PubRank

Konrad Sandhoff

Author PubWeight™ 102.94‹?›

Top papers

Rank Title Journal Year PubWeight™‹?›
1 Direct observation of the nanoscale dynamics of membrane lipids in a living cell. Nature 2008 6.38
2 Hsp70 stabilizes lysosomes and reverts Niemann-Pick disease-associated lysosomal pathology. Nature 2010 2.62
3 Apoptotic vesicles crossprime CD8 T cells and protect against tuberculosis. Immunity 2006 2.61
4 Enhanced insulin sensitivity in mice lacking ganglioside GM3. Proc Natl Acad Sci U S A 2003 2.56
5 Principles of lysosomal membrane digestion: stimulation of sphingolipid degradation by sphingolipid activator proteins and anionic lysosomal lipids. Annu Rev Cell Dev Biol 2005 2.41
6 The epidermal barrier function is dependent on the serine protease CAP1/Prss8. J Cell Biol 2005 2.36
7 Combinatorial ganglioside biosynthesis. J Biol Chem 2002 2.06
8 Sphingolipid metabolism diseases. Biochim Biophys Acta 2006 2.01
9 Saposin C is required for lipid presentation by human CD1b. Nat Immunol 2004 1.88
10 Acid ceramidase overexpression prevents the inhibitory effects of saturated fatty acids on insulin signaling. J Biol Chem 2005 1.63
11 Adult ceramide synthase 2 (CERS2)-deficient mice exhibit myelin sheath defects, cerebellar degeneration, and hepatocarcinomas. J Biol Chem 2009 1.58
12 Lysosomal degradation of membrane lipids. FEBS Lett 2009 1.50
13 Principles of lysosomal membrane degradation: Cellular topology and biochemistry of lysosomal lipid degradation. Biochim Biophys Acta 2008 1.46
14 Systemic inflammation in glucocerebrosidase-deficient mice with minimal glucosylceramide storage. J Clin Invest 2002 1.45
15 Interruption of ganglioside synthesis produces central nervous system degeneration and altered axon-glial interactions. Proc Natl Acad Sci U S A 2005 1.43
16 Identification of the protein receptor binding site of botulinum neurotoxins B and G proves the double-receptor concept. Proc Natl Acad Sci U S A 2006 1.41
17 Normal epidermal differentiation but impaired skin-barrier formation upon keratinocyte-restricted IKK1 ablation. Nat Cell Biol 2007 1.40
18 Nrf2 links epidermal barrier function with antioxidant defense. EMBO Mol Med 2012 1.35
19 Integrity and barrier function of the epidermis critically depend on glucosylceramide synthesis. J Biol Chem 2006 1.27
20 Lysosomal lipid storage diseases. Cold Spring Harb Perspect Biol 2011 1.26
21 Development of an assay for the intermembrane transfer of cholesterol by Niemann-Pick C2 protein. Biol Chem 2007 1.25
22 Deficiency of epidermal protein-bound omega-hydroxyceramides in atopic dermatitis. J Invest Dermatol 2002 1.25
23 Purification and characterization of recombinant, human acid ceramidase. Catalytic reactions and interactions with acid sphingomyelinase. J Biol Chem 2003 1.24
24 Insertional mutagenesis of the mouse acid ceramidase gene leads to early embryonic lethality in homozygotes and progressive lipid storage disease in heterozygotes. Genomics 2002 1.22
25 Inhibition of glycosphingolipid biosynthesis reduces secretion of the beta-amyloid precursor protein and amyloid beta-peptide. J Biol Chem 2005 1.21
26 Interactions of acid sphingomyelinase and lipid bilayers in the presence of the tricyclic antidepressant desipramine. FEBS Lett 2004 1.19
27 The X-ray crystal structure of human beta-hexosaminidase B provides new insights into Sandhoff disease. J Mol Biol 2003 1.16
28 Sphingolipid storage affects autophagic metabolism of the amyloid precursor protein and promotes Abeta generation. J Neurosci 2011 1.16
29 Golgi-to-phagosome transport of acid sphingomyelinase and prosaposin is mediated by sortilin. J Cell Sci 2010 1.10
30 Hexosaminidase assays. Glycoconj J 2009 1.10
31 PAR2 absence completely rescues inflammation and ichthyosis caused by altered CAP1/Prss8 expression in mouse skin. Nat Commun 2011 1.09
32 Activation of Nrf2 in keratinocytes causes chloracne (MADISH)-like skin disease in mice. EMBO Mol Med 2014 1.08
