Gene Symbol: CTSA
Description: cathepsin A
Alias: GLB2, GSL, NGBE, PPCA, PPGB, lysosomal protective protein, beta-galactosidase 2, beta-galactosidase protective protein, carboxypeptidase C, carboxypeptidase Y-like kininase, carboxypeptidase-L, deamidase, lysosomal carboxypeptidase A, protective protein cathepsin A, urinary kininase
Species: human
Products:     CTSA

Top Publications

  1. Zhou X, Galjart N, Willemsen R, Gillemans N, Galjaard H, d Azzo A. A mutation in a mild form of galactosialidosis impairs dimerization of the protective protein and renders it unstable. EMBO J. 1991;10:4041-8 pubmed
    ..These results clarify the mechanism underlying the combined deficiency in these patients, and give new insight into the structure-function relationship of the wild type protein. ..
  2. Van der Spoel A, Bonten E, d Azzo A. Transport of human lysosomal neuraminidase to mature lysosomes requires protective protein/cathepsin A. EMBO J. 1998;17:1588-97 pubmed
    ..in the neuraminidase gene, and galactosialidosis, in which a primary defect of protective protein/cathepsin A (PPCA) leads to a combined deficiency of neuraminidase and beta-D-galactosidase...
  3. Groener J, Maaswinkel Mooy P, Smit V, van der Hoeven M, Bakker J, Campos Y, et al. New mutations in two Dutch patients with early infantile galactosialidosis. Mol Genet Metab. 2003;78:222-8 pubmed
    ..beta-galactosidase and neuraminidase as a result of a primary defect in the protective protein/cathepsin A (PPCA). We report the first 2 Dutch cases of early infantile galactosialidosis, both presenting with neonatal ascites...
  4. Galjart N, Morreau H, Willemsen R, Gillemans N, Bonten E, d Azzo A. Human lysosomal protective protein has cathepsin A-like activity distinct from its protective function. J Biol Chem. 1991;266:14754-62 pubmed
    ..These findings indicate that the catalytic activity and protective function of the protective protein are distinct. ..
  5. Shimmoto M, Fukuhara Y, Itoh K, Oshima A, Sakuraba H, Suzuki Y. Protective protein gene mutations in galactosialidosis. J Clin Invest. 1993;91:2393-8 pubmed
    ..Pulse-chase analysis detected a small amount of the mature form, as well as the precursor, in the cells transfected with the Y249N cDNA. Only precursor proteins were detected, mature proteins not appearing for the other mutant cDNAs. ..
  6. Pshezhetsky A, Potier M. Association of N-acetylgalactosamine-6-sulfate sulfatase with the multienzyme lysosomal complex of beta-galactosidase, cathepsin A, and neuraminidase. Possible implication for intralysosomal catabolism of keratan sulfate. J Biol Chem. 1996;271:28359-65 pubmed
    ..We suggest that the 1.27-MDa complex of lysosomal hydrolases is essential for KS catabolism and that the disruption of this complex may be responsible for the KS accumulation in beta-galactosidosis and galactosialidosis patients. ..
  7. Zhou X, Van der Spoel A, Rottier R, Hale G, Willemsen R, Berry G, et al. Molecular and biochemical analysis of protective protein/cathepsin A mutations: correlation with clinical severity in galactosialidosis. Hum Mol Genet. 1996;5:1977-87 pubmed
    Mutations in the gene encoding lysosomal protective protein/cathepsin A (PPCA) are the cause of the lysosomal disorder galactosialidosis (GS)...
  8. Cuervo A, Mann L, Bonten E, D AZZO A, Dice J. Cathepsin A regulates chaperone-mediated autophagy through cleavage of the lysosomal receptor. EMBO J. 2003;22:47-59 pubmed
    Protective protein/cathepsin A (PPCA) has a serine carboxypeptidase activity of unknown physiological function...
  9. Takiguchi K, Itoh K, Shimmoto M, Ozand P, Doi H, Sakuraba H. Structural and functional study of K453E mutant protective protein/cathepsin A causing the late infantile form of galactosialidosis. J Hum Genet. 2000;45:200-6 pubmed
    ..basis of the late infantile form of galactosialidosis, we characterized a defective protective protein/cathepsin A (PPCA) gene product with the K453E mutation newly found in an Arabic patient with this disease...

More Information

Publications125 found, 100 shown here

  1. Kaissi A, Chehida F, Grill F, Ganger R, Kircher S. Turning the backbone into an ankylosed concrete-like structure: Case report. Medicine (Baltimore). 2018;97:e0278 pubmed publisher
    ..A homozygous for CTSA gene mutation, the gene associated with Galactosialidosis was identified via whole exome sequencing (Next-..
  2. Sun Y, Ran H, Zamzow M, Kitatani K, Skelton M, Williams M, et al. Specific saposin C deficiency: CNS impairment and acid beta-glucosidase effects in the mouse. Hum Mol Genet. 2010;19:634-47 pubmed publisher
    ..These results support the view that saposin C has multiple roles in glycosphingolipid (GSL) catabolism as well as a prominent function in CNS and axonal integrity independent of its role as an optimizer/..
