Published in PLoS One on February 21, 2012
Diffusion tensor magnetic resonance imaging in the analysis of neurodegenerative diseases. J Vis Exp (2013) 0.95
Oxidative stress, DNA damage, and the telomeric complex as therapeutic targets in acute neurodegeneration. Neurochem Int (2013) 0.92
DNA damage in the oligodendrocyte lineage and its role in brain aging. Mech Ageing Dev (2016) 0.75
Defective repair replication of DNA in xeroderma pigmentosum. Nature (1968) 8.62
Understanding the odd science of aging. Cell (2005) 6.50
Xeroderma pigmentosum. Cutaneous, ocular, and neurologic abnormalities in 830 published cases. Arch Dermatol (1987) 5.88
A new progeroid syndrome reveals that genotoxic stress suppresses the somatotroph axis. Nature (2006) 5.57
The problem of functional localization in the human brain. Nat Rev Neurosci (2002) 4.85
Defective DNA repair and neurodegenerative disease. Cell (2007) 2.57
Xeroderma pigmentosum, trichothiodystrophy and Cockayne syndrome: a complex genotype-phenotype relationship. Neuroscience (2007) 2.55
Cockayne syndrome and xeroderma pigmentosum. Neurology (2000) 2.24
Are amygdalar volume alterations in children with Tourette syndrome due to ADHD comorbidity? Dev Med Child Neurol (2008) 2.11
Transcription - guarding the genome by sensing DNA damage. Nat Rev Cancer (2004) 2.10
Incidence of DNA repair deficiency disorders in western Europe: Xeroderma pigmentosum, Cockayne syndrome and trichothiodystrophy. DNA Repair (Amst) (2008) 2.02
Trichothiodystrophy: a systematic review of 112 published cases characterises a wide spectrum of clinical manifestations. J Med Genet (2008) 1.85
Neurological symptoms and natural course of xeroderma pigmentosum. Brain (2008) 1.78
Xeroderma pigmentosum and related disorders: defects in DNA repair and transcription. Adv Genet (2001) 1.67
Neurological disease in xeroderma pigmentosum. Documentation of a late onset type of the juvenile onset form. Brain (1991) 1.49
Preservation of diffusion tensor properties during spatial normalization by use of tensor imaging and fibre tracking on a normal brain database. Phys Med Biol (2007) 1.32
Relationship of neurologic degeneration to genotype in three xeroderma pigmentosum group G patients. J Invest Dermatol (2002) 1.32
Phenotypic heterogeneity in the XPB DNA helicase gene (ERCC3): xeroderma pigmentosum without and with Cockayne syndrome. Hum Mutat (2006) 1.31
The case for 8,5'-cyclopurine-2'-deoxynucleosides as endogenous DNA lesions that cause neurodegeneration in xeroderma pigmentosum. Neuroscience (2006) 1.26
Xeroderma pigmentosum/cockayne syndrome complex: first neuropathological study and review of eight other cases. Eur J Paediatr Neurol (2001) 1.13
Correction of high-order eddy current induced geometric distortion in diffusion-weighted echo-planar images. Magn Reson Med (2004) 0.99
Mutations in the XPD gene leading to xeroderma pigmentosum symptoms. Hum Mutat (1997) 0.98
Tissue-specific accelerated aging in nucleotide excision repair deficiency. Mech Ageing Dev (2008) 0.97
Idiopathic chronic inflammatory demyelinating polyneuropathy: an epidemiological study in Italy. J Neurol Neurosurg Psychiatry (2007) 0.95
DNA repair in aging rat neurons. Neuroscience (2006) 0.93
Diffusion tensor imaging and tractwise fractional anisotropy statistics: quantitative analysis in white matter pathology. Biomed Eng Online (2007) 0.92
Intersubject variability in the analysis of diffusion tensor images at the group level: fractional anisotropy mapping and fiber tracking techniques. Magn Reson Imaging (2008) 0.91
Comparison of manual direct and automated indirect measurement of hippocampus using magnetic resonance imaging. Eur J Radiol (2007) 0.91
Are users of lipid-lowering drugs at increased risk of peripheral neuropathy? Eur J Clin Pharmacol (2001) 0.89
Peripheral neuropathy in xeroderma pigmentosum. Brain (1990) 0.88
Brainstem and basal ganglia lesions in xeroderma pigmentosum group A. J Neuropathol Exp Neurol (2004) 0.84
