PubRank

Stephan Kemp

Author PubWeight™ 42.94‹?›

Top papers

Rank Title Journal Year PubWeight™‹?›
1 Histone deacetylases (HDACs): characterization of the classical HDAC family. Biochem J 2003 11.27
2 X-linked adrenoleukodystrophy (X-ALD): clinical presentation and guidelines for diagnosis, follow-up and management. Orphanet J Rare Dis 2012 2.28
3 X-linked adrenoleukodystrophy in women: a cross-sectional cohort study. Brain 2014 2.16
4 Analysis of very long-chain fatty acids using electrospray ionization mass spectrometry. Mol Genet Metab 2003 1.28
5 Early oxidative damage underlying neurodegeneration in X-adrenoleukodystrophy. Hum Mol Genet 2008 1.27
6 The role of ELOVL1 in very long-chain fatty acid homeostasis and X-linked adrenoleukodystrophy. EMBO Mol Med 2010 1.26
7 A novel defect of peroxisome division due to a homozygous non-sense mutation in the PEX11β gene. J Med Genet 2012 1.17
8 Inactivation of the peroxisomal ABCD2 transporter in the mouse leads to late-onset ataxia involving mitochondria, Golgi and endoplasmic reticulum damage. Hum Mol Genet 2005 1.06
9 The peroxisomal ABC transporter family. Pflugers Arch 2006 1.05
10 Valproic acid induces antioxidant effects in X-linked adrenoleukodystrophy. Hum Mol Genet 2010 1.03
11 Lovastatin in X-linked adrenoleukodystrophy. N Engl J Med 2010 1.01
12 X-linked adrenoleukodystrophy: pathogenesis and treatment. Curr Neurol Neurosci Rep 2014 0.96
13 Characterization of the human omega-oxidation pathway for omega-hydroxy-very-long-chain fatty acids. FASEB J 2008 0.96
14 Gene expression profiling in response to the histone deacetylase inhibitor BL1521 in neuroblastoma. Exp Cell Res 2005 0.94
15 A very long-chain acyl-CoA synthetase-deficient mouse and its relevance to X-linked adrenoleukodystrophy. Hum Mol Genet 2003 0.90
16 Omega-oxidation of very long-chain fatty acids in human liver microsomes. Implications for X-linked adrenoleukodystrophy. J Biol Chem 2006 0.89
17 Contiguous gene deletion of ELOVL7, ERCC8 and NDUFAF2 in a patient with a fatal multisystem disorder. Hum Mol Genet 2009 0.89
18 Fatty acid omega-oxidation as a rescue pathway for fatty acid oxidation disorders in humans. FEBS J 2010 0.89
19 The novel histone deacetylase inhibitor BL1521 inhibits proliferation and induces apoptosis in neuroblastoma cells. Biochem Pharmacol 2004 0.86
20 Bezafibrate lowers very long-chain fatty acids in X-linked adrenoleukodystrophy fibroblasts by inhibiting fatty acid elongation. J Inherit Metab Dis 2012 0.86
21 Evidence for two enzymatic pathways for omega-oxidation of docosanoic acid in rat liver microsomes. J Lipid Res 2005 0.85
22 Enzymatic diagnosis of Sjögren-Larsson syndrome using electrospray ionization mass spectrometry. J Chromatogr B Analyt Technol Biomed Life Sci 2008 0.82
23 Mouse very long-chain acyl-CoA synthetase in X-linked adrenoleukodystrophy. J Biol Chem 2002 0.81
24 Bezafibrate for X-linked adrenoleukodystrophy. PLoS One 2012 0.81
25 Conservation of targeting but divergence in function and quality control of peroxisomal ABC transporters: an analysis using cross-kingdom expression. Biochem J 2011 0.81
26 A novel cell model to study the function of the adrenoleukodystrophy-related protein. Biochem Biophys Res Commun 2006 0.80
27 X-linked adrenomyeloneuropathy due to a novel missense mutation in the ABCD1 start codon presenting as demyelinating neuropathy. J Peripher Nerv Syst 2011 0.80
28 Accumulation of very long-chain fatty acids does not affect mitochondrial function in adrenoleukodystrophy protein deficiency. Hum Mol Genet 2005 0.78
29 X-linked adrenoleukodystrophy: molecular and functional analysis of the ABCD1 gene in Argentinean patients. PLoS One 2012 0.77
30 Hematopoietic cell transplantation does not prevent myelopathy in X-linked adrenoleukodystrophy: a retrospective study. J Inherit Metab Dis 2014 0.77
31 Intellectual disability and hemizygous GPD2 mutation. Am J Med Genet A 2013 0.77
32 The cystathionine beta-synthase variant c.844_845ins68 protects against CNS demyelination in X-linked adrenoleukodystrophy. Hum Mutat 2006 0.76
33 Clinical utility gene card for: adrenoleukodystrophy. Eur J Hum Genet 2011 0.75
34 Comment on the paper "Effect of statin treatment on adrenomyeloneuropathy with cerebral inflammation: a revisit". Clin Neurol Neurosurg 2013 0.75