  3. Iwabuchi K. Gangliosides in the Immune System: Role of Glycosphingolipids and Glycosphingolipid-Enriched Lipid Rafts in Immunological Functions. Methods Mol Biol. 2018;1804:83-95 pubmed publisher
    ..Additional hydrophilic cis interactions among GSL headgroups have been found to promote their lateral associations with surrounding lipid and protein membrane ..
  4. Bhat V, Bernhart E, Plastira I, Fan K, Ghaffari Tabrizi Wizsy N, Wadsack C, et al. Pharmacological Inhibition of Serine Palmitoyl Transferase and Sphingosine Kinase-1/-2 Inhibits Merkel Cell Carcinoma Cell Proliferation. J Invest Dermatol. 2019;139:807-817 pubmed publisher
    ..Our data suggest that pharmacological inhibition of sphingolipid synthesis could represent a potential therapeutic approach in Merkel cell carcinoma. ..
  5. Legros N, Pohlentz G, Steil D, Kouzel I, Liashkovich I, Mellmann A, et al. Membrane assembly of Shiga toxin glycosphingolipid receptors and toxin refractiveness of MDCK II epithelial cells. J Lipid Res. 2018;59:1383-1401 pubmed publisher
    ..Globotriaosylceramide and globotetraosylceramide, known as receptors for Stx1a, Stx2a, and Stx2e, and Forssman GSL as a specific receptor for Stx2e, were found to cooccur with SM and cholesterol in DRMs of MDCK II cells, which was ..
  6. Subathra M, Korrapati M, Howell L, Arthur J, Shayman J, Schnellmann R, et al. Kidney glycosphingolipids are elevated early in diabetic nephropathy and mediate hypertrophy of mesangial cells. Am J Physiol Renal Physiol. 2015;309:F204-15 pubmed publisher
    ..Importantly, urinary GSL levels were higher in patients with DN compared with healthy control subjects, implicating a role for these lipids ..
  7. Steil D, Bonse R, Meisen I, Pohlentz G, Vallejo G, Karch H, et al. A Topographical Atlas of Shiga Toxin 2e Receptor Distribution in the Tissues of Weaned Piglets. Toxins (Basel). 2016;8: pubmed
    ..All probed tissues harbored GSL receptors, ranging from high (category I) over moderate (category II) to low content (category III)...
  8. Annunziata I, Sano R, D AZZO A. Mitochondria-associated ER membranes (MAMs) and lysosomal storage diseases. Cell Death Dis. 2018;9:328 pubmed publisher
    ..We will focus on the lipid components of the MAMs, and highlight how failure to digest or process the sialylated GSL, GM1 ganglioside, in lysosomes alters the lipid conformation and functional properties of the MAMs and leads to ..
  9. Dou L, Wang S, Sun L, Huang X, Zhang Y, Shen T, et al. Mir-338-3p Mediates Tnf-A-Induced Hepatic Insulin Resistance by Targeting PP4r1 to Regulate PP4 Expression. Cell Physiol Biochem. 2017;41:2419-2431 pubmed publisher
    ..research showed that the down-regulated miR-338-3p resulted in the impaired AKT/ glycogen synthase kinase 3 beta (GSl·Gβ) signalling pathway and glycogen synthesis...
  10. Klein R, Rosa C, Colares E, Robaldo R, Martinez P, Bianchini A. Antioxidant defense system and oxidative status in Antarctic fishes: The sluggish rockcod Notothenia coriiceps versus the active marbled notothen Notothenia rossii. J Therm Biol. 2017;68:119-127 pubmed publisher
    ..Brain and gill antioxidant capacity against peroxyl radicals (ACAP); gill enzyme [glutamate-cysteine ligase (GSL), superoxide dismutase (SOD) and catalase (CAT)] activity; liver GCL and SOD activity; and white muscle CAT ..
  11. Yadgary L, Wong E, Uni Z. Temporal transcriptome analysis of the chicken embryo yolk sac. BMC Genomics. 2014;15:690 pubmed publisher
    ..Several lysosomal genes (CTSA, PSAP, NPC2) and apolipoproteins genes (apoA1, A2, B, C3) were among the highest expressed, reflecting the ..
  12. Meschiari C, Jung M, Iyer R, Yabluchanskiy A, Toba H, Garrett M, et al. Macrophage overexpression of matrix metalloproteinase-9 in aged mice improves diastolic physiology and cardiac wound healing following myocardial infarction. Am J Physiol Heart Circ Physiol. 2017;:ajpheart.00453.2017 pubmed publisher
    ..05), consistent with increased diastolic volumes. Vessel numbers (by GSL-I lectin staining) were decreased in the TG compared to WT at both D0 and D7 post-MI (P<0.05 for both)...
  13. Sam S, Scoccia B, Yalamanchi S, Mazzone T. Metabolic dysfunction in obese Hispanic women with polycystic ovary syndrome. Hum Reprod. 2015;30:1358-64 pubmed publisher
    ..This project was supported by the following National Institutes of Health grants: K23 DK080988-01A1 to S.S. and UL1RR029879 to CTSA at University of Illinois. None of the authors report any conflict of interests.
  14. Kopitz J. Lipid glycosylation: a primer for histochemists and cell biologists. Histochem Cell Biol. 2017;147:175-198 pubmed publisher
    ..Besides their contribution to normal cell behavior, GSL expression patterns also influence disease processes by inducing cellular malfunctions when aberrant, as ..
