Gene Symbol: CSTB
Description: cystatin B
Alias: CPI-B, CST6, EPM1, EPM1A, PME, STFB, ULD, cystatin-B, cystatin B (stefin B), liver thiol proteinase inhibitor
Species: human
Products:     CSTB

Top Publications

  1. Pennacchio L, Lehesjoki A, Stone N, Willour V, Virtaneva K, Miao J, et al. Mutations in the gene encoding cystatin B in progressive myoclonus epilepsy (EPM1). Science. 1996;271:1731-4 pubmed
    ..3. The gene encoding cystatin B was shown to be localized to this region, and levels of messenger RNA encoded by this gene were found to be ..
  2. Cipollini E, Riccio M, Di Giaimo R, Dal Piaz F, Pulice G, Catania S, et al. Cystatin B and its EPM1 mutants are polymeric and aggregate prone in vivo. Biochim Biophys Acta. 2008;1783:312-22 pubmed
    Progressive myoclonus epilepsy type 1 (EPM1) is a neurodegenerative disease correlating with mutations of the cystatin B gene. Cystatin B is described as a monomeric protein with antiprotease function...
  3. Skerget K, Vilfan A, Pompe Novak M, Turk V, Waltho J, Turk D, et al. The mechanism of amyloid-fibril formation by stefin B: temperature and protein concentration dependence of the rates. Proteins. 2009;74:425-36 pubmed publisher
    ..We have extended previous studies of the kinetics of amyloid-fibril formation by human stefin B (cystatin B) and some of its mutants, and proposed an improved model for the reaction...
  4. Ceru S, Konjar S, Maher K, Repnik U, Krizaj I, Bencina M, et al. Stefin B interacts with histones and cathepsin L in the nucleus. J Biol Chem. 2010;285:10078-86 pubmed publisher
    Stefin B (cystatin B) is an endogenous inhibitor of cysteine proteinases localized in the nucleus and the cytosol...
  5. Turk V, Bode W. The cystatins: protein inhibitors of cysteine proteinases. FEBS Lett. 1991;285:213-9 pubmed
  6. Lenarcic B, Kos J, Dolenc I, Lucovnik P, Krizaj I, Turk V. Cathepsin D inactivates cysteine proteinase inhibitors, cystatins. Biochem Biophys Res Commun. 1988;154:765-72 pubmed
  7. Kos J, Lah T. Cysteine proteinases and their endogenous inhibitors: target proteins for prognosis, diagnosis and therapy in cancer (review). Oncol Rep. 1998;5:1349-61 pubmed
    ..Their application for diagnosis, follow-up and the anticancer therapy has also been proposed. ..
  8. Anderluh G, Gutierrez Aguirre I, Rabzelj S, Ceru S, Kopitar Jerala N, Macek P, et al. Interaction of human stefin B in the prefibrillar oligomeric form with membranes. Correlation with cellular toxicity. FEBS J. 2005;272:3042-51 pubmed
    ..This study is aimed to contribute to the general model of cellular toxicity induced by prefibrillar oligomers of amyloidogenic proteins, not necessarily involved in pathology. ..
  9. Joensuu T, Kuronen M, Alakurtti K, Tegelberg S, Hakala P, Aalto A, et al. Cystatin B: mutation detection, alternative splicing and expression in progressive myclonus epilepsy of Unverricht-Lundborg type (EPM1) patients. Eur J Hum Genet. 2007;15:185-93 pubmed
    ..Unverricht-Lundborg type (EPM1) is an autosomal recessive neurodegenerative disorder caused by mutations in the cystatin B gene (CSTB) that encodes an inhibitor of several lysosomal cathepsins...

More Information

Publications129 found, 100 shown here

  1. Rabzelj S, Viero G, Gutierrez Aguirre I, Turk V, Dalla Serra M, Anderluh G, et al. Interaction with model membranes and pore formation by human stefin B: studying the native and prefibrillar states. FEBS J. 2008;275:2455-66 pubmed publisher
    ..Similarities to other amyloidogenic proteins and the possible physiological implications of our findings are discussed. ..
  2. Skerget K, Taler Vercic A, Bavdek A, Hodnik V, Ceru S, Tusek Znidaric M, et al. Interaction between oligomers of stefin B and amyloid-beta in vitro and in cells. J Biol Chem. 2010;285:3201-10 pubmed publisher
    ..in Alzheimer disease and in neuroprotection in general, we studied the interaction between human stefin B (cystatin B) and amyloid-beta-(1-40) peptide (Abeta)...
  3. Ceru S, Layfield R, Zavasnik Bergant T, Repnik U, Kopitar Jerala N, Turk V, et al. Intracellular aggregation of human stefin B: confocal and electron microscopy study. Biol Cell. 2010;102:319-34 pubmed publisher
    ..Mutations in the CSTB (cystatin B) gene [StB (stefin B)] cause EPM1 (progressive myoclonus epilepsy of type 1), an epilepsy syndrome with features ..
  4. Chauhan S, Tomar R. Efficient expression and purification of biologically active human cystatin proteins. Protein Expr Purif. 2016;118:10-7 pubmed publisher
    ..Their biological activities were tested by inhibition assays against cathepsin L and H3 protease. ..
  5. Zerovnik E. Possible Mechanisms by which Stefin B could Regulate Proteostasis and Oxidative Stress. Cells. 2019;8: pubmed publisher
    ..It is also suggested how more knowledge about the role of stefin B in a cell's response to misfolded proteins could be used to modulate progressive myoclonus epilepsy of type 1 EPM1 disease.