Ultraviolet-B-induced apoptosis and cytokine release in xeroderma pigmentosum keratinocytes. J Invest Dermatol (2000) 0.79
Clinical and photobiological characteristics of xeroderma pigmentosum complementation group F: a review of cases from Japan. Br J Dermatol (1989) 0.78
Neurodegeneration in Xeroderma Pigmentosum: a trinucleotide repeat mutation analysis. J Neurol Sci (1999) 0.77
The "Dutch DNA Repair Group", in retrospect. Mutat Res (2001) 0.76
Genome-wide association study identifies 19p13.3 (UNC13A) and 9p21.2 as susceptibility loci for sporadic amyotrophic lateral sclerosis. Nat Genet (2009) 4.38
An unrestrained proinflammatory M1 macrophage population induced by iron impairs wound healing in humans and mice. J Clin Invest (2011) 3.69
A canonical to non-canonical Wnt signalling switch in haematopoietic stem-cell ageing. Nature (2013) 2.62
Cdc42 activity regulates hematopoietic stem cell aging and rejuvenation. Cell Stem Cell (2012) 2.60
p16INK4A is a robust in vivo biomarker of cellular aging in human skin. Aging Cell (2006) 2.34
X-linked bulbospinal neuronopathy: Kennedy disease. Arch Neurol (2002) 2.21
Are amygdalar volume alterations in children with Tourette syndrome due to ADHD comorbidity? Dev Med Child Neurol (2008) 2.11
Role of beta2-integrins for homing and neovascularization capacity of endothelial progenitor cells. J Exp Med (2004) 2.04
Incidence of DNA repair deficiency disorders in western Europe: Xeroderma pigmentosum, Cockayne syndrome and trichothiodystrophy. DNA Repair (Amst) (2008) 2.02
Energy metabolism in amyotrophic lateral sclerosis. Lancet Neurol (2010) 2.00
Proteins of nucleotide and base excision repair pathways interact in mitochondria to protect from loss of subcutaneous fat, a hallmark of aging. J Exp Med (2010) 1.86
Granulomatous mycosis fungoides and granulomatous slack skin: a multicenter study of the Cutaneous Lymphoma Histopathology Task Force Group of the European Organization For Research and Treatment of Cancer (EORTC). Arch Dermatol (2008) 1.78
Two German kindreds with familial amyotrophic lateral sclerosis due to TARDBP mutations. Arch Neurol (2008) 1.78
Four familial ALS pedigrees discordant for two SOD1 mutations: are all SOD1 mutations pathogenic? J Neurol Neurosurg Psychiatry (2010) 1.68
Wound healing defect of Vav3-/- mice due to impaired {beta}2-integrin-dependent macrophage phagocytosis of apoptotic neutrophils. Blood (2009) 1.66
Pathogenic role for skin macrophages in a mouse model of keratinocyte-induced psoriasis-like skin inflammation. J Clin Invest (2006) 1.65
Activated macrophages are essential in a murine model for T cell-mediated chronic psoriasiform skin inflammation. J Clin Invest (2006) 1.58
Ultraviolet-B irradiation and matrix metalloproteinases: from induction via signaling to initial events. Ann N Y Acad Sci (2002) 1.56
Global brain atrophy and corticospinal tract alterations in ALS, as investigated by voxel-based morphometry of 3-D MRI. Amyotroph Lateral Scler Other Motor Neuron Disord (2005) 1.55
Keratin 14 Cre transgenic mice authenticate keratin 14 as an oocyte-expressed protein. Genesis (2004) 1.48
Interleukin-12 suppresses ultraviolet radiation-induced apoptosis by inducing DNA repair. Nat Cell Biol (2002) 1.48
Diagnosis and treatment of bulbar symptoms in amyotrophic lateral sclerosis. Nat Clin Pract Neurol (2008) 1.48
Images in cardiovascular medicine. Cardiomyopathy in a Duchenne muscular dystrophy carrier and her diseased son: similar pattern revealed by cardiovascular MRI. Circulation (2010) 1.47
Angiogenin variants in Parkinson disease and amyotrophic lateral sclerosis. Ann Neurol (2011) 1.45
Manganese superoxide dismutase and aldehyde dehydrogenase deficiency increase mitochondrial oxidative stress and aggravate age-dependent vascular dysfunction. Cardiovasc Res (2008) 1.43
Heterozygous R1101K mutation of the DCTN1 gene in a family with ALS and FTD. Ann Neurol (2005) 1.42