  15. Sandhoff R, Schulze H, Sandhoff K. Ganglioside Metabolism in Health and Disease. Prog Mol Biol Transl Sci. 2018;156:1-62 pubmed publisher
    ..Defects in GSL metabolism cause fatal human diseases...
  16. Scandolera A, Odoul L, Salesse S, Guillot A, Blaise S, Kawecki C, et al. The Elastin Receptor Complex: A Unique Matricellular Receptor with High Anti-tumoral Potential. Front Pharmacol. 2016;7:32 pubmed publisher
    ..a peripheral subunit, named elastin binding protein (EBP), associated to the protective protein/cathepsin A (PPCA). The latter is bound to a membrane-associated protein called Neuraminidase-1 (Neu-1)...
  17. Haznedaroglu I, Malkan U. Local bone marrow renin-angiotensin system in the genesis of leukemia and other malignancies. Eur Rev Med Pharmacol Sci. 2016;20:4089-4111 pubmed
    ..Most of the RAS molecules including ACE, ACE2, AGT, AGTR1, AGTR2, AKR1C4, AKR1D1, ANPEP, ATP6AP2, CMA1, CPA3, CTSA, CTSD, CTSG, CYP11A1, CYP11B1, CYP11B2, CYP17A1, CYP21A2, DPP3, EGFR, ENPEP, GPER, HSD11B1, HSD11B2, IGF2R, KLK1, ..
  18. Soundararajan P, Kim J. Anti-Carcinogenic Glucosinolates in Cruciferous Vegetables and Their Antagonistic Effects on Prevention of Cancers. Molecules. 2018;23: pubmed publisher
    Glucosinolates (GSL) are naturally occurring β-d-thioglucosides found across the cruciferous vegetables. Core structure formation and side-chain modifications lead to the synthesis of more than 200 types of GSLs in Brassicaceae...
  19. Davidson C, Ali N, Micsenyi M, Stephney G, Renault S, Dobrenis K, et al. Chronic cyclodextrin treatment of murine Niemann-Pick C disease ameliorates neuronal cholesterol and glycosphingolipid storage and disease progression. PLoS ONE. 2009;4:e6951 pubmed publisher
    ..at either P7 or P21 and continuing every other day delayed clinical onset, reduced intraneuronal cholesterol and GSL storage as well as free sphingosine accumulation, reduced markers of neurodegeneration, and led to longer survival ..
  20. Gizaw S, Ohashi T, Tanaka M, Hinou H, Nishimura S. Glycoblotting method allows for rapid and efficient glycome profiling of human Alzheimer's disease brain, serum and cerebrospinal fluid towards potential biomarker discovery. Biochim Biophys Acta. 2016;1860:1716-27 pubmed publisher
    ..Alteration of the expression levels of major N- and GSL-glycans in human brain tissues, serum and CSF of AD patients can be monitored quantitatively by means of the ..
  21. Lester C, Mott D, Chui M. The Influence of a Community Pharmacy Automatic Prescription Refill Program on Medicare Part D Adherence Metrics. J Manag Care Spec Pharm. 2016;22:801-7 pubmed publisher
    ..The project described was supported by the Clinical and Translational Science Award (CTSA) program through the NIH National Center for Advancing Translational Sciences (NCATS), grant UL1TR000427...
  22. Cinotti R, Delater A, Fortuit C, Roquilly A, Mahé P, Demeure dit latte D, et al. Speckle-Tracking analysis of left ventricular systolic function in the intensive care unit. Anaesthesiol Intensive Ther. 2015;47:482-6 pubmed publisher
    ..In experimental model and human studies in septic shock, speckle-tracking analysis suggests that GSL is impaired along with preserved left ventricular ejection fraction...
  23. Shishido F, Uemura S, Kashimura M, Inokuchi J. Identification of a new B4GalNAcT1 (GM2/GD2/GA2 synthase) isoform, and regulation of enzyme stability and intracellular transport by arginine-based motif. Biochim Biophys Acta Biomembr. 2017;1859:2001-2011 pubmed publisher
    ..Disruption of GSL homeostasis is the cause of numerous diseases...
  24. Legros N, Dusny S, Humpf H, Pohlentz G, Karch H, Müthing J. Shiga toxin glycosphingolipid receptors and their lipid membrane ensemble in primary human blood-brain barrier endothelial cells. Glycobiology. 2017;27:99-109 pubmed publisher
    ..and the B-pentamers of the two major Stx subtypes Stx1a and Stx2a preferentially bind to the glycosphingolipid (GSL) globotriaosylceramide (Gb3Cer) expressed by human endothelial cells...
  25. Klokk T, Kavaliauskiene S, Sandvig K. Cross-linking of glycosphingolipids at the plasma membrane: consequences for intracellular signaling and traffic. Cell Mol Life Sci. 2016;73:1301-16 pubmed publisher
    ..Here, we demonstrate that Shiga and cholera toxins, which both possess multivalent GSL-binding capacity, induce dissociation of the cytosolic cPLA2α-AnxA1 complex in HeLa and HMEC-1 cells...
