PubRank

G P Bates

Author PubWeight™ 122.48‹?›

Top papers

Rank Title Journal Year PubWeight™‹?›
1 Exon 1 of the HD gene with an expanded CAG repeat is sufficient to cause a progressive neurological phenotype in transgenic mice. Cell 1996 12.59
2 Aggregation of huntingtin in neuronal intranuclear inclusions and dystrophic neurites in brain. Science 1997 11.53
3 Formation of neuronal intranuclear inclusions underlies the neurological dysfunction in mice transgenic for the HD mutation. Cell 1997 9.57
4 A candidate for the cystic fibrosis locus isolated by selection for methylation-free islands. Nature 1987 7.88
5 Huntingtin-encoded polyglutamine expansions form amyloid-like protein aggregates in vitro and in vivo. Cell 1997 7.59
6 Self-assembly of polyglutamine-containing huntingtin fragments into amyloid-like fibrils: implications for Huntington's disease pathology. Proc Natl Acad Sci U S A 1999 4.58
7 Characterization of progressive motor deficits in mice transgenic for the human Huntington's disease mutation. J Neurosci 1999 4.56
8 The Huntington's disease protein interacts with p53 and CREB-binding protein and represses transcription. Proc Natl Acad Sci U S A 2000 4.01
9 A novel gene encoding an integral membrane protein is mutated in nephropathic cystinosis. Nat Genet 1998 3.24
10 Instability of highly expanded CAG repeats in mice transgenic for the Huntington's disease mutation. Nat Genet 1997 2.37
11 Altered brain neurotransmitter receptors in transgenic mice expressing a portion of an abnormal human huntington disease gene. Proc Natl Acad Sci U S A 1998 2.14
12 Nonapoptotic neurodegeneration in a transgenic mouse model of Huntington's disease. Proc Natl Acad Sci U S A 2000 2.00
13 Intranuclear neuronal inclusions in Huntington's disease and dentatorubral and pallidoluysian atrophy: correlation between the density of inclusions and IT15 CAG triplet repeat length. Neurobiol Dis 1998 1.96
14 Selective discrimination learning impairments in mice expressing the human Huntington's disease mutation. J Neurosci 1999 1.86
15 Ultrastructural localization and progressive formation of neuropil aggregates in Huntington's disease transgenic mice. Hum Mol Genet 1999 1.86
16 Abnormal synaptic plasticity and impaired spatial cognition in mice transgenic for exon 1 of the human Huntington's disease mutation. J Neurosci 2000 1.75
17 Impaired glutamate uptake in the R6 Huntington's disease transgenic mice. Neurobiol Dis 2001 1.71
18 Formation of polyglutamine inclusions in non-CNS tissue. Hum Mol Genet 1999 1.70
19 Structure and expression of the Huntington's disease gene: evidence against simple inactivation due to an expanded CAG repeat. Somat Cell Mol Genet 1994 1.66
20 SH3GL3 associates with the Huntingtin exon 1 protein and promotes the formation of polygln-containing protein aggregates. Mol Cell 1998 1.64
21 Sequence comparison of human and yeast telomeres identifies structurally distinct subtelomeric domains. Hum Mol Genet 1997 1.57
22 Microdissection of and microcloning from the short arm of human chromosome 2. Mol Cell Biol 1986 1.55
23 Heat exhaustion in a deep underground metalliferous mine. Occup Environ Med 2000 1.49
24 Centrosome disorganization in fibroblast cultures derived from R6/2 Huntington's disease (HD) transgenic mice and HD patients. Hum Mol Genet 2001 1.42
25 The huntingtin interacting protein HIP1 is a clathrin and alpha-adaptin-binding protein involved in receptor-mediated endocytosis. Hum Mol Genet 2001 1.25
26 Inhibition of polyglutamine aggregation in R6/2 HD brain slices-complex dose-response profiles. Neurobiol Dis 2001 1.23
27 Fluid losses and hydration status of industrial workers under thermal stress working extended shifts. Occup Environ Med 2003 1.18
28 Altered neurotransmitter receptor expression in transgenic mouse models of Huntington's disease. Philos Trans R Soc Lond B Biol Sci 1999 1.18
29 Are neuronal intranuclear inclusions the common neuropathology of triplet-repeat disorders with polyglutamine-repeat expansions? Lancet 1998 1.17
