Published in J Mol Biol on May 02, 2003
High-throughput screening for human lysosomal beta-N-Acetyl hexosaminidase inhibitors acting as pharmacological chaperones. Chem Biol (2007) 1.85
Hexosaminidase assays. Glycoconj J (2009) 1.10
Structural determinants of an insect beta-N-Acetyl-D-hexosaminidase specialized as a chitinolytic enzyme. J Biol Chem (2010) 0.98
Phylogenetic analyses suggest multiple changes of substrate specificity within the glycosyl hydrolase 20 family. BMC Evol Biol (2008) 0.97
Structural basis for the substrate specificity of a novel β-N-acetylhexosaminidase StrH protein from Streptococcus pneumoniae R6. J Biol Chem (2011) 0.96
A sensitive fluorescence-based assay for monitoring GM2 ganglioside hydrolysis in live patient cells and their lysates. Glycobiology (2009) 0.92
Ganglioside biochemistry. ISRN Biochem (2012) 0.89
Characterization of the mutant β-subunit of β-hexosaminidase for dimer formation responsible for the adult form of Sandhoff disease with the motor neuron disease phenotype. J Biochem (2012) 0.84
Production of recombinant beta-hexosaminidase A, a potential enzyme for replacement therapy for Tay-Sachs and Sandhoff diseases, in the methylotrophic yeast Ogataea minuta. Appl Environ Microbiol (2007) 0.84
Structure of the dimeric N-glycosylated form of fungal beta-N-acetylhexosaminidase revealed by computer modeling, vibrational spectroscopy, and biochemical studies. BMC Struct Biol (2007) 0.82
Crystal structures of a glycoside hydrolase family 20 lacto-N-biosidase from Bifidobacterium bifidum. J Biol Chem (2013) 0.82
Crystal structure of β-hexosaminidase B in complex with pyrimethamine, a potential pharmacological chaperone. J Med Chem (2011) 0.81
Assessing the severity of the small inframe deletion mutation in the alpha-subunit of beta-hexosaminidase A found in the Turkish population by reproducing it in the more stable beta-subunit. J Inherit Metab Dis (2004) 0.81
In cellulo examination of a beta-alpha hybrid construct of beta-hexosaminidase A subunits, reported to interact with the GM2 activator protein and hydrolyze GM2 ganglioside. PLoS One (2013) 0.80
Molecular and biochemical characterization of a novel β-N-acetyl-D-hexosaminidase with broad substrate-spectrum from the Aisan corn borer, Ostrinia furnacalis. Int J Biol Sci (2012) 0.79
Construction of a hybrid β-hexosaminidase subunit capable of forming stable homodimers that hydrolyze GM2 ganglioside in vivo. Mol Ther Methods Clin Dev (2016) 0.77
Probing the Catalytic Mechanism of Vibrio harveyi GH20 β-N-Acetylglucosaminidase by Chemical Rescue. PLoS One (2016) 0.77
Incidence and carrier frequency of Sandhoff disease in Saskatchewan determined using a novel substrate with detection by tandem mass spectrometry and molecular genetic analysis. Mol Genet Metab (2014) 0.76
Expression, purification, crystallization and preliminary crystallographic analysis of a GH20 β-N-acetylglucosaminidase from the marine bacterium Vibrio harveyi. Acta Crystallogr F Struct Biol Commun (2015) 0.75
Molecular phylogeny and predicted 3D structure of plant beta-D-N-acetylhexosaminidase. ScientificWorldJournal (2014) 0.75
Computational study of β-N-acetylhexosaminidase from Talaromyces flavus, a glycosidase with high substrate flexibility. BMC Bioinformatics (2015) 0.75
Tay-Sachs disease mutations in HEXA target the α chain of hexosaminidase A to endoplasmic reticulum-associated degradation. Mol Biol Cell (2016) 0.75
A crystal structure-guided rational design switching non-carbohydrate inhibitors' specificity between two β-GlcNAcase homologs. Sci Rep (2014) 0.75
Towards complete cofactor arrangement in the 3.0 A resolution structure of photosystem II. Nature (2005) 6.40
Direct observation of the nanoscale dynamics of membrane lipids in a living cell. Nature (2008) 6.38
Cyanobacterial photosystem II at 2.9-A resolution and the role of quinones, lipids, channels and chloride. Nat Struct Mol Biol (2009) 4.29
Where water is oxidized to dioxygen: structure of the photosynthetic Mn4Ca cluster. Science (2006) 3.29
