Published in Eur J Cell Biol on December 01, 2004
The epidermal barrier function is dependent on the serine protease CAP1/Prss8. J Cell Biol (2005) 2.36
PAR2 absence completely rescues inflammation and ichthyosis caused by altered CAP1/Prss8 expression in mouse skin. Nat Commun (2011) 1.09
Schlank, a member of the ceramide synthase family controls growth and body fat in Drosophila. EMBO J (2009) 1.03
c-MYC-induced sebaceous gland differentiation is controlled by an androgen receptor/p53 axis. Cell Rep (2013) 0.97
Functional and genetic characterization of the non-lysosomal glucosylceramidase 2 as a modifier for Gaucher disease. Orphanet J Rare Dis (2013) 0.84
Accumulation of glucosylceramide in the absence of the beta-glucosidase GBA2 alters cytoskeletal dynamics. PLoS Genet (2015) 0.77
Analysis of the expression pattern of involucrin in human scalp skin and hair follicles: hair cycle-associated alterations. Histochem Cell Biol (2012) 0.75
Direct observation of the nanoscale dynamics of membrane lipids in a living cell. Nature (2008) 6.38
New consensus nomenclature for mammalian keratins. J Cell Biol (2006) 3.27
Hormone-sensitive lipase deficiency in mice causes diglyceride accumulation in adipose tissue, muscle, and testis. J Biol Chem (2001) 3.10
Hsp70 stabilizes lysosomes and reverts Niemann-Pick disease-associated lysosomal pathology. Nature (2010) 2.62
Apoptotic vesicles crossprime CD8 T cells and protect against tuberculosis. Immunity (2006) 2.61
Enhanced insulin sensitivity in mice lacking ganglioside GM3. Proc Natl Acad Sci U S A (2003) 2.56
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
The epidermal barrier function is dependent on the serine protease CAP1/Prss8. J Cell Biol (2005) 2.36
Comprehensive analysis of keratin gene clusters in humans and rodents. Eur J Cell Biol (2004) 2.20
Combinatorial ganglioside biosynthesis. J Biol Chem (2002) 2.06
Sphingolipid metabolism diseases. Biochim Biophys Acta (2006) 2.01
Saposin C is required for lipid presentation by human CD1b. Nat Immunol (2004) 1.88
Loss-of-function mutations in the keratin 5 gene lead to Dowling-Degos disease. Am J Hum Genet (2006) 1.78
Loss of keratin 10 leads to mitogen-activated protein kinase (MAPK) activation, increased keratinocyte turnover, and decreased tumor formation in mice. J Invest Dermatol (2004) 1.78
Acid ceramidase overexpression prevents the inhibitory effects of saturated fatty acids on insulin signaling. J Biol Chem (2005) 1.63
Adult ceramide synthase 2 (CERS2)-deficient mice exhibit myelin sheath defects, cerebellar degeneration, and hepatocarcinomas. J Biol Chem (2009) 1.58
Lysosomal degradation of membrane lipids. FEBS Lett (2009) 1.50
Principles of lysosomal membrane degradation: Cellular topology and biochemistry of lysosomal lipid degradation. Biochim Biophys Acta (2008) 1.46
Systemic inflammation in glucocerebrosidase-deficient mice with minimal glucosylceramide storage. J Clin Invest (2002) 1.45
Interruption of ganglioside synthesis produces central nervous system degeneration and altered axon-glial interactions. Proc Natl Acad Sci U S A (2005) 1.43
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
Normal epidermal differentiation but impaired skin-barrier formation upon keratinocyte-restricted IKK1 ablation. Nat Cell Biol (2007) 1.40
Nrf2 links epidermal barrier function with antioxidant defense. EMBO Mol Med (2012) 1.35
Keratins regulate protein biosynthesis through localization of GLUT1 and -3 upstream of AMP kinase and Raptor. J Cell Biol (2009) 1.29
Functional complexity of intermediate filament cytoskeletons: from structure to assembly to gene ablation. Int Rev Cytol (2003) 1.28
Hyperproliferation, induction of c-Myc and 14-3-3sigma, but no cell fragility in keratin-10-null mice. J Cell Sci (2002) 1.28
Integrity and barrier function of the epidermis critically depend on glucosylceramide synthesis. J Biol Chem (2006) 1.27
Lysosomal lipid storage diseases. Cold Spring Harb Perspect Biol (2011) 1.26
Deficiency of epidermal protein-bound omega-hydroxyceramides in atopic dermatitis. J Invest Dermatol (2002) 1.25
Development of an assay for the intermembrane transfer of cholesterol by Niemann-Pick C2 protein. Biol Chem (2007) 1.25
Cytoskeleton in motion: the dynamics of keratin intermediate filaments in epithelia. J Cell Biol (2011) 1.25
Purification and characterization of recombinant, human acid ceramidase. Catalytic reactions and interactions with acid sphingomyelinase. J Biol Chem (2003) 1.24
Keratins significantly contribute to cell stiffness and impact invasive behavior. Proc Natl Acad Sci U S A (2013) 1.23
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
Inhibition of glycosphingolipid biosynthesis reduces secretion of the beta-amyloid precursor protein and amyloid beta-peptide. J Biol Chem (2005) 1.21
Interactions of acid sphingomyelinase and lipid bilayers in the presence of the tricyclic antidepressant desipramine. FEBS Lett (2004) 1.19
Keratin 1 maintains skin integrity and participates in an inflammatory network in skin through interleukin-18. J Cell Sci (2012) 1.18
Type II keratins precede type I keratins during early embryonic development. Eur J Cell Biol (2005) 1.18
The X-ray crystal structure of human beta-hexosaminidase B provides new insights into Sandhoff disease. J Mol Biol (2003) 1.16