  6. Rubboli G, Veggiotti P, Pini A, Berardinelli A, Cantalupo G, Bertini E, et al. Spinal muscular atrophy associated with progressive myoclonic epilepsy: A rare condition caused by mutations in ASAH1. Epilepsia. 2015;56:692-8 pubmed publisher
    ..investigations in three patients with spinal muscular atrophy associated with progressive myoclonic epilepsy (SMA-PME), a rare condition caused by mutations in the N-acylsphingosine amidohydrosilase 1 (ASAH1) gene...
  7. Kim H, Lee S, Choi M, Kim H, Hwang H, Choi J, et al. Familial cases of progressive myoclonic epilepsy caused by maternal somatic mosaicism of a recurrent KCNC1 p.Arg320His mutation. Brain Dev. 2018;40:429-432 pubmed publisher
    ..Arg320His) has been identified recently as one of the important genetic causes of progress myoclonic epilepsy (PME)...
  8. Manconi B, Liori B, Cabras T, Iavarone F, Manni A, Messana I, et al. Top-down HPLC-ESI-MS proteomic analysis of saliva of edentulous subjects evidenced high levels of cystatin A, cystatin B and SPRR3. Arch Oral Biol. 2017;77:68-74 pubmed publisher
    ..Levels of cystatin A, S-glutathionylated, S-cystenylated, S-S dimer derivatives of cystatin B and S-glutathionylated derivative of SPRR3, were found significantly higher in edentulous subjects with respect ..
  9. Filosto M, Aureli M, Castellotti B, Rinaldi F, Schiumarini D, Valsecchi M, et al. ASAH1 variant causing a mild SMA phenotype with no myoclonic epilepsy: a clinical, biochemical and molecular study. Eur J Hum Genet. 2016;24:1578-1583 pubmed publisher
    ..early-onset Farber disease and rare cases of spinal muscular atrophy (SMA) with progressive myoclonic epilepsy (SMA-PME), phenotypically characterized by childhood onset of proximal muscle weakness and atrophy due to spinal motor ..
  10. Tang Y, Cao G, Min X, Wang T, Sun S, Du X, et al. Cathepsin B inhibition ameliorates the non-alcoholic steatohepatitis through suppressing caspase-1 activation. J Physiol Biochem. 2018;74:503-510 pubmed publisher
    ..CTSB inhibitor (CA-074) was used to suppress the expression of CSTB. Expressions of CTSB and caspase-1 were evaluated by immunohistochemical staining...
  11. van den Ameele J, Jedličková I, Přistoupilová A, Sieben A, Van Mossevelde S, Ceuterick de Groote C, et al. Teenage-onset progressive myoclonic epilepsy due to a familial C9orf72 repeat expansion. Neurology. 2018;90:e658-e663 pubmed publisher
    The progressive myoclonic epilepsies (PME) are a heterogeneous group of disorders in which a specific diagnosis cannot be made in a subset of patients, despite exhaustive investigation...
  12. Evangelista D, de Paula F, Rodrigues A, Henrique Silva F. Pectinases from Sphenophorus levis Vaurie, 1978 (Coleoptera: Curculionidae): putative accessory digestive enzymes. J Insect Sci. 2015;15:168 pubmed publisher
    ..Two cDNA sequences identified in a cDNA library of the insect larvae coding for a pectin methylesterase (PME) and an endo-polygalacturonase (endo-PG)-denominated Sl-PME and Sl-endoPG, respectively-were isolated and ..
  13. Nascimento F, Andrade D. Myoclonus epilepsy and ataxia due to potassium channel mutation (MEAK) is caused by heterozygous KCNC1 mutations. Epileptic Disord. 2016;18:135-138 pubmed
    Progressive myoclonus epilepsy (PME) is a distinct group of seizure disorders characterized by gradual neurological decline with ataxia, myoclonus and recurring seizures...
  14. Muona M, Berkovic S, Dibbens L, Oliver K, Maljevic S, Bayly M, et al. A recurrent de novo mutation in KCNC1 causes progressive myoclonus epilepsy. Nat Genet. 2015;47:39-46 pubmed publisher
    ..We sequenced the exomes of 84 unrelated individuals with PME of unknown cause and molecularly solved 26 cases (31%). Remarkably, a recurrent de novo mutation, c.959G>A (p...
  15. Manconi B, Liori B, Cabras T, Vincenzoni F, Iavarone F, Castagnola M, et al. Salivary Cystatins: Exploring New Post-Translational Modifications and Polymorphisms by Top-Down High-Resolution Mass Spectrometry. J Proteome Res. 2017;16:4196-4207 pubmed publisher
    ..In the present study, various proteoforms of cystatin A, cystatin B, cystatin S, cystatin SN, and cystatin SA were detected in the acid-soluble fraction of human saliva and ..
  16. Tamhane T, Lllukkumbura R, Lu S, Maelandsmo G, Haugen M, Brix K. Nuclear cathepsin L activity is required for cell cycle progression of colorectal carcinoma cells. Biochimie. 2016;122:208-18 pubmed publisher
    ..Furthermore, we hypothesize that nuclear cathepsin L accelerates cell cycle progression of HCT116 cells thereby supporting the notion that cysteine cathepsins may play significant roles in carcinogenesis due to deregulated trafficking. ..
  17. Teufel M, Seidel H, Köchert K, Meinhardt G, Finn R, Llovet J, et al. Biomarkers Associated With Response to Regorafenib in Patients With Hepatocellular Carcinoma. Gastroenterology. 2019;156:1731-1741 pubmed publisher
    ..Decreased baseline plasma concentrations of 5 of 266 evaluable proteins (angiopoietin 1, cystatin B, the latency-associated peptide of transforming growth factor beta 1, oxidized low-density lipoprotein receptor ..
