+[[Category: Dworkowski, F.]]
+[[Category: Fuchs, M R.]]
+[[Category: Furrer, J.]]
+[[Category: Golczak, M.]]
+[[Category: Helbling, R E.]]
+[[Category: Owen, R L.]]
+[[Category: Palczewski, K.]]
+[[Category: Pearson, A R.]]
+[[Category: Pompidor, G.]]
+[[Category: Stocker, A.]]
+[[Category: 9-cis-retinal]]
+[[Category: Isomerase]]
+[[Category: Visual cycle]]