Retinoids assist the cellular folding of the autosomal dominant retinitis pigmentosa opsin mutant P23H. (Q44760460)

6 December 2017

Retinoids assist the cellular folding of the autosomal dominant retinitis pigmentosa opsin mutant P23H. (English)

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6 December 2017

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5

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6 December 2017

Syed M Noorwez

1

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6 December 2017

Ritu Malhotra

2

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6 December 2017

J Hugh McDowell

3

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6 December 2017

Karen A Smith

4

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6 December 2017

Shalesh Kaushal

6

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6 December 2017

language of work or name

English

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publication date

9 February 2004

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Journal of Biological Chemistry

6 December 2017

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6 December 2017

279

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6 December 2017

16

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6 December 2017

16278-16284

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Interaction of rhodopsin with the G-protein, transducin

6 December 2017

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Crystal structure of rhodopsin: A G protein-coupled receptor

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Molecular genetics of Oguchi disease, fundus albipunctatus, and other forms of stationary night blindness: LVII Edward Jackson Memorial Lecture.

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On the genetics of retinitis pigmentosa and on mutation-independent approaches to therapeutic intervention

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The eye photoreceptor protein rhodopsin. Structural implications for retinal disease

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Structure and function in rhodopsin. 7. Point mutations associated with autosomal dominant retinitis pigmentosa

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Rhodopsin mutations in autosomal dominant retinitis pigmentosa

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Pharmacological chaperones: a new twist on receptor folding.

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Thermal destabilization of rhodopsin and opsin by proteolytic cleavage in bovine rod outer segment disk membranes

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Effect of vitamin A supplementation on rhodopsin mutants threonine-17 --> methionine and proline-347 --> serine in transgenic mice and in cell cultures

21 January 2018

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Pharmacological chaperone-mediated in vivo folding and stabilization of the P23H-opsin mutant associated with autosomal dominant retinitis pigmentosa

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Phyletic aspects of the distribution of 3-hydroxyretinal in the class Insecta.

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Structure and function in rhodopsin. Separation and characterization of the correctly folded and misfolded opsins produced on expression of an opsin mutant gene containing only the native intradiscal cysteine codons

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A rhodopsin mutant linked to autosomal dominant retinitis pigmentosa is prone to aggregate and interacts with the ubiquitin proteasome system

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Functional equivalence of metarhodopsin II and the Gt-activating form of photolyzed bovine rhodopsin

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Deactivation of rhodopsin in the transition from the signaling state meta II to meta III involves a thermal isomerization of the retinal chromophore C[double bond]D.

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Signaling states of rhodopsin. Formation of the storage form, metarhodopsin III, from active metarhodopsin II

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Functional heterogeneity of mutant rhodopsins responsible for autosomal dominant retinitis pigmentosa

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Structure and function in rhodopsin: correct folding and misfolding in two point mutants in the intradiscal domain of rhodopsin identified in retinitis pigmentosa

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Role of the intradiscal domain in rhodopsin assembly and function

21 January 2018

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Structure and function in rhodopsin: Mass spectrometric identification of the abnormal intradiscal disulfide bond in misfolded retinitis pigmentosa mutants

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Stability of dark state rhodopsin is mediated by a conserved ion pair in intradiscal loop E-2.

21 January 2018

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Altered functionality in rhodopsin point mutants associated with retinitis pigmentosa

21 January 2018

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Addition of the chromophore to rat rhodopsin is an early post-translational event

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Conformational disease

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Unfolding the role of protein misfolding in neurodegenerative diseases

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Serpinopathies and the conformational dementias

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Aggresomes: a cellular response to misfolded proteins

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A mutation of the Wilson disease protein, ATP7B, is degraded in the proteasomes and forms protein aggregates

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Deletion of exon 4 from human surfactant protein C results in aggresome formation and generation of a dominant negative

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Protein misfolding and disease; protein refolding and therapy

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Absorption Spectrum of Rhodopsin denatured with Acid

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Differential scanning calorimetry of bovine rhodopsin in rod-outer-segment disk membranes

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38 Thermal stability of rhodopsin and opsin in some novel detergents

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Quantitative determination of retinals with complete retention of their geometric configuration

21 January 2018

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Biosynthesis and assembly of the rod outer segment membrane system. Formation and fate of visual pigment in the frog retina

21 January 2018

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Incorporation of leucine into rhodopsin in isolated bovine retina

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21 January 2018

Identifiers

DOI

10.1074/JBC.M312101200

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6 December 2017

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