  18. Cheng W, Tang M, Xie Y, Xu L, Wang Y, Luo X, et al. Transcriptome-based gene expression profiling of diploid radish (Raphanus sativus L.) and the corresponding autotetraploid. Mol Biol Rep. 2019;46:933-945 pubmed publisher
    ..Moreover, the xyloglucan endotransglucosylase/hydrolase (XTH) and pectin methylesterases (PME) family members related to cell enlargement and cell wall construction were found to be enriched in GO enrichment ..
  19. Wang Y, Duan X, Xu S, Wang R, Ouyang Z, Shen W. Linking hydrogen-mediated boron toxicity tolerance with improvement of root elongation, water status and reactive oxygen species balance: a case study for rice. Ann Bot. 2016;118:1279-1291 pubmed
    ..Meanwhile, activities of catalase (CAT) and peroxidase (POX) were increased. Suppression of pectin methylesterase (PME) activity and downregulation of PME transcripts by H2 were consistent with the alleviation of root ..
  20. Puglia M, Landi C, Gagliardi A, Breslin L, Armini A, Brunetti J, et al. The proteome speciation of an immortalized cystic fibrosis cell line: New perspectives on the pathophysiology of the disease. J Proteomics. 2018;170:28-42 pubmed publisher
    ..Our data could open new perspectives into the understanding of CF, identifying potential targets for drug treatments in order to alleviate Δ508CFTR membrane instability and consequently increase life expectancy for CF patients. ..
  21. Žerovnik E. Putative alternative functions of human stefin B (cystatin B): binding to amyloid-beta, membranes, and copper. J Mol Recognit. 2017;30: pubmed publisher
    ..We also briefly mention our studies on aggregation of some of the missense EPM1 mutants of stefin B in cells, which mimic additional pathological traits (gain in toxic function) in selected ..
  22. Opri R, Fabrizi G, Cantalupo G, Ferrarini M, Simonati A, Dalla Bernardina B, et al. Progressive Myoclonus Epilepsy in Congenital Generalized Lipodystrophy type 2: Report of 3 cases and literature review. Seizure. 2016;42:1-6 pubmed publisher
    ..Herein we describe clinical and EEG features of three patients presenting with Progressive Myoclonus Epilepsy (PME) and Congenital Generalized Lipodystrophy type 2 (CGL2) related to novel Seipin mutations...
  23. Martins V, Garcia A, Costa C, Sottomayor M, Gerós H. Calcium- and hormone-driven regulation of secondary metabolism and cell wall enzymes in grape berry cells. J Plant Physiol. 2018;231:57-67 pubmed publisher
    ..Modulation of the expression and activity of pectin methyl esterases (PME) and polygalacturonases (PG) by calcium was also evident, confirming an important role of calcium in cell wall ..
  24. Maubach G, Lim M, Zhuo L. Nuclear cathepsin F regulates activation markers in rat hepatic stellate cells. Mol Biol Cell. 2008;19:4238-48 pubmed publisher
    ..The treatment for 48 h leads to a decrease in activity, which is coupled to an increase in cystatin B and C transcripts...
  25. Liu G, Bergenholm D, Nielsen J. Genome-Wide Mapping of Binding Sites Reveals Multiple Biological Functions of the Transcription Factor Cst6p in Saccharomyces cerevisiae. MBio. 2016;7: pubmed publisher
    ..The retarded growth of the CST6 deletion mutant on ethanol is attributed to the markedly decreased expression of NCE103, encoding a carbonic ..
  26. Hakkarainen A, Puustinen L, Kivisaari R, Boyd S, Nieminen U, Arkkila P, et al. Metabolic profile of liver damage in non-cirrhotic virus C and autoimmune hepatitis: A proton decoupled 31P-MRS study. Eur J Radiol. 2017;90:205-211 pubmed publisher
    ..0T MR imager. Intensities of phosphomonoesters (PME) of phosphoethanolamine (PE) and phosphocholine (PC), phosphodiesters (PDE) of glycerophosphoethanolamine (GPE) and ..
  27. Xu W, Mukherjee S, Ning Y, Hsu F, Zhang K. Cyclopropane fatty acid synthesis affects cell shape and acid resistance in Leishmania mexicana. Int J Parasitol. 2018;48:245-256 pubmed publisher
    ..Our data indicate that L. mexicana CFAS modifies the fatty acid chain of plasmenylethanolamine (PME), the dominant class of ethanolamine glycerophospholipids in Leishmania, generating CFA-PME...
  28. Sendler M, Maertin S, John D, Persike M, Weiss F, Krüger B, et al. Cathepsin B Activity Initiates Apoptosis via Digestive Protease Activation in Pancreatic Acinar Cells and Experimental Pancreatitis. J Biol Chem. 2016;291:14717-31 pubmed publisher
    ..Caspase 3-mediated apoptosis depends on intravesicular trypsinogen activation induced by CTSB, not CTSB activity directly, and this mechanism is pancreas-specific. ..
  29. Yang D, Stavrides P, Saito M, Kumar A, Rodriguez Navarro J, Pawlik M, et al. Defective macroautophagic turnover of brain lipids in the TgCRND8 Alzheimer mouse model: prevention by correcting lysosomal proteolytic deficits. Brain. 2014;137:3300-18 pubmed publisher
    ..lysosomal proteolytic capacity and autophagy flux via genetic deletion of the lysosomal protease inhibitor, cystatin B. Here we present evidence that macroautophagy is a significant pathway for lipid turnover, which is defective in ..