30 A zinc-finger gene ZNF141 mapping at 4p16.3/D4S90 is a candidate gene for the Wolf-Hirschhorn (4p-) syndrome. Hum Mol Genet 1993 1.15
31 The organization of the human complement factor I gene (IF): a member of the serine protease gene family. Genomics 1994 1.13
32 Mapping of cosmid clones in Huntington's disease region of chromosome 4. Somat Cell Mol Genet 1991 1.11
33 Detection of polyglutamine aggregation in mouse models. Methods Enzymol 1999 1.09
34 The direct screening of cosmid libraries with YAC clones. Nucleic Acids Res 1991 1.04
35 Amyloid-like inclusions in Huntington's disease. Neuroscience 2000 0.99
36 The risk of heat exhaustion at a deep underground metalliferous mine in relation to surface temperatures. Occup Med (Lond) 2000 0.98
37 HAP1-huntingtin interactions do not contribute to the molecular pathology in Huntington's disease transgenic mice. FEBS Lett 1998 0.98
38 The telomeric 60 kb of chromosome arm 4p is homologous to telomeric regions on 13p, 15p, 21p, and 22p. Genomics 1992 0.97
39 The risk of heat exhaustion at a deep underground metalliferous mine in relation to body-mass index and predicted VO2max. Occup Med (Lond) 2000 0.95
40 Brain neurotransmitter deficits in mice transgenic for the Huntington's disease mutation. J Neurochem 1999 0.94
41 Inclusion formation in Huntington's disease R6/2 mouse muscle cultures. J Neurochem 2003 0.91
42 Striking changes in anxiety in Huntington's disease transgenic mice. Brain Res 1998 0.90
43 Aberrant processing of the Fugu HD (FrHD) mRNA in mouse cells and in transgenic mice. Hum Mol Genet 1997 0.88
44 Transgenic models of Huntington's disease. Philos Trans R Soc Lond B Biol Sci 1999 0.87
45 Loss of cortical and thalamic neuronal tenascin-C expression in a transgenic mouse expressing exon 1 of the human Huntington disease gene. J Comp Neurol 2001 0.86
46 From neuronal inclusions to neurodegeneration: neuropathological investigation of a transgenic mouse model of Huntington's disease. Philos Trans R Soc Lond B Biol Sci 1999 0.86
47 Huntington's disease. Exploiting expression. Nature 2001 0.83
48 Partial resistance to malonate-induced striatal cell death in transgenic mouse models of Huntington's disease is dependent on age and CAG repeat length. J Neurochem 2001 0.82
49 A comparison of the effects of different substrata on chondrocyte morphology and the synthesis of collagen types IX and X. In Vitro Cell Dev Biol 1987 0.82
50 Cloning of the alpha-adducin gene from the Huntington's disease candidate region of chromosome 4 by exon amplification. Nat Genet 1992 0.81
51 Preparation and analysis of the products of non-enzymatic protein glycosylation and their relationship to cross-linking of proteins. Biochim Biophys Acta 1983 0.81
52 Fatigue in industrial workers under thermal stress on extended shift lengths. Occup Med (Lond) 2001 0.81
53 Oculodentodigital dysplasia. Mo Med 1977 0.79
54 The isolation of cDNAs within the Huntington disease region by hybridisation of yeast artificial chromosomes to a cDNA library. Hum Mol Genet 1993 0.78
55 A comparison of the morphological, histochemical and biochemical features of embryonic chick sternal chondrocytes in vivo with chondrocytes cultured in three-dimensional collagen gels. Bone Miner 1988 0.77
56 Transcript map of the human chromosome 4p16.3 consisting of 627 cDNA clones derived from 1 Mb of the Huntington's disease locus. DNA Res 1996 0.77
57 Increased prolylhydroxylase activity in chondrocytes but not synovial cells from adjuvant arthritic rat knees. Coll Relat Res 1986 0.76
58 RFLP for pHM20 (D2S12), an anonymous DNA sequence localised to 2p23-2pter. Nucleic Acids Res 1987 0.75
59 Exon trapping and sequence-based methods of gene finding in transcript mapping of human 4p16.3. Somat Cell Mol Genet 1997 0.75
60 Generation of high-density DNA markers from yeast artificial chromosome DNA by single unique primer-polymerase chain reaction. Genet Anal Tech Appl 1993 0.75
61 Distribution of trinucleotide repeat sequences across a 2 Mbp region containing the Huntington's disease gene. Hum Mol Genet 1994 0.75
62 Combustion safety in the aircraft environment. Aerosp Med 1969 0.75