  26. Sharma D, Brown J, Choudhury A, Peterson T, Holicky E, Marks D, et al. Selective stimulation of caveolar endocytosis by glycosphingolipids and cholesterol. Mol Biol Cell. 2004;15:3114-22 pubmed
    ..Stimulation of caveolar endocytosis did not occur using ceramide or phosphatidylcholine and was not due to GSL degradation because similar results were obtained using a nonhydrolyzable GSL analog...
  27. Gadola S, Silk J, Jeans A, Illarionov P, Salio M, Besra G, et al. Impaired selection of invariant natural killer T cells in diverse mouse models of glycosphingolipid lysosomal storage diseases. J Exp Med. 2006;203:2293-303 pubmed
    ..The percentages and frequency of iNKT cells were reduced in multiple mouse models of lysosomal GSL storage disease, irrespective of the specific genetic defect or lipid species stored...
  28. Boudewyn L, Sikora J, Kuchar L, Ledvinova J, Grishchuk Y, Wang S, et al. N-butyldeoxynojirimycin delays motor deficits, cerebellar microgliosis, and Purkinje cell loss in a mouse model of mucolipidosis type IV. Neurobiol Dis. 2017;105:257-270 pubmed publisher
    ..Quantitative mass spectrometry analyses provided new data on the GSL profiles of murine MLIV brain tissue and showed that miglustat partially restored the wild type profile of white ..
  29. Binnington B, Nguyen L, Kamani M, Hossain D, Marks D, Budani M, et al. Inhibition of Rab prenylation by statins induces cellular glycosphingolipid remodeling. Glycobiology. 2016;26:166-80 pubmed publisher
    ..pyrophosphate (GGPP), a downstream product of HMG Co-A reductase, reversed statin-induced glycosyltransferase and GSL elevation...
  30. Furukawa K, Ohmi Y, Ohkawa Y, Tajima O, Furukawa K. Glycosphingolipids in the regulation of the nervous system. Adv Neurobiol. 2014;9:307-20 pubmed publisher
    ..may reflect an abnormal structure and a resultant altered function of lipid rafts caused by alterations in their GSL composition...
  31. Fu Q, Yang N, Gao C, Tian M, Zhou S, Mu X, et al. Characterization, expression signatures and microbial binding analysis of cathepsin A in turbot, Scophthalmus maximus L.(SmCTSA). Fish Shellfish Immunol. 2018;81:21-28 pubmed publisher
    ..This study provides valuable data for understanding the roles of ctsa in the host defense against bacterial infections.
  32. Vieira D, Thur K, Sultana S, Priestman D, van der Spoel A. Verification and refinement of cellular glycosphingolipid profiles using HPLC. Biochem Cell Biol. 2015;93:581-6 pubmed publisher
    ..In spite of their biological relevance, GSL profiles of commonly used cell lines have been analyzed to different degrees...
  33. Lichtblau Y, Zimmermann K, Haldemann B, Lenze D, Hummel M, Leser U. Comparative assessment of differential network analysis methods. Brief Bioinform. 2017;18:837-850 pubmed publisher
    ..Next, we assess the results of applying DiNA algorithms on these data sets using a gold standard list (GSL)...
  34. Lee H, Wu Q, Li H, Bae G, Kim A, Ryu J. A sesquiterpene lactone from Siegesbeckia glabrescens suppresses Hedgehog/Gli-mediated transcription in pancreatic cancer cells. Oncol Lett. 2016;12:2912-2917 pubmed
    ..A germacranolide sesquiterpene lactone (GSL) was isolated from Siegesbeckia glabrescens as an inhibitor of Gli-mediated transcription...
  35. Cottler L, Striley C, Elliott A, Zulich A, Kwiatkowski E, Nelson D. Pragmatic trial of a Study Navigator Model (NAU) vs. Ambassador Model (N+) to increase enrollment to health research among community members who use illicit drugs. Drug Alcohol Depend. 2017;175:146-150 pubmed publisher
    ..Towards Drug Users study (Navigation Study) were recruited through a Clinical and Translational Science Award (CTSA) community engagement model...
  36. Kubota K, Inaba S, Nakano R, Watanabe M, Sakurai H, Fukushima Y, et al. Identification of activating enzymes of a novel FBPase inhibitor prodrug, CS-917. Pharmacol Res Perspect. 2015;3:e00138 pubmed publisher
    ..We identified cathepsin A (CTSA) and elastase 3B (ELA3B) as CS-917 esterases in the small intestine by mass spectrometry, whereas we found CTSA and ..
  37. St Clair E, Boyer D. Lower molar shape and size in prosimian and platyrrhine primates. Am J Phys Anthropol. 2016;161:237-58 pubmed publisher
    ..Principal components analysis and phylogenetic principal components analysis (pPCA) were performed on species average landmark co-ordinates...
  38. Itoh K, Shimmoto M, Utsumi K, Mizoguchi N, Miharu N, Ohama K, et al. Protective protein/cathepsin A loss in cultured cells derived from an early-infantile form of galactosialidosis patients homozygous for the A1184-G transition (Y395C mutation). Biochem Biophys Res Commun. 1998;247:12-7 pubmed
    ..a human autosomal recessive lysosomal storage disease caused by a genetic defect of protective protein/cathepsin A (PPCA)...
  39. Villegas Amtmann S, Simmons S, Kuhn C, Huckstadt L, Costa D. Latitudinal range influences the seasonal variation in the foraging behavior of marine top predators. PLoS ONE. 2011;6:e23166 pubmed publisher
    ..californianus) (CSL) whose population is increasing and the endangered Galapagos sea lion (Zalophus wollebaeki) (GSL) whose population is declining...