  30. Taha M, de Oliveira J, Dias Borges M, de Lucca Melo F, Gualtieri F, E Silva Aidar A, et al. L-Arginine Modulates Intestinal Inflammation in Rats Submitted to Mesenteric Ischemia-Reperfusion Injury. Transplant Proc. 2016;48:512-5 pubmed publisher
    ..Eighteen Wistar-EPM1 male rats (250-300 g) were anesthetized and subjected to laparotomy...
  31. Tayal P, Raj S, Sharma E, Kumar M, Dayaman V, Verma N, et al. A Botrytis cinerea KLP-7 Kinesin acts as a Virulence Determinant during Plant Infection. Sci Rep. 2017;7:10664 pubmed publisher
    ..of oxalic acid (OA) and exhibited reduced activities of polygalacturonase (PG) and pectin methyl esterases (PME). TAIL-PCR followed by BLAST search identified the tagged gene as KLP-7 that encodes for kinesin...
  32. Liu Q, Talbot M, Llewellyn D. Pectin methylesterase and pectin remodelling differ in the fibre walls of two gossypium species with very different fibre properties. PLoS ONE. 2013;8:e65131 pubmed publisher
    ..in Golgi, secreted into the wall as methylesters and subsequently de-esterified by pectin methylesterase (PME)...
  33. Bilska Kos A, Solecka D, Dziewulska A, Ochodzki P, Jończyk M, Bilski H, et al. Low temperature caused modifications in the arrangement of cell wall pectins due to changes of osmotic potential of cells of maize leaves (Zea mays L.). Protoplasma. 2017;254:713-724 pubmed publisher
    ..the effect of cold on the cell wall properties, the content and localization of pectins and pectin methylesterase (PME) activity, were studied in two maize inbred lines characterized by different sensitivity to cold...
  34. Meyer M, Huttenlocher F, Cedzich A, Procopio S, Stroeder J, Pau Roblot C, et al. The subtilisin-like protease SBT3 contributes to insect resistance in tomato. J Exp Bot. 2016;67:4325-38 pubmed publisher
    ..While such a role has been described in other systems, PME activity and the degree of pectin methylesterification did not correlate with the level of insect resistance in ..
  35. Kaushik N, Rao P, Mishra H. Effect of high pressure and thermal processing on spoilage-causing enzymes in mango (Mangifera indica). Food Res Int. 2017;100:885-893 pubmed publisher
    ..The inactivation of enzymes pectin methylesterase (PME), polyphenol oxidase (PPO) and peroxidase (POD) was studied in mango pulp within the pressure, temperature and hold-..
  36. Christiaens S, Van Buggenhout S, Houben K, Chaula D, van Loey A, Hendrickx M. Unravelling process-induced pectin changes in the tomato cell wall: An integrated approach. Food Chem. 2012;132:1534-1543 pubmed publisher
    The activity of the pectin-modifying enzymes pectin-methylesterase (PME) and polygalacturonase (PG) in tomato fruit was tailored by processing...
  37. Ren S, Xie S, Zheng T, Wang Y, Xu S, Xue B, et al. Synthesis of silyl iron hydride via Si-H activation and its dual catalytic application in the hydrosilylation of carbonyl compounds and dehydration of benzamides. Dalton Trans. 2018;47:4352-4359 pubmed publisher
    The hydrido silyl iron complex (o-Ph2PC6H4SiMe2)Fe(PMe3)3H (2) was obtained via the activation of the Si-H bond of the bidentate silyl ligand o-Ph2P(C6
  38. L Enfant M, Domon J, Rayon C, Desnos T, Ralet M, Bonnin E, et al. Substrate specificity of plant and fungi pectin methylesterases: Identification of novel inhibitors of PMEs. Int J Biol Macromol. 2015;81:681-91 pubmed publisher
    ..purpose, we used two plant PMEs (from orange peel: Citrus sinensis and from Arabidopsis thaliana) and one fungal PME (from Botrytis cinerea)...
  39. Kaur A, Westermarck J. Regulation of protein phosphatase 2A (PP2A) tumor suppressor function by PME-1. Biochem Soc Trans. 2016;44:1683-1693 pubmed
    ..Protein phosphatase methylesterase-1 (PME-1) negatively regulates PP2A activity by highly complex mechanisms that are reviewed here...
  40. Hocq L, Sénéchal F, Lefebvre V, Lehner A, Domon J, Mollet J, et al. Combined Experimental and Computational Approaches Reveal Distinct pH Dependence of Pectin Methylesterase Inhibitors. Plant Physiol. 2017;173:1075-1093 pubmed publisher
    ..proteinaceous pectin methylesterase inhibitors (PMEIs; 76 members in Arabidopsis) questions the specificity of the PME-PMEI interaction and the functional role of such abundance...
  41. Khouri K, Cardoso E, Khouri R. Percutaneous Mesh Expansion: A Regenerative Wound Closure Alternative. Plast Reconstr Surg. 2018;141:451-457 pubmed publisher
    ..It sums these regenerated gaps in a mesh pattern that expands tissues to close complex wounds without flaps or additional incisions. Therapeutic, IV. ..
  42. Lv Y, Wang Z, Liu C, Cui L. Identification of a novel CACNA1A mutation in a Chinese family with autosomal recessive progressive myoclonic epilepsy. Neuropsychiatr Dis Treat. 2017;13:2631-2636 pubmed publisher
    Progressive myoclonic epilepsy (PME) is a heterogeneous neurodegenerative disorder, which is commonly manifested with refractory seizures and neurologic deterioration. The prognosis of PME is poor, so early diagnosis of PME is critical...
