Evidence map›Paper›PMID 25422369›Full record

ArticleFASEB journal : official publication of the Federation of American Societies for Experimental Biology2015

Mistrafficking of prenylated proteins causes retinitis pigmentosa 2.

Houbin Zhang, Christin Hanke-Gogokhia, Li Jiang, Xiaobo Li, Pu Wang, Cecilia D Gerstner, Jeanne M Frederick, Zhenglin Yang, Wolfgang Baehr

Open access · greenAbstract read
PubMed Full text at PMC Publisher
In one paragraph

Article in FASEB journal : official publication of the Federation of American Societies for Experimental Biology, 2015. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 41 papers.

0numbers the graph read from it
0cells of the map it votes in
41citing papers in PubMed
3.7field-weighted citation impact, top 6% of its field
1 · What the graph read from it

What it found

Each row is one number read from the abstract, on the scale the paper reported it, with its interval. Left of the dashed line favours the treatment, right favours the comparator. Under each row is the sentence it came from. New to these charts? A ten-minute tutorial.

The abstract states no effect estimate the extractor could read, or names no intervention and outcome on the map, so this paper lights no cell and moves no belief. It is still indexed, cited and linked below.

2 · The registry

The trial behind it

Trials whose registry record cites this paper, or whose number appears in the abstract. A trial that started after this paper was published is citing it as background, not reporting it.

Neither the registry nor the abstract names a trial number. If this is a trial report, that itself is worth knowing.

3 · Its place in the literature

Who cites it

41 citing papers in PubMed, 64 citations in OpenAlex.

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  9. Dawn and dusk peaks of outer segment phagocytosis, and visual cycle function require Rab28.FASEB journal : official publication of the Federation of American Societies for Experimental Biology · 2022
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  15. An improved method for preparation of mouse retinal cryosections.European journal of histochemistry : EJH · 2020
    Article
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4 · The record

Corrections and comments

PubMed lists nothing against this paper. Absence here is not a guarantee, only a check that was made.

5 · Who and what money

Authors and funding

9 authors at 4 institutions in 3 countries.