Recommendation: daily sun protection in the prevention of chronic UV-induced skin damage. J Dtsch Dermatol Ges (2007) 1.41
Hepatic activation of IKK/NFκB signaling induces liver fibrosis via macrophage-mediated chronic inflammation. Hepatology (2012) 1.40
Severity of depressive symptoms and quality of life in patients with amyotrophic lateral sclerosis. Neurorehabil Neural Repair (2005) 1.40
The chemokine CXCL13 is a prognostic marker in clinically isolated syndrome (CIS). PLoS One (2010) 1.32
Amyotrophic lateral sclerosis--a model of corticofugal axonal spread. Nat Rev Neurol (2013) 1.29
The chemokine CXCL13 in acute neuroborreliosis. J Neurol Neurosurg Psychiatry (2009) 1.26
IgG antibodies against measles, rubella, and varicella zoster virus predict conversion to multiple sclerosis in clinically isolated syndrome. PLoS One (2009) 1.25
Neutrophil primary granule proteins HBP and HNP1-3 boost bacterial phagocytosis by human and murine macrophages. J Clin Invest (2008) 1.25
Beta2 integrins separate graft-versus-host disease and graft-versus-leukemia effects. Blood (2007) 1.23
Active MAC-1 (CD11b/CD18) on DCs inhibits full T-cell activation. Blood (2006) 1.23
Daptomycin is highly efficacious against penicillin-resistant and penicillin- and quinolone-resistant pneumococci in experimental meningitis. Antimicrob Agents Chemother (2004) 1.23
Whole brain-based analysis of regional white matter tract alterations in rare motor neuron diseases by diffusion tensor imaging. Hum Brain Mapp (2010) 1.23
Wound-healing defect of CD18(-/-) mice due to a decrease in TGF-beta1 and myofibroblast differentiation. EMBO J (2005) 1.22
Riluzole in Huntington's disease: a 3-year, randomized controlled study. Ann Neurol (2007) 1.21
Treatment of melasma. J Am Acad Dermatol (2006) 1.21
Beta2 integrins are required for skin homing of primed T cells but not for priming naive T cells. J Clin Invest (2002) 1.18
The Vav binding site of the non-receptor tyrosine kinase Syk at Tyr 348 is critical for beta2 integrin (CD11/CD18)-mediated neutrophil migration. Blood (2006) 1.18
No evidence for increased skin cancer risk in psoriasis patients treated with broadband or narrowband UVB phototherapy: a first retrospective study. Acta Derm Venereol (2004) 1.16
Disease severity and progression in progressive supranuclear palsy and multiple system atrophy: validation of the NNIPPS--Parkinson Plus Scale. PLoS One (2011) 1.15
Alternative trial design in amyotrophic lateral sclerosis saves time and patients. Amyotroph Lateral Scler (2007) 1.15
The role of ultraviolet radiation in melanomagenesis. Exp Dermatol (2010) 1.14
Heterozygous deficiency of manganese superoxide dismutase results in severe lipid peroxidation and spontaneous apoptosis in murine myocardium in vivo. Free Radic Biol Med (2005) 1.14
The modulation of Amyotrophic Lateral Sclerosis risk by ataxin-2 intermediate polyglutamine expansions is a specific effect. Neurobiol Dis (2011) 1.12
Neuroectodermally converted human mesenchymal stromal cells provide cytoprotective effects on neural stem cells and inhibit their glial differentiation. Cytotherapy (2010) 1.10
Tumour necrosis factor α secretion induces protease activation and acinar cell necrosis in acute experimental pancreatitis in mice. Gut (2012) 1.09
Accelerated aging phenotype in mice with conditional deficiency for mitochondrial superoxide dismutase in the connective tissue. Aging Cell (2010) 1.09
A randomized, double blind, placebo-controlled trial of pioglitazone in combination with riluzole in amyotrophic lateral sclerosis. PLoS One (2012) 1.09
A point mutation in the dynein heavy chain gene leads to striatal atrophy and compromises neurite outgrowth of striatal neurons. Hum Mol Genet (2010) 1.09
Histopathological analysis of skeletal muscle in patients with Parkinson's disease and 'dropped head'/'bent spine' syndrome. Parkinsonism Relat Disord (2009) 1.08