  43. Jinadatha C, Villamaria F, Coppin J, Dale C, Williams M, Whitworth R, et al. Interaction of healthcare worker hands and portable medical equipment: a sequence analysis to show potential transmission opportunities. BMC Infect Dis. 2017;17:800 pubmed publisher
    ..importance of a clean health care environment, there is a lack of research on the role portable medical equipment (PME) play in the transmission cycle of healthcare-acquired infections (HAIs)...
  44. He R, Li Y, Xiang P, Li C, Cui X, Ma L. Impact of particle size on distribution and human exposure of flame retardants in indoor dust. Environ Res. 2018;162:166-172 pubmed publisher
    ..retardants (NBFRs), and 2 dechlorane plus (DPs)) in composite dust samples from offices, public microenvironments (PME), and cars in Nanjing, China...
  45. Dai M, Liu W, Hong H, Lu H, Liu J, Jia H, et al. Exogenous phosphorus enhances cadmium tolerance by affecting cell wall polysaccharides in two mangrove seedlings Avicennia marina (Forsk.) Vierh and Kandelia obovata (S., L.) Yong differing in cadmium accumulation. Mar Pollut Bull. 2018;126:86-92 pubmed publisher
    ..marina under Cd stress. In addition, cell wall pectin methylesterase (PME) activity was markedly increased in the presence of Cd and P compared with Cd alone...
  46. Kupsch J, Jackisch T, Matzel K, Zimmer J, Schreiber A, Sims A, et al. Outcome of bowel function following anterior resection for rectal cancer-an analysis using the low anterior resection syndrome (LARS) score. Int J Colorectal Dis. 2018;33:787-798 pubmed publisher
    ..2% affected patients) compared to total mesorectal excision (TME, 58.4%) and partial mesorectal excision (PME, 38.0%, p?=?0.001). Radio(chemo)therapy resulted in LARS scores >?20 in 64.6% of patients compared to 43...
  47. Kaur A, Elzagheid A, Birkman E, Avoranta T, Kytölä V, Korkeila E, et al. Protein phosphatase methylesterase-1 (PME-1) expression predicts a favorable clinical outcome in colorectal cancer. Cancer Med. 2015;4:1798-808 pubmed publisher
    ..Here, we report the role of PP2A inhibitor PME-1 in CRC...
  48. Rangu S, Basu B, Muralidharan B, Tripathi S, Apte S. Involvement of phosphoesterases in tributyl phosphate degradation in Sphingobium sp. strain RSMS. Appl Microbiol Biotechnol. 2016;100:461-8 pubmed publisher
    ..Cellular proteins of Sphingobium SS22 showed none or negligible phosphodiesterase (PDE) and phosphomonoesterase (PME) activities at pH 7 and displayed approximately five- and approximately twofold less DBP and monobutyl phosphate ..
  49. Pusey M, Bail S, Xu Y, Buiakova O, Nestor M, Yang J, et al. Inhibition of protein methylesterase 1 decreased cancerous phenotypes in endometrial adenocarcinoma cell lines and xenograft tumor models. Tumour Biol. 2016;37:11835-11842 pubmed
    Protein methylesterase 1 (PME-1) promotes cancerous phenotypes through the demethylation and inactivation of protein phosphatase 2A...
  50. Wambo T, Rodriguez R, CHEN L. Computing osmotic permeabilities of aquaporins AQP4, AQP5, and GlpF from near-equilibrium simulations. Biochim Biophys Acta Biomembr. 2017;1859:1310-1316 pubmed publisher
    ..AQPs (water channels AQP4 and AQP5 and glycerol-water channel GlpF) using the mature particle mesh Ewald technique (PME) for which the established force fields have been optimized with known accuracy...
  51. Kwiatkowski M, Donald K, Stein D, Ipser J, Thomas K, Roos A. Cognitive outcomes in prenatal methamphetamine exposed children aged six to seven years. Compr Psychiatry. 2017;80:24-33 pubmed publisher
    ..Research delineating the cognitive outcomes of prenatal methamphetamine exposure (PME) in early childhood is limited, however...
  52. Jia H, Jiu S, Zhang C, Wang C, Tariq P, Liu Z, et al. Abscisic acid and sucrose regulate tomato and strawberry fruit ripening through the abscisic acid-stress-ripening transcription factor. Plant Biotechnol J. 2016;14:2045-65 pubmed publisher
    ..of several ripening-related genes such as CHS, CHI, F3H, DFR, ANS, UFGT, PG, PL, EXP1/2, XET16, Cel1/2 and PME. Taken together, this study may provide new evidence on the important role of ASR in cross-signalling between ABA ..
  53. Li Z, Heng J, Yan J, Guo X, Tang L, Chen M, et al. Integrated analysis of gene expression and methylation profiles of 48 candidate genes in breast cancer patients. Breast Cancer Res Treat. 2016;160:371-383 pubmed
    ..Total 23 genes (ACADL, APC, BRCA2, CADM1, CAV1, CCND2, CST6, EGFR, ESR2, GSTP1, ICAM5, NPY, PGR, PTGS2, RECK, RUNX3, SFRP1, SOX17, SYK, TGFBR2, TNFRSF10D, WIF1, and WRN) ..
