PubRank

Michael E Cheetham

Author PubWeight™ 87.29‹?›

Top papers

Rank Title Journal Year PubWeight™‹?›
1 Guidelines for the nomenclature of the human heat shock proteins. Cell Stress Chaperones 2008 4.30
2 Mechanisms of cell death in rhodopsin retinitis pigmentosa: implications for therapy. Trends Mol Med 2005 2.96
3 Mutations in MRAP, encoding a new interacting partner of the ACTH receptor, cause familial glucocorticoid deficiency type 2. Nat Genet 2005 2.83
4 The cellular fate of mutant rhodopsin: quality control, degradation and aggresome formation. J Cell Sci 2002 2.65
5 EYS, encoding an ortholog of Drosophila spacemaker, is mutated in autosomal recessive retinitis pigmentosa. Nat Genet 2008 2.46
6 Mutations in LCA5, encoding the ciliary protein lebercilin, cause Leber congenital amaurosis. Nat Genet 2007 2.16
7 Not all J domains are created equal: implications for the specificity of Hsp40-Hsp70 interactions. Protein Sci 2005 2.10
8 Targeting amyloid-beta in glaucoma treatment. Proc Natl Acad Sci U S A 2007 1.77
9 Oxidative stress affects the junctional integrity of retinal pigment epithelial cells. Invest Ophthalmol Vis Sci 2004 1.75
10 Pharmacological manipulation of gain-of-function and dominant-negative mechanisms in rhodopsin retinitis pigmentosa. Hum Mol Genet 2008 1.52
11 Cystamine and cysteamine increase brain levels of BDNF in Huntington disease via HSJ1b and transglutaminase. J Clin Invest 2006 1.44
12 Suppression of protein aggregation by chaperone modification of high molecular weight complexes. Brain 2012 1.42
13 Deep intronic mutation in OFD1, identified by targeted genomic next-generation sequencing, causes a severe form of X-linked retinitis pigmentosa (RP23). Hum Mol Genet 2012 1.38
14 Localization in the human retina of the X-linked retinitis pigmentosa protein RP2, its homologue cofactor C and the RP2 interacting protein Arl3. Hum Mol Genet 2002 1.35
15 HSJ1 is a neuronal shuttling factor for the sorting of chaperone clients to the proteasome. Curr Biol 2005 1.33
16 The retinitis pigmentosa protein RP2 links pericentriolar vesicle transport between the Golgi and the primary cilium. Hum Mol Genet 2010 1.32
17 MRAP and MRAP2 are bidirectional regulators of the melanocortin receptor family. Proc Natl Acad Sci U S A 2009 1.31
18 The chaperone environment at the cytoplasmic face of the endoplasmic reticulum can modulate rhodopsin processing and inclusion formation. J Biol Chem 2003 1.28
19 The Leber congenital amaurosis gene product AIPL1 is localized exclusively in rod photoreceptors of the adult human retina. Hum Mol Genet 2002 1.25
20 Identification of novel RPGR ORF15 mutations in X-linked progressive cone-rod dystrophy (XLCORD) families. Invest Ophthalmol Vis Sci 2005 1.25
21 Differential expression of two distinct functional isoforms of melanopsin (Opn4) in the mammalian retina. J Neurosci 2009 1.22
22 An assessment of the apex microarray technology in genotyping patients with Leber congenital amaurosis and early-onset severe retinal dystrophy. Invest Ophthalmol Vis Sci 2007 1.21
23 A dual role for EDEM1 in the processing of rod opsin. J Cell Sci 2009 1.18
24 Molecular chaperone-mediated rescue of mitophagy by a Parkin RING1 domain mutant. Hum Mol Genet 2010 1.15
25 Inherited ACTH insensitivity illuminates the mechanisms of ACTH action. Trends Endocrinol Metab 2005 1.13
26 Late stage treatment with arimoclomol delays disease progression and prevents protein aggregation in the SOD1 mouse model of ALS. J Neurochem 2008 1.13
27 The melanocortin 2 receptor accessory protein exists as a homodimer and is essential for the function of the melanocortin 2 receptor in the mouse y1 cell line. Endocrinology 2007 1.12
28 Treatment with extracellular HSP70/HSC70 protein can reduce polyglutamine toxicity and aggregation. J Neurochem 2005 1.11
29 The ataxia protein sacsin is a functional co-chaperone that protects against polyglutamine-expanded ataxin-1. Hum Mol Genet 2009 1.09
30 Distinct melanocortin 2 receptor accessory protein domains are required for melanocortin 2 receptor interaction and promotion of receptor trafficking. Endocrinology 2008 1.09
31 Delineation of the plasma membrane targeting domain of the X-linked retinitis pigmentosa protein RP2. Invest Ophthalmol Vis Sci 2002 1.07
32 Mutations in the CACNA1F and NYX genes in British CSNBX families. Hum Mutat 2003 1.07
33 The cell stress machinery and retinal degeneration. FEBS Lett 2013 1.07
34 The Leber congenital amaurosis protein AIPL1 functions as part of a chaperone heterocomplex. Invest Ophthalmol Vis Sci 2008 1.07
35 The expression of the Leber congenital amaurosis protein AIPL1 coincides with rod and cone photoreceptor development. Invest Ophthalmol Vis Sci 2003 1.06