Sphingolipid storage affects autophagic metabolism of the amyloid precursor protein and promotes Abeta generation. J Neurosci (2011) 1.16
Keratins mediate localization of hemidesmosomes and repress cell motility. J Invest Dermatol (2012) 1.15
Dominant cataract formation in association with a vimentin assembly disrupting mutation. Hum Mol Genet (2009) 1.12
Golgi-to-phagosome transport of acid sphingomyelinase and prosaposin is mediated by sortilin. J Cell Sci (2010) 1.10
Hexosaminidase assays. Glycoconj J (2009) 1.10
PAR2 absence completely rescues inflammation and ichthyosis caused by altered CAP1/Prss8 expression in mouse skin. Nat Commun (2011) 1.09
Keratins control intercellular adhesion involving PKC-α-mediated desmoplakin phosphorylation. J Cell Biol (2013) 1.09
Activation of Nrf2 in keratinocytes causes chloracne (MADISH)-like skin disease in mice. EMBO Mol Med (2014) 1.08
Insulin receptor and lipid metabolism pathology in ataxin-2 knock-out mice. Hum Mol Genet (2008) 1.07
Lipid-binding proteins in membrane digestion, antigen presentation, and antimicrobial defense. J Biol Chem (2005) 1.06
The reverse activity of human acid ceramidase. J Biol Chem (2003) 1.06
Crystal structures of human saposins C andD: implications for lipid recognition and membrane interactions. Structure (2008) 1.04
Mechanisms of fibroblast cell therapy for dystrophic epidermolysis bullosa: high stability of collagen VII favors long-term skin integrity. Mol Ther (2009) 1.03
Keratins as the main component for the mechanical integrity of keratinocytes. Proc Natl Acad Sci U S A (2013) 1.03
Schlank, a member of the ceramide synthase family controls growth and body fat in Drosophila. EMBO J (2009) 1.03
Genetic background effects of keratin 8 and 18 in a DDC-induced hepatotoxicity and Mallory-Denk body formation mouse model. Lab Invest (2012) 1.02
Characterization of human saposins by NMR spectroscopy. Biochemistry (2006) 1.02
Role of endosomal membrane lipids and NPC2 in cholesterol transfer and membrane fusion. J Lipid Res (2010) 1.01
Amplification and overexpression of prosaposin in prostate cancer. Genes Chromosomes Cancer (2005) 0.99
Mutations in vimentin disrupt the cytoskeleton in fibroblasts and delay execution of apoptosis. Eur J Cell Biol (2005) 0.98
Induction of inflammatory cytokines by a keratin mutation and their repression by a small molecule in a mouse model for EBS. J Invest Dermatol (2007) 0.98
Physiological substrates for human lysosomal beta -hexosaminidase S. J Biol Chem (2001) 0.97
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
Role for LAMP-2 in endosomal cholesterol transport. J Cell Mol Med (2011) 0.97
A mutation of keratin 18 within the coil 1A consensus motif causes widespread keratin aggregation but cell type-restricted lethality in mice. Exp Cell Res (2007) 0.96
Keratin transgenic and knockout mice: functional analysis and validation of disease-causing mutations. Methods Mol Biol (2007) 0.94
Biological function of the cellular lipid BMP-BMP as a key activator for cholesterol sorting and membrane digestion. Neurochem Res (2010) 0.94
Purified recombinant human prosaposin forms oligomers that bind procathepsin D and affect its autoactivation. Biochem J (2004) 0.93
Sphingolipid metabolism during epidermal barrier development in mice. J Lipid Res (2002) 0.93
Human acid sphingomyelinase. Eur J Biochem (2003) 0.93
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
A novel mass spectrometric assay for the cerebroside sulfate activator protein (saposin B) and arylsulfatase A. J Lipid Res (2005) 0.92
Prion-induced activation of cholesterogenic gene expression by Srebp2 in neuronal cells. J Biol Chem (2009) 0.92
Defolliculated (dfl): a dominant mouse mutation leading to poor sebaceous gland differentiation and total elimination of pelage follicles. J Invest Dermatol (2002) 0.91
Postnatal requirement of the epithelial sodium channel for maintenance of epidermal barrier function. J Biol Chem (2007) 0.90
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
Cytokines as genetic modifiers in K5-/- mice and in human epidermolysis bullosa simplex. Hum Mutat (2009) 0.89
Conditional LoxP-flanked glucosylceramide synthase allele controlling glycosphingolipid synthesis. Genesis (2005) 0.89
Rescue of atypical protein kinase C in epithelia by the cytoskeleton and Hsp70 family chaperones. J Cell Sci (2009) 0.89
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
Phosphatidylinositol-3,5-Bisphosphate is a potent and selective inhibitor of acid sphingomyelinase. Biol Chem (2003) 0.89
Regulation of the NPC2 protein-mediated cholesterol trafficking by membrane lipids. J Neurochem (2011) 0.88
Optimization of submerged keratinocyte cultures for the synthesis of barrier ceramides. Eur J Cell Biol (2007) 0.88
Characterization of two Turkish beta-hexosaminidase mutations causing Tay-Sachs disease. Brain Dev (2003) 0.88
Identification of a keratin-associated protein with a putative role in vesicle transport. Eur J Cell Biol (2007) 0.88
A dominant vimentin mutant upregulates Hsp70 and the activity of the ubiquitin-proteasome system, and causes posterior cataracts in transgenic mice. J Cell Sci (2008) 0.88
CLN3 loss disturbs membrane microdomain properties and protein transport in brain endothelial cells. J Neurosci (2013) 0.88