Houbin Zhang*The Sichuan Provincial Key Laboratory for Human Disease Gene Study, The Institute of Laboratory Medicine, Hospital of University of Electronic Science and Technology of China and Sichuan Provincial People's Hospital, Chengdu, Sichuan, China; School of Medicine, University of Electronic Science and Technology of China, Chengdu, Sichuan, China; Department of Ophthalmology and Visual Sciences, John A. Moran Eye Center, and Department of Neurobiology and Anatomy, University of Utah School of Medicine, Salt Lake City, Utah, USA; Department of Biochemistry and Biology, University of Potsdam, Potsdam-Golm, Germany; and Department of Biology, University of Utah, Salt Lake City, Utah, USA houbin.zhang@gmail.com wbaehr@hsc.utah.edu.
Christin Hanke-Gogokhia*The Sichuan Provincial Key Laboratory for Human Disease Gene Study, The Institute of Laboratory Medicine, Hospital of University of Electronic Science and Technology of China and Sichuan Provincial People's Hospital, Chengdu, Sichuan, China; School of Medicine, University of Electronic Science and Technology of China, Chengdu, Sichuan, China; Department of Ophthalmology and Visual Sciences, John A. Moran Eye Center, and Department of Neurobiology and Anatomy, University of Utah School of Medicine, Salt Lake City, Utah, USA; Department of Biochemistry and Biology, University of Potsdam, Potsdam-Golm, Germany; and Department of Biology, University of Utah, Salt Lake City, Utah, USA.
Li Jiang*The Sichuan Provincial Key Laboratory for Human Disease Gene Study, The Institute of Laboratory Medicine, Hospital of University of Electronic Science and Technology of China and Sichuan Provincial People's Hospital, Chengdu, Sichuan, China; School of Medicine, University of Electronic Science and Technology of China, Chengdu, Sichuan, China; Department of Ophthalmology and Visual Sciences, John A. Moran Eye Center, and Department of Neurobiology and Anatomy, University of Utah School of Medicine, Salt Lake City, Utah, USA; Department of Biochemistry and Biology, University of Potsdam, Potsdam-Golm, Germany; and Department of Biology, University of Utah, Salt Lake City, Utah, USA.
Xiaobo Li*The Sichuan Provincial Key Laboratory for Human Disease Gene Study, The Institute of Laboratory Medicine, Hospital of University of Electronic Science and Technology of China and Sichuan Provincial People's Hospital, Chengdu, Sichuan, China; School of Medicine, University of Electronic Science and Technology of China, Chengdu, Sichuan, China; Department of Ophthalmology and Visual Sciences, John A. Moran Eye Center, and Department of Neurobiology and Anatomy, University of Utah School of Medicine, Salt Lake City, Utah, USA; Department of Biochemistry and Biology, University of Potsdam, Potsdam-Golm, Germany; and Department of Biology, University of Utah, Salt Lake City, Utah, USA.
Pu Wang*The Sichuan Provincial Key Laboratory for Human Disease Gene Study, The Institute of Laboratory Medicine, Hospital of University of Electronic Science and Technology of China and Sichuan Provincial People's Hospital, Chengdu, Sichuan, China; School of Medicine, University of Electronic Science and Technology of China, Chengdu, Sichuan, China; Department of Ophthalmology and Visual Sciences, John A. Moran Eye Center, and Department of Neurobiology and Anatomy, University of Utah School of Medicine, Salt Lake City, Utah, USA; Department of Biochemistry and Biology, University of Potsdam, Potsdam-Golm, Germany; and Department of Biology, University of Utah, Salt Lake City, Utah, USA.
Cecilia D Gerstner*The Sichuan Provincial Key Laboratory for Human Disease Gene Study, The Institute of Laboratory Medicine, Hospital of University of Electronic Science and Technology of China and Sichuan Provincial People's Hospital, Chengdu, Sichuan, China; School of Medicine, University of Electronic Science and Technology of China, Chengdu, Sichuan, China; Department of Ophthalmology and Visual Sciences, John A. Moran Eye Center, and Department of Neurobiology and Anatomy, University of Utah School of Medicine, Salt Lake City, Utah, USA; Department of Biochemistry and Biology, University of Potsdam, Potsdam-Golm, Germany; and Department of Biology, University of Utah, Salt Lake City, Utah, USA.
Jeanne M Frederick*The Sichuan Provincial Key Laboratory for Human Disease Gene Study, The Institute of Laboratory Medicine, Hospital of University of Electronic Science and Technology of China and Sichuan Provincial People's Hospital, Chengdu, Sichuan, China; School of Medicine, University of Electronic Science and Technology of China, Chengdu, Sichuan, China; Department of Ophthalmology and Visual Sciences, John A. Moran Eye Center, and Department of Neurobiology and Anatomy, University of Utah School of Medicine, Salt Lake City, Utah, USA; Department of Biochemistry and Biology, University of Potsdam, Potsdam-Golm, Germany; and Department of Biology, University of Utah, Salt Lake City, Utah, USA.
Zhenglin Yang*The Sichuan Provincial Key Laboratory for Human Disease Gene Study, The Institute of Laboratory Medicine, Hospital of University of Electronic Science and Technology of China and Sichuan Provincial People's Hospital, Chengdu, Sichuan, China; School of Medicine, University of Electronic Science and Technology of China, Chengdu, Sichuan, China; Department of Ophthalmology and Visual Sciences, John A. Moran Eye Center, and Department of Neurobiology and Anatomy, University of Utah School of Medicine, Salt Lake City, Utah, USA; Department of Biochemistry and Biology, University of Potsdam, Potsdam-Golm, Germany; and Department of Biology, University of Utah, Salt Lake City, Utah, USA.
Wolfgang Baehr*The Sichuan Provincial Key Laboratory for Human Disease Gene Study, The Institute of Laboratory Medicine, Hospital of University of Electronic Science and Technology of China and Sichuan Provincial People's Hospital, Chengdu, Sichuan, China; School of Medicine, University of Electronic Science and Technology of China, Chengdu, Sichuan, China; Department of Ophthalmology and Visual Sciences, John A. Moran Eye Center, and Department of Neurobiology and Anatomy, University of Utah School of Medicine, Salt Lake City, Utah, USA; Department of Biochemistry and Biology, University of Potsdam, Potsdam-Golm, Germany; and Department of Biology, University of Utah, Salt Lake City, Utah, USA houbin.zhang@gmail.com wbaehr@hsc.utah.edu.
Sichuan Provincial Hospital of Traditional Chinese Medicine · CNUniversity of Utah · USUniversity of Electronic Science and Technology of China · CNUniversity of Potsdam · DE