36 Molecular chaperones and photoreceptor function. Prog Retin Eye Res 2008 1.04
37 Nuclear translocation of the Hsp70/Hsp90 organizing protein mSTI1 is regulated by cell cycle kinases. J Cell Sci 2004 1.02
38 The Nance-Horan syndrome protein encodes a functional WAVE homology domain (WHD) and is important for co-ordinating actin remodelling and maintaining cell morphology. Hum Mol Genet 2010 0.99
39 Calnexin is not essential for mammalian rod opsin biogenesis. Mol Vis 2008 0.97
40 Mutations in ARL2BP, encoding ADP-ribosylation-factor-like 2 binding protein, cause autosomal-recessive retinitis pigmentosa. Am J Hum Genet 2013 0.97
41 X-linked cone dystrophy caused by mutation of the red and green cone opsins. Am J Hum Genet 2010 0.96
42 The Leber congenital amaurosis protein AIPL1 modulates the nuclear translocation of NUB1 and suppresses inclusion formation by NUB1 fragments. J Biol Chem 2004 0.96
43 Identification of novel mutations in the carbohydrate sulfotransferase gene (CHST6) causing macular corneal dystrophy. Invest Ophthalmol Vis Sci 2002 0.95
44 Hsp90 inhibition protects against inherited retinal degeneration. Hum Mol Genet 2013 0.93
45 Isolation and characterization of murine Cds (CDP-diacylglycerol synthase) 1 and 2. Gene 2005 0.92
46 Arl3 and RP2 mediated assembly and traffic of membrane associated cilia proteins. Vision Res 2012 0.92
47 The X-linked retinitis pigmentosa protein RP2 facilitates G protein traffic. Hum Mol Genet 2011 0.92
48 Molecular chaperone mediated late-stage neuroprotection in the SOD1(G93A) mouse model of amyotrophic lateral sclerosis. PLoS One 2013 0.91
49 Predominant rod photoreceptor degeneration in Leber congenital amaurosis. Mol Vis 2005 0.90
50 Knockdown of the zebrafish ortholog of the retinitis pigmentosa 2 (RP2) gene results in retinal degeneration. Invest Ophthalmol Vis Sci 2011 0.90
51 Focus on molecules: X-linked Retinitis Pigmentosa 2 protein, RP2. Exp Eye Res 2005 0.89
52 BiP prevents rod opsin aggregation. Mol Biol Cell 2012 0.89
53 Molecular mechanisms of disease for mutations at Gly-90 in rhodopsin. J Biol Chem 2011 0.88
54 Leber congenital amaurosis associated with AIPL1: challenges in ascribing disease causation, clinical findings, and implications for gene therapy. PLoS One 2012 0.87
55 The retinitis pigmentosa-mutated RP2 protein exhibits exonuclease activity and translocates to the nucleus in response to DNA damage. Exp Cell Res 2006 0.86
56 NUB1 modulation of GSK3β reduces tau aggregation. Hum Mol Genet 2012 0.86
57 Downstream caspases are novel targets for the antiapoptotic activity of the molecular chaperone hsp70. Cell Stress Chaperones 2004 0.86
58 X-linked megalocornea caused by mutations in CHRDL1 identifies an essential role for ventroptin in anterior segment development. Am J Hum Genet 2012 0.85
59 Novel retinal and cone photoreceptor transcripts revealed by human macular expression profiling. Invest Ophthalmol Vis Sci 2007 0.85
60 Three different cone opsin gene array mutational mechanisms with genotype-phenotype correlation and functional investigation of cone opsin variants. Hum Mutat 2014 0.84
61 Pharmacological manipulation of rhodopsin retinitis pigmentosa. Adv Exp Med Biol 2010 0.81
62 Focus on molecules: nyctalopin. Exp Eye Res 2005 0.81
63 The role of HSP70 and its co-chaperones in protein misfolding, aggregation and disease. Subcell Biochem 2015 0.81
64 Focus on molecules: centrosomal protein 290 (CEP290). Exp Eye Res 2010 0.81
65 Assay and functional analysis of the ARL3 effector RP2 involved in X-linked retinitis pigmentosa. Methods Enzymol 2005 0.80
66 Modulation of Sub-RPE deposits in vitro: a potential model for age-related macular degeneration. Invest Ophthalmol Vis Sci 2004 0.79
67 The chaperone function of the LCA protein AIPL1. AIPL1 chaperone function. Adv Exp Med Biol 2006 0.78
68 Organization on the plasma membrane of the retinitis pigmentosa protein RP2: investigation of association with detergent-resistant membranes and polarized sorting. Biochem J 2003 0.78
69 The inherited blindness protein AIPL1 regulates the ubiquitin-like FAT10 pathway. PLoS One 2012 0.78
70 The role of the X-linked retinitis pigmentosa protein RP2 in vesicle traffic and cilia function. Adv Exp Med Biol 2012 0.78
71 A novel missense mutation in both OPN1LW and OPN1MW cone opsin genes causes X-linked cone dystrophy (XLCOD5). Adv Exp Med Biol 2012 0.77
72 A simple cell based assay to measure Parkin activity. J Neurochem 2010 0.76
73 Functional characterization of a novel c.614-622del rhodopsin mutation in a French pedigree with retinitis pigmentosa. Mol Vis 2012 0.76
74 The binding of the molecular chaperone Hsc70 to the prion protein PrP is modulated by pH and copper. Int J Biochem Cell Biol 2010 0.75