  54. Sorber L, Zwaenepoel K, Deschoolmeester V, Roeyen G, Lardon F, Rolfo C, et al. A Comparison of Cell-Free DNA Isolation Kits: Isolation and Quantification of Cell-Free DNA in Plasma. J Mol Diagn. 2017;19:162-168 pubmed publisher
    ..the isolation efficiency of the QIAamp circulating nucleic acid kit (QIA) with four other cfDNA isolation kits: the PME free-circulating DNA Extraction Kit (PME), the Maxwell RSC ccfDNA Plasma Kit (RSC), the EpiQuick Circulating Cell-..
  55. Stubbs M, Laber B, Bode W, Huber R, Jerala R, Lenarcic B, et al. The refined 2.4 A X-ray crystal structure of recombinant human stefin B in complex with the cysteine proteinase papain: a novel type of proteinase inhibitor interaction. EMBO J. 1990;9:1939-47 pubmed
    ..The interaction is dominated by hydrophobic contacts. Inhibition by the cysteine proteinase inhibitors is fundamentally different to that observed for the serine proteinase inhibitors. ..
  56. Assenza G, Benvenga A, Gennaro E, Tombini M, Campana C, Assenza F, et al. A novel c132-134del mutation in Unverricht-Lundborg disease and the review of literature of heterozygous compound patients. Epilepsia. 2017;58:e31-e35 pubmed publisher
    ..or progressive myoclonic epilepsy type 1 (EPM1) is an autosomal recessive disease caused by mutation of the cystatin B gene (CSTB), located on chromosome 21q22.3...
  57. Oortveld M, van Vlijmen Willems I, Kersten F, Cheng T, Verdoes M, van Erp P, et al. Cathepsin B as a potential cystatin M/E target in the mouse hair follicle. FASEB J. 2017;31:4286-4294 pubmed publisher
    Deficiency of the cysteine protease inhibitor cystatin M/E (Cst6) in mice leads to disturbed epidermal cornification, impaired barrier function, and neonatal lethality...
  58. Zhu X, Wan J, Wu Q, Zhao X, Zheng S, Shen R. PARVUS affects aluminium sensitivity by modulating the structure of glucuronoxylan in Arabidopsis thaliana. Plant Cell Environ. 2017;40:1916-1925 pubmed publisher
    ..levels of Al in its roots and cell walls than WT despite having cell wall pectin content and pectin methylesterase (PME) activity similar to those of WT...
  59. Praschberger R, Lowe S, Malintan N, Giachello C, Patel N, Houlden H, et al. Mutations in Membrin/GOSR2 Reveal Stringent Secretory Pathway Demands of Dendritic Growth and Synaptic Integrity. Cell Rep. 2017;21:97-109 pubmed publisher
    ..NSF attachment protein] receptor) protein Membrin (encoded by the GOSR2 gene) cause progressive myoclonus epilepsy (PME). Membrin is a ubiquitous and essential protein mediating ER-to-Golgi membrane fusion...
  60. Rigano M, Lionetti V, Raiola A, Bellincampi D, Barone A. Pectic enzymes as potential enhancers of ascorbic acid production through the D-galacturonate pathway in Solanaceae. Plant Sci. 2018;266:55-63 pubmed publisher
    ..AsA content was studied in relation with pectin methylesterase (PME) activity and the degree of pectin methylesterification (DME)...
  61. Haider M, Habeeb Y, Al Nakkas E, Al Anzi H, Zaki M, Al Tawari A, et al. Lack of an association between candidate gene loci and idiopathic generalized epilepsy in Kuwaiti Arab children. J Biomed Sci. 2005;12:815-8 pubmed
    ..A 2-bp deletion in Cystatin B gene was detected by PCR-RFLP using XcmI...
  62. Hewko S, Cummings G. Performance management in healthcare: a critical analysis. Leadersh Health Serv (Bradf Engl). 2016;29:52-68 pubmed publisher
    ..underlying theoretical assumptions and implications of current micro-level performance management and evaluation (PME) practices, specifically within health-care organizations...
  63. Eze N, Smith L, Lagasse L, Derauf C, Newman E, Arria A, et al. School-Aged Outcomes following Prenatal Methamphetamine Exposure: 7.5-Year Follow-Up from the Infant Development, Environment, and Lifestyle Study. J Pediatr. 2016;170:34-8.e1 pubmed publisher
    To assess the relationship between prenatal methamphetamine exposure (PME) and behavior problems at age 7.5 years and the extent to which early adversity mediated this relationship...
  64. Rajulapati V, Goyal A. Molecular Cloning, Expression and Characterization of Pectin Methylesterase (CtPME) from Clostridium thermocellum. Mol Biotechnol. 2017;59:128-140 pubmed publisher
    Many phytopathogenic micro-organisms such as bacteria and fungi produce pectin methylesterases (PME) during plant invasion. Plants and insects also produce PME to degrade plant cell wall...
  65. Koch F, Berkefeld A. Reactant or reagent? Oxidation of H2 at electronically distinct nickel-thiolate sites [Ni2(?-SR)2]+ and [Ni-SR]. Dalton Trans. 2018;47:10561-10568 pubmed publisher
    ..In this context, the effect of metal containing side-products such as [HNi(PMe3)4]+ on overall H2 oxidation reactivity displayed by Ni-S structures ..
  66. Yüksel C, Tegin C, O Connor L, Du F, Ahat E, Cohen B, et al. Phosphorus magnetic resonance spectroscopy studies in schizophrenia. J Psychiatr Res. 2015;68:157-66 pubmed publisher
    ..Studies that measured (31)P metabolites in SZ patients were included. This search identified 52 studies. Reduced PME and elevated PDE reported in earlier studies were not replicated in several subsequent studies...