33 Insulin receptor and lipid metabolism pathology in ataxin-2 knock-out mice. Hum Mol Genet 2008 1.07
34 Lipid-binding proteins in membrane digestion, antigen presentation, and antimicrobial defense. J Biol Chem 2005 1.06
35 The reverse activity of human acid ceramidase. J Biol Chem 2003 1.06
36 Crystal structures of human saposins C andD: implications for lipid recognition and membrane interactions. Structure 2008 1.04
37 Schlank, a member of the ceramide synthase family controls growth and body fat in Drosophila. EMBO J 2009 1.03
38 Characterization of human saposins by NMR spectroscopy. Biochemistry 2006 1.02
39 Role of endosomal membrane lipids and NPC2 in cholesterol transfer and membrane fusion. J Lipid Res 2010 1.01
40 Amplification and overexpression of prosaposin in prostate cancer. Genes Chromosomes Cancer 2005 0.99
41 Physiological substrates for human lysosomal beta -hexosaminidase S. J Biol Chem 2001 0.97
42 Separation and mass spectrometric characterization of covalently bound skin ceramides using LC/APCI-MS and Nano-ESI-MS/MS. J Chromatogr B Analyt Technol Biomed Life Sci 2007 0.97
43 Role for LAMP-2 in endosomal cholesterol transport. J Cell Mol Med 2011 0.97
44 Biological function of the cellular lipid BMP-BMP as a key activator for cholesterol sorting and membrane digestion. Neurochem Res 2010 0.94
45 Purified recombinant human prosaposin forms oligomers that bind procathepsin D and affect its autoactivation. Biochem J 2004 0.93
46 Sphingolipid metabolism during epidermal barrier development in mice. J Lipid Res 2002 0.93
47 Ablation of neuronal ceramide synthase 1 in mice decreases ganglioside levels and expression of myelin-associated glycoprotein in oligodendrocytes. J Biol Chem 2012 0.93
48 Human acid sphingomyelinase. Eur J Biochem 2003 0.93
49 A novel mass spectrometric assay for the cerebroside sulfate activator protein (saposin B) and arylsulfatase A. J Lipid Res 2005 0.92
50 Prion-induced activation of cholesterogenic gene expression by Srebp2 in neuronal cells. J Biol Chem 2009 0.92
51 Postnatal requirement of the epithelial sodium channel for maintenance of epidermal barrier function. J Biol Chem 2007 0.90
52 Severe subacute GM2 gangliosidosis caused by an apparently silent HEXA mutation (V324V) that results in aberrant splicing and reduced HEXA mRNA. Am J Med Genet A 2004 0.90
53 Saposin A mobilizes lipids from low cholesterol and high bis(monoacylglycerol)phosphate-containing membranes: patient variant Saposin A lacks lipid extraction capacity. J Biol Chem 2006 0.89
54 Phosphatidylinositol-3,5-Bisphosphate is a potent and selective inhibitor of acid sphingomyelinase. Biol Chem 2003 0.89
55 Conditional LoxP-flanked glucosylceramide synthase allele controlling glycosphingolipid synthesis. Genesis 2005 0.89
56 Saposin B mobilizes lipids from cholesterol-poor and bis(monoacylglycero)phosphate-rich membranes at acidic pH. Unglycosylated patient variant saposin B lacks lipid-extraction capacity. FEBS J 2007 0.89
57 Regulation of the NPC2 protein-mediated cholesterol trafficking by membrane lipids. J Neurochem 2011 0.88
58 Optimization of submerged keratinocyte cultures for the synthesis of barrier ceramides. Eur J Cell Biol 2007 0.88
59 CLN3 loss disturbs membrane microdomain properties and protein transport in brain endothelial cells. J Neurosci 2013 0.88
60 Characterization of two Turkish beta-hexosaminidase mutations causing Tay-Sachs disease. Brain Dev 2003 0.88
61 Loss of keratin 10 is accompanied by increased sebocyte proliferation and differentiation. Eur J Cell Biol 2004 0.88
62 Functional characterization of the postulated intramolecular sphingolipid activator protein domain of human acid sphingomyelinase. Biol Chem 2004 0.87
63 Photoaffinity labelling of the human GM2-activator protein. Mechanistic insight into ganglioside GM2 degradation. Eur J Biochem 2004 0.87
64 Synthesis of novel NBD-GM1 and NBD-GM2 for the transfer activity of GM2-activator protein by a FRET-based assay system. Glycobiology 2005 0.87
