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Literature for inhibitor I25.003: cystatin B

Summary Gene structure Alignment Tree Sequences Sequence features Distribution Structure Literature

(Topics flags: S Structure, A Assay, M Mutation, K Knockout, E Expression, V Review. To select only the references relevant to a single topic, click the link above. See explanation.)

    2025
  1. Karger,A.B. and Shlipak,M.G.
    Glomerular Filtration Rate (GFR) Estimation with Cystatin C-Past, Present, and Future
    Clin Chem (2025) PubMed  Europe PubMed DOI  V
  2. Wu,Y., Cleverley,K. and Wiseman,F.K.
    Reduction of Cystatin B results in increased cathepsin B activity in disomic but not Trisomy 21 human cellular and mouse models
    PLoS ONE (2025) 20, e0316822-e0316822. PubMed  Europe PubMed DOI  PMC  EPMC
  3. Zaben,B., Tuqan,A.R., Hammad,O., Jweiles,R., Awad,S., Barabrah,A.M., Atawneh,O. and Jo'beh,S.
    Infantile-Onset Unverricht-Lundborg Disease: A Unique Case With Severe Neurological Impairment and Positive Family History
    J Investig Med High Impact Case Rep (2025) 13, 23247096251407837-23247096251407837. PubMed  Europe PubMed DOI  PMC  EPMC
  4. Zhang,D., Sun,B.Y., Wu,J.F., Wang,Z.T., Zheng,S.S., Sun,G.Q., Gao,X.K., Zhou,J., Fan,J., Hu,B., Qiu,S.J. and Zhang,B.H.
    Cystatin B Promotes the Proliferation, Migration, and Invasion of Intrahepatic Cholangiocarcinoma
    Curr Oncol (2025) 32 PubMed  Europe PubMed DOI  PMC  EPMC  K
    5. 2024
  5. Singh,S. and Hamalainen,R.H.
    The Roles of Cystatin B in the Brain and Pathophysiological Mechanisms of Progressive Myoclonic Epilepsy Type 1
    Cells (2024) 13 PubMed  Europe PubMed DOI  PMC  EPMC  V
    6. 2023
  6. Wu,Y., Mumford,P., Noy,S., Cleverley,K., Mrzyglod,A., Luo,D., van Dalen,F., Verdoes,M., Fisher,E.M.C. and Wiseman,F.K.
    Cathepsin B abundance, activity and microglial localisation in Alzheimer's disease-Down syndrome and early onset Alzheimer's disease; the role of elevated cystatin B
    Acta Neuropathol Commun (2023) 11, 132-132. PubMed  Europe PubMed DOI  PMC  EPMC
    7. 2022
  7. Wei,S., Han,H., Xu,S., Huang,Y., Wei,J. and Qin,Q.
    Identification and functional characterization of Cystatin B in orange-spotted grouper, Epinephelus coioides
    Dev Comp Immunol (2022) 132, 104383-104383. PubMed  Europe PubMed DOI  K
    8. 2020
  8. Di Matteo,F., Pipicelli,F., Kyrousi,C., Tovecci,I., Penna,E., Crispino,M., Chambery,A., Russo,R., Ayo-Martin,A.C., Giordano,M., Hoffmann,A., Ciusani,E., Canafoglia,L., Gotz,M., Di Giaimo,R. and Cappello,S.
    Cystatin B is essential for proliferation and interneuron migration in individuals with EPM1 epilepsy
    EMBO Mol Med (2020) , e11419-e11419. PubMed  Europe PubMed DOI  M
  9. Gordin,E., Gordin,D., Viitanen,S., Szlosek,D., Coyne,M., Farace,G., Murphy,R., Quinn,J., Yerramilli,M., Yerramilli,M. and Spillmann,T.
    Urinary clusterin and cystatin B as biomarkers of tubular injury in dogs following envenomation by the European adder
    Res Vet Sci (2020) 134, 12-18. PubMed  Europe PubMed DOI
    10. 2019
  10. Penna,E., Cerciello,A., Chambery,A., Russo,R., Cernilogar,F.M., Pedone,E.M., Perrone-Capano,C., Cappello,S., Di Giaimo,R. and Crispino,M.
    Cystatin B involvement in synapse physiology of rodent brains and human cerebral organoids
    Front Mol Neurosci (2019) 12, 195-195. PubMed  Europe PubMed DOI  PMC  EPMC
  11. Zerovnik,E.
    Possible mechanisms by which stefin B could regulate proteostasis and oxidative stress
    Cells (2019) 8 PubMed  Europe PubMed DOI
    12. 2018
  12. Kim,K.H., Song,J.S., Park,C.W., Ki,C.S. and Heo,K.
    First molecular diagnosis of a patient with Unverricht-Lundborg disease in Korea
    Yonsei Med J (2018) 59, 798-800. PubMed  Europe PubMed DOI  PMC  EPMC  M
    13. 2016
  13. Chauhan,S. and Tomar,R.S.
    Efficient expression and purification of biologically active human cystatin proteins
