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Top papers

Rank Title Journal Year PubWeight™‹?›
1 Targeted disruption of the acid alpha-glucosidase gene in mice causes an illness with critical features of both infantile and adult human glycogen storage disease type II. J Biol Chem 1998 2.24
2 Conditional up-regulation of MHC class I in skeletal muscle leads to self-sustaining autoimmune myositis and myositis-specific autoantibodies. Proc Natl Acad Sci U S A 2000 2.13
3 Carrier frequency for glycogen storage disease type II in New York and estimates of affected individuals born with the disease. Am J Med Genet 1998 1.75
4 International consensus outcome measures for patients with idiopathic inflammatory myopathies. Development and initial validation of myositis activity and damage indices in patients with adult onset disease. Rheumatology (Oxford) 2003 1.71
5 Systemic correction of the muscle disorder glycogen storage disease type II after hepatic targeting of a modified adenovirus vector encoding human acid-alpha-glucosidase. Proc Natl Acad Sci U S A 1999 1.46
6 Surprises of genetic engineering: a possible model of polyglucosan body disease. Neurology 2001 1.29
7 A motif in human histidyl-tRNA synthetase which is shared among several aminoacyl-tRNA synthetases is a coiled-coil that is essential for enzymatic activity and contains the major autoantigenic epitope. J Biol Chem 1994 1.22
8 A variety of cytokines and immunologically relevant surface molecules are expressed by normal human skeletal muscle cells under proinflammatory stimuli. Clin Exp Immunol 1998 1.17
9 Prosthetic joint infection with Mycoplasma hominis. J Infect Dis 1986 1.16
10 Pathway of B1-Alu expression in microinjected oocytes: Xenopus laevis proteins associated with nuclear precursor and processed cytoplasmic RNAs. Mol Cell Biol 1988 1.08
11 The inhibition of apoptosis in myositis and in normal muscle cells. J Immunol 2000 1.03
12 Detection and imaging of non-contractile inclusions and sarcomeric anomalies in skeletal muscle by second harmonic generation combined with two-photon excited fluorescence. J Struct Biol 2008 1.02
13 A 5' splice junction mutation leading to exon deletion in an Ashkenazic Jewish family with phosphofructokinase deficiency (Tarui disease). J Biol Chem 1993 1.00
14 Modulation of disease severity in mice with targeted disruption of the acid alpha-glucosidase gene. Neuromuscul Disord 2000 1.00
15 Functional expression of human mutant phosphofructokinase in yeast: genetic defects in French Canadian and Swiss patients with phosphofructokinase deficiency. Am J Hum Genet 1995 0.96
16 Human alanyl-tRNA synthetase: conservation in evolution of catalytic core and microhelix recognition. Biochemistry 1995 0.95
17 Magnetic resonance imaging in the idiopathic inflammatory myopathies. J Rheumatol 1991 0.91
18 Human histidyl-tRNA synthetase: recognition of amino acid signature regions in class 2a aminoacyl-tRNA synthetases. Nucleic Acids Res 1992 0.88
19 Symmetry of binding sites of a mouse IgA myeloma protein (MOPC 315). Biochemistry 1978 0.84
20 Correction of glycogen storage disease type II by enzyme replacement with a recombinant human acid maltase produced by over-expression in a CHO-DHFR(neg) cell line. Biochem Biophys Res Commun 2000 0.83
21 Genetic defects in patients with glycogenosis type II (acid maltase deficiency). Muscle Nerve Suppl 1995 0.80
22 Isometric exercise increases strength and does not produce sustained creatinine phosphokinase increases in a patient with polymyositis. J Rheumatol 1993 0.79
23 Quantitative assessment of myositis in thigh muscles using magnetic resonance imaging. Magn Reson Imaging 1999 0.76
24 Letter: Effect of salicylates on creatinine clearance. Br Med J 1976 0.75
25 Adeno-associated virus in normal and myositis human skeletal muscle. Neurology 2000 0.75
26 Various classes of mutations in patients with phosphofructokinase deficiency (Tarui's disease). Muscle Nerve Suppl 1995 0.75
27 The new refuseniks. Science 1984 0.75