65 Normal phase liquid chromatography coupled to quadrupole time of flight atmospheric pressure chemical ionization mass spectrometry for separation, detection and mass spectrometric profiling of neutral sphingolipids and cholesterol. J Chromatogr B Analyt Technol Biomed Life Sci 2009 0.86
66 Cancer: Killing from the inside. Nature 2013 0.86
67 Expression of the GM2-activator protein in the methylotrophic yeast Pichia pastoris, purification, isotopic labeling, and biophysical characterization. Protein Expr Purif 2004 0.86
68 Procathepsin D interacts with prosaposin in cancer cells but its internalization is not mediated by LDL receptor-related protein. Exp Cell Res 2002 0.85
69 TCF/Lef1-mediated control of lipid metabolism regulates skin barrier function. J Invest Dermatol 2011 0.85
70 Saposin C stimulates growth and invasion, activates p42/44 and SAPK/JNK signaling pathways of MAPK and upregulates uPA/uPAR expression in prostate cancer and stromal cells. Asian J Androl 2005 0.85
71 Neural precursor cell cultures from GM2 gangliosidosis animal models recapitulate the biochemical and molecular hallmarks of the brain pathology. J Neurochem 2009 0.85
72 Expression of recombinant human GM2-activator protein in insect cells: purification and characterization by mass spectrometry. Protein Expr Purif 2003 0.84
73 Functional and genetic characterization of the non-lysosomal glucosylceramidase 2 as a modifier for Gaucher disease. Orphanet J Rare Dis 2013 0.84
74 Prosaptide TX14A stimulates growth, migration, and invasion and activates the Raf-MEK-ERK-RSK-Elk-1 signaling pathway in prostate cancer cells. Prostate 2004 0.84
75 Overexpression and mass spectrometry analysis of mature human acid ceramidase. Biol Chem 2007 0.84
76 The enzyme-binding region of human GM2-activator protein. FEBS J 2006 0.82
77 Phospholipid synthesis is decreased in neuronal tissue in a mouse model of Sandhoff disease. J Neurochem 2004 0.82
78 Nitric oxide regulates synthesis of gene products involved in keratinocyte differentiation and ceramide metabolism. Eur J Cell Biol 2004 0.80
79 An inducible mouse model of late onset Tay-Sachs disease. Neurobiol Dis 2002 0.80
80 Cis-4-methylsphingosine phosphate induces apoptosis in neuroblastoma cells by opposite effects on p38 and ERK mitogen-activated protein kinases. Biol Chem 2002 0.79
81 Mass spectrometric analysis of neutral sphingolipids: methods, applications, and limitations. Biochim Biophys Acta 2011 0.79
82 Saposin B-dependent reconstitution of arylsulfatase A activity in vitro and in cell culture models of metachromatic leukodystrophy. J Biol Chem 2009 0.79
83 Identification of ceramide binding proteins in neuronal cells: a critical point of view. Neurochem Res 2002 0.78
84 Crystallization and preliminary characterization of three different crystal forms of human saposin C heterologously expressed in Pichia pastoris. Acta Crystallogr Sect F Struct Biol Cryst Commun 2006 0.77
85 Loss of CB1 receptors leads to decreased cathepsin D levels and accelerated lipofuscin accumulation in the hippocampus. Mech Ageing Dev 2013 0.76
86 Site-specific cleavage--a model system for the identification of lipid-modified glutamate residues in proteins. Chembiochem 2005 0.76
87 A new point mutation (G412 to A) at the last nucleotide of exon 3 of hexosaminidase alpha-subunit gene affects splicing. Brain Dev 2003 0.76
88 Physiological relevance of sphingolipid activator proteins in cultured human fibroblasts. Biochimie 2003 0.76
89 Interaction of the GM2 activator protein with sulfated and sialylated glycosphingolipids. Methods Enzymol 2003 0.75
90 RNA interference: analyzing the function of glycoproteins and glycosylating proteins in mammalian cells. Methods Enzymol 2003 0.75
91 Detection and identification of Vav1 protein in primary cultured murine cerebellar neurons and in neuroblastoma cells (SH-SY5Y and Neuro-2a). Neurosci Lett 2003 0.75
92 Recombinant ganglioside GM2 synthase--expression in insect cells and enzyme assay. Methods Enzymol 2003 0.75