  67. Tahmasbian I, Safari Sinegani A. Improving the efficiency of phytoremediation using electrically charged plant and chelating agents. Environ Sci Pollut Res Int. 2016;23:2479-86 pubmed publisher
    ..In a pot experiment using mine soil, poultry manure extract (PME), cow manure extract (CME), and ethylenediaminetetraacetic acid (EDTA) were applied to soil as chelating agents (2 ..
  68. Bandi K, Upadhyay A, Singh A, Jain A. Nano-level monitoring of Er(III) by fabrication of coated graphite electrode based on newly synthesized Schiff base as neutral carrier. Mater Sci Eng C Mater Biol Appl. 2016;62:9-17 pubmed publisher
    ..The performance of the PME based on S2 was compared with CGE. The electrodes exhibit Nernstian slope for Er (III) ion with detection limit 5...
  69. Sonon K, Richardson G, Cornelius J, Kim K, Day N. Developmental pathways from prenatal marijuana exposure to Cannabis Use Disorder in young adulthood. Neurotoxicol Teratol. 2016;58:46-52 pubmed publisher
    Earlier studies reported an association between prenatal marijuana exposure (PME) and cognitive and behavioral problems in the offspring. A recent publication demonstrated the relation between PME and offspring marijuana use at age 22...
  70. Parisi C, Mastoraki S, Markou A, Strati A, Chimonidou M, Georgoulias V, et al. Development and validation of a multiplex methylation specific PCR-coupled liquid bead array for liquid biopsy analysis. Clin Chim Acta. 2016;461:156-64 pubmed publisher
    ..MMSPA) for the simultaneous detection of the methylation status of three tumor and metastasis suppressor genes (CST6, SOX17 and BRMS1) in liquid biopsy material (CTCs, corresponding ctDNA) and paired primary breast tumors...
  71. AlMatar M, Makky E. Cladosporium cladosporioides from the perspectives of medical and biotechnological approaches. 3 Biotech. 2016;6:4 pubmed publisher
    ..p-methylbenzoic acid, ergosterol peroxide (EP) and calphostin C as well as enzymes including pectin methylesterase (PME), polygalacturonase (PG) and chlorpyrifos hydrolase...
  72. Kamal A, Shaikh A, Fida M. Improvement in Peer Assessment Rating scores after nonextraction, premolar extraction, and mandibular incisor extraction treatments in patients with Class I malocclusion. Am J Orthod Dentofacial Orthop. 2017;151:685-690 pubmed publisher
    ..the occlusal characteristics of Class I patients treated with nonextraction (NE), all first premolar extractions (PME), and mandibular incisor extraction (MIE) as assessed by the percentage of improvement in Peer Assessment Rating (..
  73. Unsleber J, Neugebauer J, Morris R. DFT methods applied to answer the question: how accurate is the ligand acidity constant method for estimating the pKa of transition metal hydride complexes MHXL4 when X is varied?. Dalton Trans. 2018;47:2739-2747 pubmed publisher
    ..isomers of iron-group hydride complexes MHXL4 where M = Fe, Ru, Os, L4 = (CO)4 or (PMe2CH2CH2PMe2)2 for a wide range of anionic ligands X...
  74. Pol E, Bjork I. Role of the single cysteine residue, Cys 3, of human and bovine cystatin B (stefin B) in the inhibition of cysteine proteinases. Protein Sci. 2001;10:1729-38 pubmed
    b>Cystatin B is unique among cysteine proteinase inhibitors of the cystatin superfamily in having a free Cys in the N-terminal segment of the proteinase binding region...
  75. Gole B, Huszthy P, Popovic M, Jeruc J, Ardebili Y, Bjerkvig R, et al. The regulation of cysteine cathepsins and cystatins in human gliomas. Int J Cancer. 2012;131:1779-89 pubmed publisher
    ..Moreover, we reveal for the first time that an elevated StefA mRNA level is a highly significant prognostic factor for patient survival. ..
  76. Polajnar M, Zavasnik Bergant T, Skerget K, Vizovišek M, Vidmar R, Fonovic M, et al. Human stefin B role in cell's response to misfolded proteins and autophagy. PLoS ONE. 2014;9:e102500 pubmed publisher
  77. Mancini G, Schot R, de Wit M, de Coo R, Oostenbrink R, Bindels de Heus K, et al. CSTB null mutation associated with microcephaly, early developmental delay, and severe dyskinesia. Neurology. 2016;86:877-8 pubmed publisher
  78. O Brien A, Marshall C, Blaser S, Ray P, Yoon G. Severe neurodegeneration, progressive cerebral volume loss and diffuse hypomyelination associated with a homozygous frameshift mutation in CSTB. Eur J Hum Genet. 2017;25:775-778 pubmed publisher
    Mutations of the cystatin B gene (CSTB; OMIM 601145) are known to cause Unverricht-Lundborg disease or progressive myoclonic epilepsy-1A (EPM1A, MIM #254800)...
  79. Natarajan M, Faujdar H, Mobin S, Stein M, Kaur Ghumaan S. A mononuclear iron carbonyl complex [Fe(?-bdt)(CO)2(PTA)2] with bulky phosphine ligands: a model for the [FeFe] hydrogenase enzyme active site with an inverted redox potential. Dalton Trans. 2017;46:10050-10056 pubmed publisher
    ..Electrochemical investigations of 1 are compared to the closely related [Fe(bdt)(CO)2(PMe3)2] compound...
  80. Canafoglia L, Ferlazzo E, Michelucci R, Striano P, Magaudda A, Gambardella A, et al. Variable course of Unverricht-Lundborg disease: Early prognostic factors. Neurology. 2017;89:1691-1697 pubmed publisher
    ..All of these factors may be genetically determined, but the greater hyperexcitability underlying more severe seizures and myoclonus at onset may also play a role by increasing cell damage due to reduced cystatin B activity.