  40. Marques A, Gabriel T, Aten J, van Roomen C, Ottenhoff R, Claessen N, et al. Gpnmb Is a Potential Marker for the Visceral Pathology in Niemann-Pick Type C Disease. PLoS ONE. 2016;11:e0147208 pubmed publisher
    ..7 macrophages to the NPC-phenotype-inducing drug U18666A also upregulated Gpnmb expression. Inhibition of GSL synthesis with the glucosylceramide synthase (GCS) inhibitor N-butyl-1-deoxynojirimycin prevented U18666A-induced ..
  41. Kopp R, Kemp A, Bittermann K, Horton B, Donnelly J, Gehrels W, et al. Temperature-driven global sea-level variability in the Common Era. Proc Natl Acad Sci U S A. 2016;113:E1434-41 pubmed publisher
    We assess the relationship between temperature and global sea-level (GSL) variability over the Common Era through a statistical metaanalysis of proxy relative sea-level reconstructions and tide-gauge data. GSL rose at 0.1 ± 0...
  42. Russo D, Parashuraman S, D Angelo G. Glycosphingolipid-Protein Interaction in Signal Transduction. Int J Mol Sci. 2016;17: pubmed
    ..The synthesis of specific GSLs depends on the expression of distinctive sets of GSL synthesizing enzymes that is tightly regulated during development...
  43. Jakubowski A, Kunins H, Huxley Reicher Z, Siegler A. Knowledge of the 911 Good Samaritan Law and 911-calling behavior of overdose witnesses. Subst Abus. 2018;39:233-238 pubmed publisher
    ..We investigated the relationship between knowledge of a 911 Good Samaritan Law (GSL) and 911-calling behavior of study participants trained in opioid overdose rescue...
  44. Rodi P, Maggio B, Bagatolli L. Direct visualization of the lateral structure of giant vesicles composed of pseudo-binary mixtures of sulfatide, asialo-GM1 and GM1 with POPC. Biochim Biophys Acta Biomembr. 2018;1860:544-555 pubmed publisher
    ..of giant unilamellar vesicles (GUVs) composed of three pseudo binary mixtures of different glycosphingolipid (GSL), i.e. sulfatide, asialo-GM1 or GM1, with POPC...
  45. Wegner M, Schömel N, Gruber L, Örtel S, Kjellberg M, Mattjus P, et al. UDP-glucose ceramide glucosyltransferase activates AKT, promoted proliferation, and doxorubicin resistance in breast cancer cells. Cell Mol Life Sci. 2018;75:3393-3410 pubmed publisher
    ..of glycosylated sphingolipids, since this enzyme generates the precursor for all complex glycosphingolipids (GSL), the GlcCer...
  46. Glaros E, Kim W, Quinn C, Wong J, Gelissen I, Jessup W, et al. Glycosphingolipid accumulation inhibits cholesterol efflux via the ABCA1/apolipoprotein A-I pathway: 1-phenyl-2-decanoylamino-3-morpholino-1-propanol is a novel cholesterol efflux accelerator. J Biol Chem. 2005;280:24515-23 pubmed
    Cellular glycosphingolipid (GSL) storage is known to promote cholesterol accumulation...
  47. Reid M, O DONOVAN M, Murphy J, Fleming C, Kennedy E, Lewis E. The effect of high and low levels of supplementation on milk production, nitrogen utilization efficiency, and milk protein fractions in late-lactation dairy cows. J Dairy Sci. 2015;98:5529-44 pubmed publisher
    ..low grass allowance (LG), grass with a low concentrate allocation (GCL), grass with a low grass silage allocation (GSL), grass with a high concentrate allocation (GCH), and grass with a high grass silage allocation (GSH)] to measure ..
  48. Bhandari S, Jo J, Lee J. Comparison of Glucosinolate Profiles in Different Tissues of Nine Brassica Crops. Molecules. 2015;20:15827-41 pubmed publisher
    Glucosinolate (GSL) profiles and concentrations in various tissues (seeds, sprouts, mature root, and shoot) were determined and compared across nine Brassica species, including cauliflower, cabbage, broccoli, radish, baemuchae, pakchoi, ..
  49. Surkis A, Hogle J, DiazGranados D, Hunt J, Mazmanian P, Connors E, et al. Classifying publications from the clinical and translational science award program along the translational research spectrum: a machine learning approach. J Transl Med. 2016;14:235 pubmed publisher
    ..of Health (NIH), as demonstrated by the substantial investment in the Clinical and Translational Science Award (CTSA) program...
  50. Bugiani M, Kevelam S, Bakels H, Waisfisz Q, Ceuterick de Groote C, Niessen H, et al. Cathepsin A-related arteriopathy with strokes and leukoencephalopathy (CARASAL). Neurology. 2016;87:1777-1786 pubmed
    ..973C>T in CTSA. Haplotype analysis revealed a shared 1,145-kb interval encompassing the CTSA variant on chromosome 20q13...