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
Role of the clathrin terminal domain in regulating coated pit dynamics revealed by small molecule inhibition. Cell (2011) 2.56
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
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
SNAREs prefer liquid-disordered over "raft" (liquid-ordered) domains when reconstituted into giant unilamellar vesicles. J Biol Chem (2004) 1.65
Crystal structure of the plasmid maintenance system epsilon/zeta: functional mechanism of toxin zeta and inactivation by epsilon 2 zeta 2 complex formation. Proc Natl Acad Sci U S A (2003) 1.64
Acid ceramidase overexpression prevents the inhibitory effects of saturated fatty acids on insulin signaling. J Biol Chem (2005) 1.63
Streptococcus pyogenes pSM19035 requires dynamic assembly of ATP-bound ParA and ParB on parS DNA during plasmid segregation. Nucleic Acids Res (2008) 1.62
Adult ceramide synthase 2 (CERS2)-deficient mice exhibit myelin sheath defects, cerebellar degeneration, and hepatocarcinomas. J Biol Chem (2009) 1.58
Molecular basis for SH3 domain regulation of F-BAR-mediated membrane deformation. Proc Natl Acad Sci U S A (2010) 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
Quinpramine is a novel compound effective in ameliorating brain autoimmune disease. Exp Neurol (2008) 1.44
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
Regulation of synaptic inhibition by phospho-dependent binding of the AP2 complex to a YECL motif in the GABAA receptor gamma2 subunit. Proc Natl Acad Sci U S A (2008) 1.36
Nrf2 links epidermal barrier function with antioxidant defense. EMBO Mol Med (2012) 1.35
Dual, HLA-B27 subtype-dependent conformation of a self-peptide. J Exp Med (2004) 1.31
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
In vitro and in vivo stability of the epsilon2zeta2 protein complex of the broad host-range Streptococcus pyogenes pSM19035 addiction system. Biol Chem (2002) 1.25
Purification and characterization of recombinant, human acid ceramidase. Catalytic reactions and interactions with acid sphingomyelinase. J Biol Chem (2003) 1.24
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
Inhibition of glycosphingolipid biosynthesis reduces secretion of the beta-amyloid precursor protein and amyloid beta-peptide. J Biol Chem (2005) 1.21
Structures of omega repressors bound to direct and inverted DNA repeats explain modulation of transcription. Nucleic Acids Res (2006) 1.21
Theory of optical spectra of photosystem II reaction centers: location of the triplet state and the identity of the primary electron donor. Biophys J (2004) 1.20
Lipids in photosystem II: interactions with protein and cofactors. Biochim Biophys Acta (2007) 1.20
Interactions of acid sphingomyelinase and lipid bilayers in the presence of the tricyclic antidepressant desipramine. FEBS Lett (2004) 1.19
Sphingolipid storage affects autophagic metabolism of the amyloid precursor protein and promotes Abeta generation. J Neurosci (2011) 1.16
How photosynthetic reaction centers control oxidation power in chlorophyll pairs P680, P700, and P870. Proc Natl Acad Sci U S A (2006) 1.16
HLA-B27 subtypes differentially associated with disease exhibit subtle structural alterations. J Biol Chem (2002) 1.13
Conjugated bile acid hydrolase is a tetrameric N-terminal thiol hydrolase with specific recognition of its cholyl but not of its tauryl product. Biochemistry (2005) 1.12
Crystal structure of CD26/dipeptidyl-peptidase IV in complex with adenosine deaminase reveals a highly amphiphilic interface. J Biol Chem (2004) 1.11
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
Structures of human N-Acetylglucosamine kinase in two complexes with N-Acetylglucosamine and with ADP/glucose: insights into substrate specificity and regulation. J Mol Biol (2006) 1.09
PAR2 absence completely rescues inflammation and ichthyosis caused by altered CAP1/Prss8 expression in mouse skin. Nat Commun (2011) 1.09
Activation of Nrf2 in keratinocytes causes chloracne (MADISH)-like skin disease in mice. EMBO Mol Med (2014) 1.08