  81. Ma H, Li J, Canty A, O Hair R. Cluster transformation of [Cu3(μ3-H)(μ3-BH4)((PPh2)2NH)3](BF4) to [Cu3(μ3-H)(μ2,μ1-S2CH)((PPh2)2NH)3](BF4) via reaction with CS2. X-ray structural characterisation and reactivity of cationic clusters explored by multistage mass spectrometry and compu. Dalton Trans. 2017;46:14995-15003 pubmed publisher
    ..of the model system [Cu3(H)(S2CH)LMe2]+ (LMe = (PMe2)2NH = dmpa) provide a feasible mechanism...
  82. Lafreniere R, Rochefort D, Chretien N, Rommens J, Cochius J, Kalviainen R, et al. Unstable insertion in the 5' flanking region of the cystatin B gene is the most common mutation in progressive myoclonus epilepsy type 1, EPM1. Nat Genet. 1997;15:298-302 pubmed
    ..Recently, two mutations in the cystatin B gene (also known as stefin B, STFB) mapping to 21q22...
  83. Pataskar S, Dash D, Brahmachari S. Intramolecular i-motif structure at acidic pH for progressive myoclonus epilepsy (EPM1) repeat d(CCCCGCCCCGCG)n. J Biomol Struct Dyn. 2001;19:307-13 pubmed
    The most common mutation associated with Progressive Myoclonus Epilepsy (EPM1) of Unverricht-Lundberg type is the expansion of a dodecamer repeat, d(CCCCGCCCCGCG)n...
  84. Osawa M, Kaneko M, Horiuchi H, Kitano T, Kawamoto Y, Saitou N, et al. Evolution of the cystatin B gene: implications for the origin of its variable dodecamer tandem repeat in humans. Genomics. 2003;81:78-84 pubmed
    The human cystatin B gene contains a variable number of 12-bp tandem repeats in its promoter region, of which the common alleles contain two or three copies and unusual expansion causes progressive myoclonus epilepsy of the Unverricht-..
  85. Lee M, Yu G, Park S, Cho B, Ahn J, Park H, et al. Identification of cystatin B as a potential serum marker in hepatocellular carcinoma. Clin Cancer Res. 2008;14:1080-9 pubmed publisher
    ..Expression of cystatin B (CSTB) was examined by mRNA expression analysis and immunohistochemistry...
  86. Luciano Montalvo C, Meléndez L. Cystatin B associates with signal transducer and activator of transcription 1 in monocyte-derived and placental macrophages. Placenta. 2009;30:464-7 pubmed publisher
    ..This is the first report to demonstrate that cystatin B interacts with STAT-1 and that the levels of STAT-1 tyrosine phosphorylation (but not serine phosphorylation) ..
  87. Wang X, Gui L, Zhang Y, Zhang J, Shi J, Xu G. Cystatin B is a progression marker of human epithelial ovarian tumors mediated by the TGF-β signaling pathway. Int J Oncol. 2014;44:1099-106 pubmed publisher
    ..b>Cystatin B (CSTB) functions as an inhibitor to suppress intracellular cysteine proteases and has been implicated in several ..
  88. Zhao X, Usdin K. The Repeat Expansion Diseases: The dark side of DNA repair. DNA Repair (Amst). 2015;32:96-105 pubmed publisher
    ..This review will summarize recent findings from patients and from mouse models of these diseases that shed light on how these pathways may interact to cause repeat expansion. ..
  89. Xie H, Li Y, Wang L, Kuang J, Lei Q, Fang W. Why different ligands can control stereochemistry selectivity of Ni-catalyzed Suzuki-Miyaura cross-coupling of benzylic carbamates with arylboronic esters: a mechanistic study. Dalton Trans. 2017;46:13010-13019 pubmed publisher
    ..volume models have been performed to illustrate the origins of reaction stereoselectivity, and the interactions between catalysts and organic moieties control the stereoselectivity for both Ni(PMe3) and Ni(SIMes) catalysts.
  90. Cui P, Wang Q, McCollom S, Manor B, Carroll P, Tomson N. Ring-Size-Modulated Reactivity of Putative Dicobalt-Bridging Nitrides: C-H Activation versus Phosphinimide Formation. Angew Chem Int Ed Engl. 2017;: pubmed publisher
    ..used to target the generation of the bridging nitrido species [(n PDI2 )Co2 (?-N)(PMe3 )2 ]3+ (PDI=2,6-pyridyldiimine; n=2, 3, corresponding to the number of catenated ..
  91. Brouwers B, Schrauwen Hinderling V, Jelenik T, Gemmink A, Sparks L, Havekes B, et al. Exercise training reduces intrahepatic lipid in people with and people without non-alcoholic fatty liver. Am J Physiol Endocrinol Metab. 2017;:ajpendo.00266.2017 pubmed publisher
    ..clamp), hepatic ATP/total phosphorus ratio and hepatic phosphomonoester/phosphodiester (PME/PDE) ratio (phosphorus magnetic resonance spectroscopy) were determined...
  92. Barrett A, Davies M, Grubb A. The place of human gamma-trace (cystatin C) amongst the cysteine proteinase inhibitors. Biochem Biophys Res Commun. 1984;120:631-6 pubmed
    ..The inhibition constants of cystatin C, taken together with its widespread distribution in human tissues and extracellular fluids, suggest that a physiological function could well be the regulation of cysteine proteinase activity. ..