  51. Waddington K, Jury E. Manipulating membrane lipid profiles to restore T-cell function in autoimmunity. Biochem Soc Trans. 2015;43:745-51 pubmed publisher
    Plasma membrane lipid rafts are heterogeneous cholesterol and glycosphingolipid (GSL)-enriched microdomains, within which the tight packing of cholesterol with the saturated-acyl chains of GSLs creates a region of liquid-order relative ..
  52. Qian L, Qian W, Snowdon R. Haplotype hitchhiking promotes trait coselection in Brassica napus. Plant Biotechnol J. 2016;14:1578-88 pubmed publisher
    ..The phenotypic rather than pleiotropic correlations between leaf chlorophyll content index and seed GSL suggest that LD may have led to inadvertent coselection for these two traits.
  53. Mi B, Liu Z, Hu W, Wei P, Jiang Z, Fei B. Investigating pyrolysis and combustion characteristics of torrefied bamboo, torrefied wood and their blends. Bioresour Technol. 2016;209:50-5 pubmed publisher
    Bamboo and masson pine was torrefied with 300°C of temperature for 2.0h of residence time using GSL 1600X tube furnace in the argon atmosphere. Torrefied bamboo and masson pine particles were uniform mixed with different weight ratios...
  54. Ramm R, Niemann H, Petersen B, Haverich A, Hilfiker A. Decellularized GGTA1-KO pig heart valves do not bind preformed human xenoantibodies. Basic Res Cardiol. 2016;111:39 pubmed publisher
    ..wild-type (wt) or α1,3-galactosyltransferase knockout (GGTA1-KO) pigs were investigated by inhibition ELISA and GSL I-B4 staining to localize and quantify matrix-bound αGal epitopes, which represent the most prominent ..
  55. Noël A, Ingrand S, Barrier L. Anti-amyloidogenic effects of glycosphingolipid synthesis inhibitors occur independently of ganglioside alterations. Mol Cell Neurosci. 2016;75:63-70 pubmed publisher
    ..In this study, we assessed the usefulness of two well-established glycosphingolipid (GSL) synthesis inhibitors, the synthetic ceramide analog D-PDMP (1-phenyl 2-decanoylamino-3-morpholino-1-propanol) and ..
  56. Boyd E, Yu R, Barkay T, Hamilton T, Baxter B, Naftz D, et al. Effect of salinity on mercury methylating benthic microbes and their activities in Great Salt Lake, Utah. Sci Total Environ. 2017;581-582:495-506 pubmed publisher
    Surface water and biota from Great Salt Lake (GSL) contain some of the highest documented concentrations of total mercury (THg) and methylmercury (MeHg) in the United States...
  57. Gowik U, Schulze S, Saladie M, Rolland V, Tanz S, Westhoff P, et al. A MEM1-like motif directs mesophyll cell-specific expression of the gene encoding the C4 carbonic anhydrase in Flaveria. J Exp Bot. 2017;68:311-320 pubmed publisher
    ..bidentis and F. trinervia ca3 translation start sites. MEM1 is located in the promoter of C4 Flaveria ppcA genes, which encode the C4-associated PEPC, and is necessary for M-specific expression...
  58. Wu G, Nielson J, Peterson R, Winter J. Bonnevillamides, Linear Heptapeptides Isolated from a Great Salt Lake-Derived Streptomyces sp. Mar Drugs. 2017;15: pubmed publisher
    i>Streptomyces sp. GSL-6B was isolated from sediment collected from the Great Salt Lake and investigation of its organic extract led to the isolation of three new linear heptapeptides, bonnevillamides A (1), B (2), and ..
  59. Kwan J, Daye D, Schmidt M, Conlon C, Kim H, Gaonkar B, et al. Exploring intentions of physician-scientist trainees: factors influencing MD and MD/PhD interest in research careers. BMC Med Educ. 2017;17:115 pubmed publisher
    ..medical centers with Medical Scientist Training Programs (MSTPs) and Clinical and Translational Science Awards (CTSA) from the NIH. Data were analyzed using bivariate or multivariate analyses...
  60. Bes D, Fernández M, Malla I, Repetto H, Buamsch D, Lopez S, et al. Management of cirrhotic ascites in children. Review and recommendations. Part 1: Pathophysiology, diagnostic evaluation, hospitalization criteria, treatment, nutritional management. Arch Argent Pediatr. 2017;115:385-390 pubmed publisher
    ..In the case of pediatric patients with cirrhotic ascites (PPCA), the following questions are raised: How are the clinical assessment and ancillary tests performed? When is ..
  61. Tan A, Sanny A, Ng S, Ho Y, Basri N, Lee A, et al. Excessive interferon-α signaling in autoimmunity alters glycosphingolipid processing in B cells. J Autoimmun. 2018;89:53-62 pubmed publisher
    ..Excessive stimulation of B cells with IFN-α altered the expression of enzymes that catalyze critical steps in GSL processing, increasing the expressions of glucosylceramide synthase (GCS) and globotrihexosylceramide synthase (..
  62. Bonten E, Campos Y, Zaitsev V, Nourse A, Waddell B, Lewis W, et al. Heterodimerization of the sialidase NEU1 with the chaperone protective protein/cathepsin A prevents its premature oligomerization. J Biol Chem. 2009;284:28430-41 pubmed publisher
    ..neuraminidase-1 (NEU1) forms a multienzyme complex with beta-galactosidase and protective protein/cathepsin A (PPCA)...