Allele-dependent similarity between viral and self-peptide presentation by HLA-B27 subtypes. J Biol Chem (2004) 1.07
Insulin receptor and lipid metabolism pathology in ataxin-2 knock-out mice. Hum Mol Genet (2008) 1.07
Recognition of DNA by omega protein from the broad-host range Streptococcus pyogenes plasmid pSM19035: analysis of binding to operator DNA with one to four heptad repeats. Nucleic Acids Res (2004) 1.06
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 structure of Homo sapiens protein hp14.5. Proteins (2004) 1.05
Crystal structures of human saposins C andD: implications for lipid recognition and membrane interactions. Structure (2008) 1.04
Energetics of a possible proton exit pathway for water oxidation in photosystem II. Biochemistry (2006) 1.03
Schlank, a member of the ceramide synthase family controls growth and body fat in Drosophila. EMBO J (2009) 1.03
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
Thermodynamic and structural equivalence of two HLA-B27 subtypes complexed with a self-peptide. J Mol Biol (2005) 1.00
Open and closed conformation of the E. coli purine nucleoside phosphorylase active center and implications for the catalytic mechanism. J Mol Biol (2002) 0.99
Amplification and overexpression of prosaposin in prostate cancer. Genes Chromosomes Cancer (2005) 0.99
Structure and function of primase RepB' encoded by broad-host-range plasmid RSF1010 that replicates exclusively in leading-strand mode. Proc Natl Acad Sci U S A (2009) 0.99
Thermodynamic and structural analysis of peptide- and allele-dependent properties of two HLA-B27 subtypes exhibiting differential disease association. J Biol Chem (2003) 0.98
Redox potentials of chlorophylls in the photosystem II reaction center. Biochemistry (2005) 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
Physiological substrates for human lysosomal beta -hexosaminidase S. J Biol Chem (2001) 0.97
Role for LAMP-2 in endosomal cholesterol transport. J Cell Mol Med (2011) 0.97
A chimeric ligand approach leading to potent antiprion active acridine derivatives: design, synthesis, and biological investigations. J Med Chem (2006) 0.96
Probing the accessibility of the Mn(4)Ca cluster in photosystem II: channels calculation, noble gas derivatization, and cocrystallization with DMSO. Structure (2009) 0.95
Recent progress in the crystallographic studies of photosystem II. Chemphyschem (2010) 0.95
Biological function of the cellular lipid BMP-BMP as a key activator for cholesterol sorting and membrane digestion. Neurochem Res (2010) 0.94
Regulation of synaptic vesicle recycling by complex formation between intersectin 1 and the clathrin adaptor complex AP2. Proc Natl Acad Sci U S A (2010) 0.94
Purified recombinant human prosaposin forms oligomers that bind procathepsin D and affect its autoactivation. Biochem J (2004) 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
Crystal structure of the antitoxin-toxin protein complex RelB-RelE from Methanococcus jannaschii. J Mol Biol (2009) 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
Conformational dimorphism of self-peptides and molecular mimicry in a disease-associated HLA-B27 subtype. J Biol Chem (2005) 0.92
Prion-induced activation of cholesterogenic gene expression by Srebp2 in neuronal cells. J Biol Chem (2009) 0.92
Cyanobacterial photosystem II at 3.2 A resolution - the plastoquinone binding pockets. Photosynth Res (2005) 0.92
A novel mass spectrometric assay for the cerebroside sulfate activator protein (saposin B) and arylsulfatase A. J Lipid Res (2005) 0.92
Three-dimensional structure of N-terminal domain of DnaB helicase and helicase-primase interactions in Helicobacter pylori. PLoS One (2009) 0.92
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
Tuning electron transfer by ester-group of chlorophylls in bacterial photosynthetic reaction center. FEBS Lett (2005) 0.90
Conditional LoxP-flanked glucosylceramide synthase allele controlling glycosphingolipid synthesis. Genesis (2005) 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