Oxidative stress in chronic venous leg ulcers. Wound Repair Regen (2005) 1.08
NF-κB is required for Smac mimetic-mediated sensitization of glioblastoma cells for γ-irradiation-induced apoptosis. Mol Cancer Ther (2011) 1.06
alpha-melanocyte-stimulating hormone suppresses bleomycin-induced collagen synthesis and reduces tissue fibrosis in a mouse model of scleroderma: melanocortin peptides as a novel treatment strategy for scleroderma? Arthritis Rheum (2009) 1.06
RhoH is important for positive thymocyte selection and T-cell receptor signaling. Blood (2006) 1.06
Late-onset motoneuron disease caused by a functionally modified AMPA receptor subunit. Proc Natl Acad Sci U S A (2005) 1.06
Linking neuron and skin: matrix metalloproteinases in amyotrophic lateral sclerosis (ALS). J Neurol Sci (2009) 1.05
A 9-centimorgan interval of chromosome 10 controls the T cell-dependent psoriasiform skin disease and arthritis in a murine psoriasis model. J Immunol (2008) 1.05
Phosphoinositide 3-kinases upregulate system xc(-) via eukaryotic initiation factor 2α and activating transcription factor 4 - A pathway active in glioblastomas and epilepsy. Antioxid Redox Signal (2014) 1.05
Mice with a mutation in the dynein heavy chain 1 gene display sensory neuropathy but lack motor neuron disease. Exp Neurol (2008) 1.05
Grey-matter abnormalities in boys with Tourette syndrome: magnetic resonance imaging study using optimised voxel-based morphometry. Br J Psychiatry (2006) 1.04
Expression of stromal cell markers in distinct compartments of human skin cancers. J Cutan Pathol (2006) 1.04
Direct comparison of [18F]FDG PET/CT with PET alone and with side-by-side PET and CT in patients with malignant melanoma. Eur J Nucl Med Mol Imaging (2007) 1.03
CD4+ T cell-associated pathophysiology critically depends on CD18 gene dose effects in a murine model of psoriasis. J Immunol (2003) 1.03
Microbiology of aggressive periodontitis. Periodontol 2000 (2014) 1.02
CD5+ B cells from individuals with systemic lupus erythematosus express granzyme B. Eur J Immunol (2010) 1.02
Heterozygous deficiency of manganese superoxide dismutase in mice (Mn-SOD+/-): a novel approach to assess the role of oxidative stress for the development of nitrate tolerance. Mol Pharmacol (2005) 1.02
A role for apoptosis in the control of neutrophil homeostasis in the circulation: insights from CD18-deficient mice. Blood (2002) 1.02
[AWMF Guideline no. 013/066. Diagnosis and therapy of circumscribed scleroderma]. J Dtsch Dermatol Ges (2009) 1.02
Laryngospasm: an underdiagnosed symptom of X-linked spinobulbar muscular atrophy. Neurology (2005) 1.01
Cardiac involvement in patients with Becker muscular dystrophy: new diagnostic and pathophysiological insights by a CMR approach. J Cardiovasc Magn Reson (2008) 1.01
Inflammatory dysregulation of blood monocytes in Parkinson's disease patients. Acta Neuropathol (2014) 1.00
MRI-based functional neuroimaging in ALS: an update. Amyotroph Lateral Scler (2009) 0.99
A novel phosphorylation site mutation in profilin 1 revealed in a large screen of US, Nordic, and German amyotrophic lateral sclerosis/frontotemporal dementia cohorts. Neurobiol Aging (2012) 0.98
Cerebrospinal fluid biomarkers of neurodegeneration in chronic neurological diseases. Expert Rev Mol Diagn (2008) 0.98
Class IA phosphoinositide 3-kinase β and δ regulate neutrophil oxidase activation in response to Aspergillus fumigatus hyphae. J Immunol (2011) 0.98
CSF concentrations of cAMP and cGMP are lower in patients with Creutzfeldt-Jakob disease but not Parkinson's disease and amyotrophic lateral sclerosis. PLoS One (2012) 0.98
Molecular mechanisms of the crosstalk between mitochondria and NADPH oxidase through reactive oxygen species-studies in white blood cells and in animal models. Antioxid Redox Signal (2013) 0.98
Low stability of Huntington muscle mitochondria against Ca2+ in R6/2 mice. Ann Neurol (2006) 0.97