  63. Baskar V, Park S. Molecular characterization of BrMYB28 and BrMYB29 paralogous transcription factors involved in the regulation of aliphatic glucosinolate profiles in Brassica rapa ssp. pekinensis. C R Biol. 2015;338:434-42 pubmed publisher
    Glucosinolates (GSL) are one of the major secondary metabolites of the Brassicaceae family...
  64. Smit C, Kies C, McWilliam H, Meeusen E, Hokke C, van Diepen A. Local Antiglycan Antibody Responses to Skin Stage and Migratory Schistosomula of Schistosoma japonicum. Infect Immun. 2016;84:21-33 pubmed publisher
    ..Also, responses to antigenic and schistosome-specific glycosphingolipid (GSL) glycans containing highly fucosylated GalNAcβ1-4(GlcNAcβ1)n stretches that are believed to be present at the ..
  65. Bell L, Methven L, Wagstaff C. The influence of phytochemical composition and resulting sensory attributes on preference for salad rocket (Eruca sativa) accessions by consumers of varying TAS2R38 diplotype. Food Chem. 2017;222:6-17 pubmed publisher
    ..Some consumers rejected rocket based on GSL/ITC concentrations, whereas some preferred hotness...
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    ..we performed a comparative genomics analysis between R. sativus and Arabidopsis thaliana. In total, 144 GSL metabolism genes were identified, and most of these GSL genes have expanded through whole-genome and tandem ..
  67. Kaufman A, Rhyne R, Anastasoff J, Ronquillo F, Nixon M, MISHRA S, et al. Health Extension and Clinical and Translational Science: An Innovative Strategy for Community Engagement. J Am Board Fam Med. 2017;30:94-99 pubmed publisher
    ..Health extension can serve as a university-funded, community-based bridge between community health needs and Clinical and Translational Science Award (CTSA) research capacity, opening avenues for translational research.
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    ..gal) and neuraminidase secondary to a defect of a lysosomal enzyme protective protein/cathepsin A (PPCA) and mutation in CTSA gene. Three subtypes are recognized: early infantile, late infantile, and juvenile/adult...
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    Cathepsin A (CTSA) is serine carboxypeptidase, an important protease in the lysosome...
  70. Latimore A, Bergstein R. "Caught with a body" yet protected by law? Calling 911 for opioid overdose in the context of the Good Samaritan Law. Int J Drug Policy. 2017;50:82-89 pubmed publisher
    ..This study, conducted during the period that followed implementation of a GSL, aimed to characterize current factors determining the decision to call for emergency medical help (911) at the ..
  71. Jha D, Kim J, Choi M, Kwon G. Pathological Brain Detection Using Weiner Filtering, 2D-Discrete Wavelet Transform, Probabilistic PCA, and Random Subspace Ensemble Classifier. Comput Intell Neurosci. 2017;2017:4205141 pubmed publisher
    ..2D-discrete wavelet transform (2D-DWT) for feature extraction, probabilistic principal component analysis (PPCA) for dimensionality reduction, and a random subspace ensemble (RSE) classifier along with the K-nearest ..
  72. Pavlova N, Li S, Li Y. Degradation of glycosphingolipids in oyster: ceramide glycanase and ceramidase in the hepatopancreas of oyster, Crassostrea virginica. Glycoconj J. 2018;35:77-86 pubmed publisher
    The hepatopancreas of oyster, Crassostrea virginica, was found to contain two unique glycosphingolipid (GSL) cleaving enzymes, ceramide glycanase (CGase) and ceramidase...
  73. Ishii N, Oshima A, Sakuraba H, Fukuyama Y, Suzuki Y. Normal serum beta-galactosidase in juvenile GM1 gangliosidosis. Pediatr Neurol. 1994;10:317-9 pubmed
    ..beta-Galactosidase assay is not always reliable, particularly with serum or plasma as an enzyme source, for the diagnosis of hereditary beta-galactosidase deficiency, unless it is conducted under well-controlled conditions. ..
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    ..Pharmacological studies in rodents indicate a remarkable influence of CatA inhibition on cardiovascular disease progression and identify CatA as a promising novel target for the treatment of heart failure. ..
  75. Lakshminarayan R, Wunder C, Becken U, Howes M, Benzing C, Arumugam S, et al. Galectin-3 drives glycosphingolipid-dependent biogenesis of clathrin-independent carriers. Nat Cell Biol. 2014;16:595-606 pubmed publisher
    ..Here, we found that a carbohydrate-binding protein, galectin-3 (Gal3), triggered the glycosphingolipid (GSL)-dependent biogenesis of a morphologically distinct class of endocytic structures, termed clathrin-independent ..
  76. Pandey C, Augustine R, Panthri M, Zia I, Bisht N, Gupta M. Arsenic affects the production of glucosinolate, thiol and phytochemical compounds: A comparison of two Brassica cultivars. Plant Physiol Biochem. 2017;111:144-154 pubmed publisher
    ..Change in morphological traits, concentration of individual as well as total GSL, sulfur related thiol proteins, sulfur content, and phytochemicals were analyzed in both cultivars...
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    As noted in part 1 of this series, periprocedural cardiac arrest (PPCA) can differ greatly in etiology and treatment from what is described by the American Heart Association advanced cardiac life support algorithms, which were largely ..