Funding

University of Utah, Core Vision Research GrantP30EY014800 · NEI · UTAH STATE HIGHER EDUCATION SYSTEM--UNIVERSITY OF UTAH · PI PAUL STEVEN BERNSTEIN · 2005 to 2026
$14.6M
RETINAL PHOTORECEPTOR CYCLIC GMP PHOSPHODIESTERASER01EY008123 · NEI · UNIVERSITY OF UTAH · PI BAEHR, WOLFGANG · 1990 to 2019
$7.8M
Membrane Protein Transport in PhotoreceptorsR01EY019298 · NEI · UNIVERSITY OF UTAH · PI BAEHR, WOLFGANG · 2009 to 2018
$3.7M
The Function of the Retinitis Pigmentosa Protein RP2R21EY020969 · NEI · UNIVERSITY OF UTAH · PI ZHANG, HOUBIN · 2010 to 2010
$150k
NEI NIH HHS EY014800-039003NEI NIH HHS EY019298NEI NIH HHS EY020969NEI NIH HHS EY08123NEI NIH HHS R01 EY008123NEI NIH HHS R01 EY019298NEI NIH HHS R21 EY020969
6 · The paper itself

Abstract

The retinitis pigmentosa 2 polypeptide (RP2) functions as a GTPase-activating protein (GAP) for ARL3 (Arf-like protein 3), a small GTPase. ARL3 is an effector of phosphodiesterase 6 Δ (PDE6D), a prenyl-binding protein and chaperone of prenylated protein in photoreceptors. Mutations in the human RP2 gene cause X-linked retinitis pigmentosa (XLRP) and cone-rod dystrophy (XL-CORD). To study mechanisms causing XLRP, we generated an RP2 knockout mouse. The Rp2h(-/-) mice exhibited a slowly progressing rod-cone dystrophy simulating the human disease. Rp2h(-/-) scotopic a-wave and photopic b-wave amplitudes declined at 1 mo of age and continued to decline over the next 6 mo. Prenylated PDE6 subunits and G-protein coupled receptor kinase 1 (GRK1) were unable to traffic effectively to the Rp2h(-/-) outer segments. Mechanistically, absence of RP2 GAP activity increases ARL3-GTP levels, forcing PDE6D to assume a predominantly "closed" conformation that impedes binding of lipids. Lack of interaction disrupts trafficking of PDE6 and GRK1 to their destination, the photoreceptor outer segments. We propose that hyperactivity of ARL3-GTP in RP2 knockout mice and human patients with RP2 null alleles leads to XLRP resembling recessive rod-cone dystrophy.

Indexed as

Protein PrenylationADP-Ribosylation FactorsAnimalsAntibody FormationCaenorhabditis elegansCiliaCyclic Nucleotide Phosphodiesterases, Type 6ElectroretinographyEye ProteinsFemaleG-Protein-Coupled Receptor Kinase 1GTP-Binding ProteinsGuanosine TriphosphateHumansImmunoblottingImmunoenzyme TechniquesADP-Ribosylation FactorsArl3 protein, mouseCyclic Nucleotide Phosphodiesterases, Type 6Eye ProteinsG-Protein-Coupled Receptor Kinase 1Grk1 protein, mouseGTP-Binding ProteinsGuanosine TriphosphateIntracellular Signaling Peptides and ProteinsMembrane ProteinsRP2 protein, humanARL3PDE6Drod-cone dystrophyRP2XLRP

Identifiers

PMID25422369
PMCPMC4422365
OpenAlexW2047179478

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Read under generation 80e0d062 · epoch 390. Bibliography from PubMed, PubMed Central and OpenAlex; grants from NIH RePORTER; trial links from ClinicalTrials.gov; estimates, votes and beliefs from the OpenQuestion graph.