  78. Morreau H, Galjart N, Willemsen R, Gillemans N, Zhou X, d Azzo A. Human lysosomal protective protein. Glycosylation, intracellular transport, and association with beta-galactosidase in the endoplasmic reticulum. J Biol Chem. 1992;267:17949-56 pubmed
    ..Mutated protective protein precursors that are retained in the endoplasmic reticulum or pre-Golgi complex interact with and withhold normal beta-galactosidase molecules in the same compartments, thereby preventing their normal routing. ..
  79. Jackman H, Tan F, Tamei H, Beurling Harbury C, Li X, Skidgel R, et al. A peptidase in human platelets that deamidates tachykinins. Probable identity with the lysosomal "protective protein". J Biol Chem. 1990;265:11265-72 pubmed
    ..Because an amidated carboxyl terminus is important for biological activity, we purified and characterized this deamidase. The enzyme, released from human platelets by thrombin, was purified to homogeneity by ammonium sulfate ..
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    ..The combined use of immunoprecipitation and immunostaining was useful for analysing the pathophysiology of the intracellular processing and transport of the mutant beta-galactosidase. ..
  81. Tatano Y, Takeuchi N, Kuwahara J, Sakuraba H, Takahashi T, Takada G, et al. Elastogenesis in cultured dermal fibroblasts from patients with lysosomal beta-galactosidase, protective protein/cathepsin A and neuraminidase-1 deficiencies. J Med Invest. 2006;53:103-12 pubmed
    ..tropoelastin and a component of receptor complex among neuraminidase-1 (NEU1) and protective protein/cathepsin A (PPCA) is suggested responsible for impaired elastogenesis in autosomal recessive beta-Gal, PPCA and NEU1 deficiencies...
  82. Bell L, Wagstaff C. Enhancement Of Glucosinolate and Isothiocyanate Profiles in Brassicaceae Crops: Addressing Challenges in Breeding for Cultivation, Storage, and Consumer-Related Traits. J Agric Food Chem. 2017;65:9379-9403 pubmed publisher
    ..In this review, we draw together data for multiple species and address how such factors can influence GSL profiles...
  83. Willemsen R, Hoogeveen A, Sips H, Van Dongen J, Galjaard H. Immunoelectron microscopical localization of lysosomal beta-galactosidase and its precursor forms in normal and mutant human fibroblasts. Eur J Cell Biol. 1986;40:9-15 pubmed
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    ..In this review a current state of knowledge is presented concerning the regulation of selected glycosidases activity by cathepsin A (EC and C (EC ..
  86. Rahikainen M, Trotta A, Alegre S, Pascual J, Vuorinen K, Overmyer K, et al. PP2A-B'? modulates foliar trans-methylation capacity and the formation of 4-methoxy-indol-3-yl-methyl glucosinolate in Arabidopsis leaves. Plant J. 2017;89:112-127 pubmed publisher
    Glucosinolates (GSL) of cruciferous plants comprise a major group of structurally diverse secondary compounds which act as deterrents against aphids and microbial pathogens and have large commercial and ecological impacts...
  87. Morgado L, Bruix M, Pokkuluri P, Salgueiro C, Turner D. Redox- and pH-linked conformational changes in triheme cytochrome PpcA from Geobacter sulfurreducens. Biochem J. 2017;474:231-246 pubmed publisher
    The periplasmic triheme cytochrome PpcA from Geobacter sulfurreducens is highly abundant; it is the likely reservoir of electrons to the outer surface to assist the reduction of extracellular terminal acceptors; these include insoluble ..
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    ..perturbation measurements were used to probe for an interaction between the most abundant periplasmic cytochrome PpcA and the dodecahaem cytochrome GSU1996, one of the proposed nanowire cytochromes in G...
  89. Maire I, Nivelon Chevallier A. Combined deficiency of beta-galactosidase and neuraminidase: three affected siblings in a French family. J Inherit Metab Dis. 1981;4:221-3 pubmed
    ..Oligosaccharides were found in urine and the enzyme activities determined in leukocytes and cultured fibroblasts. Further characterization of the defect in this family is needed. ..
  90. Chakraborty S, Rafi M, Wenger D. Mutations in the lysosomal beta-galactosidase gene that cause the adult form of GM1 gangliosidosis. Am J Hum Genet. 1994;54:1004-13 pubmed
    ..It appears that the C-->T mutation results in enough functional enzyme to produce a mild adult form of the disease, even in the presence of a second mutation that likely produces nonfunctional enzyme. ..
  91. Orenstein S, Cuervo A. Chaperone-mediated autophagy: molecular mechanisms and physiological relevance. Semin Cell Dev Biol. 2010;21:719-26 pubmed publisher
    ..Changes with age in CMA activity and the contribution of failure of CMA to the phenotype of aging and to the pathogenesis of several age-related pathologies are also described. ..
  92. Prada C, Gonzaga Jauregui C, Tannenbaum R, Penney S, Lupski J, Hopkin R, et al. Clinical utility of whole-exome sequencing in rare diseases: Galactosialidosis. Eur J Med Genet. 2014;57:339-344 pubmed publisher
    ..We identified compound heterozygous mutations in the CTSA gene, responsible for causing galactosialidosis; the molecular diagnosis was further confirmed by biochemical ..