    Protein Expr Purif (2016) 118, 10-17. PubMed  Europe PubMed DOI  E
  14. Crespel,A., Ferlazzo,E., Franceschetti,S., Genton,P., Gouider,R., Kalviainen,R., Korja,M., Lehtinen,M.K., Mervaala,E., Simonato,M. and Vaarmann,A.
    Unverricht-Lundborg disease
    Epileptic Disord (2016) 18, 28-37. PubMed  Europe PubMed DOI  K  M
  15. Mascarenhas,N.M. and Gosavi,S.
    Protein domain-swapping can be a consequence of functional residues
    J Phys Chem B (2016) 120, 6929-6938. PubMed  Europe PubMed DOI
  16. Rivera,L.E., Kraiselburd,E. and Melendez,L.M.
    Cystatin B and HIV regulate the STAT-1 signaling circuit in HIV-infected and INF-beta-treated human macrophages
    J Neurovirol (2016) 22, 666-673. PubMed  Europe PubMed DOI
  17. Zerovnik,E.
    Putative alternative functions of human stefin B (cystatin B): binding to amyloid-beta, membranes, and copper
    J Mol Recognit (2016) 30 PubMed  Europe PubMed DOI
    18. 2015
  18. Manninen,O., Puolakkainen,T., Lehto,J., Harittu,E., Kallonen,A., Peura,M., Laitala-Leinonen,T., Kopra,O., Kiviranta,R. and Lehesjoki,A.-E.
    Impaired osteoclast homeostasis in the cystatin B-deficient mouse model of progressive myoclonus epilepsy
    Bone Rep (2015) 3, 76-82. PubMed  Europe PubMed DOI  K
  19. Mumoli,L., Tarantino,P., Michelucci,R., Bianchi,A., Labate,A., Franceschetti,S., Marini,C., Striano,P., Gagliardi,M., Ferlazzo,E., Sofia,V., Pennese,L., Annesi,G., Aguglia,U., Guerrini,R., Zara,F. and Gambardella,A.
    No evidence of a role for cystatin B gene in juvenile myoclonic epilepsy
    Epilepsia (2015) 56, e40-e43. PubMed  Europe PubMed DOI
  20. Zeleznik,T.Z., Kadin,A., Turk,V. and Dolenc,I.
    Aspartic cathepsin D degrades the cytosolic cysteine cathepsin inhibitor stefin B in the cells
    Biochem Biophys Res Commun (2015) 465, 213-217. PubMed  Europe PubMed DOI
    21. 2014
  21. Maher,K., Jeric Kokelj,B., Butinar,M., Mikhaylov,G., Mancek-Keber,M., Stoka,V., Vasiljeva,O., Turk,B., Grigoryev,S.A. and Kopitar-Jerala,N.
    A role for stefin B (cystatin B) in inflammation and endotoxemia
    J Biol Chem (2014) 289, 31736-31750. PubMed  Europe PubMed DOI  K
  22. Rivera,L.E., Colon,K., Cantres-Rosario,Y.M., Zenon,F.M. and Melendez,L.M.
    Macrophage derived cystatin B/cathepsin B in HIV replication and neuropathogenesis
    Curr HIV Res (2014) 12, 111-120. PubMed  Europe PubMed DOI
  23. Ulbrich,L., Cozzolino,M., Marini,E.S., Amori,I., De Jaco,A., Carri,M.T. and Augusti-Tocco,G.
    Cystatin B and SOD1: protein-protein interaction and possible relation to neurodegeneration
    Cell Mol Neurobiol (2014) 34, 205-213. PubMed  Europe PubMed DOI
    24. 2013
  24. Ahn,S.J., Bak,H.J., Park,J.H., Kim,S.A., Kim,N.Y., Lee,J.Y., Sung,J.H., Jeon,S.J., Chung,J.K. and Lee,H.H.
    Olive flounder (Paralichthys olivaceus) cystatin B: cloning, tissue distribution, expression and inhibitory profile of piscine cystatin B
    Comp Biochem Physiol B Biochem Mol Biol (2013) 165, 211-218. PubMed  Europe PubMed DOI
  25. Polajnar,M., Vidmar,R., Vizovisek,M., Fonovic,M., Kopitar-Jerala,N. and Zerovnik,E.
    Influence of partial unfolding and aggregation of human stefin B (cystatin B) EPM1 mutants G50E and Q71P on selective cleavages by cathepsins B and S
    Biol Chem (2013) 394, 783-790. PubMed  Europe PubMed DOI
  26. Premachandra,H.K., Elvitigala,D.A., Whang,I., Kim,E., De Zoysa,M., Lim,B.S., Yeo,S.Y., Kim,S., Park,M.A., Park,H.C. and Lee,J.
    Expression profile of cystatin B ortholog from Manila clam (Ruditapes philippinarum) in host pathology with respect to its structural and functional properties
    Fish Shellfish Immunol (2013) 34, 1505-1513. PubMed  Europe PubMed DOI
  27. Urbanski,P., Pierzchala,M., Terman,A., Kamyczek,M., Kapelanaski,W., Bocian,M., Roszczyk,A., Rozycki,M. and Kuryl,J.
    The effect of cystatin B gene (CSTB) on productive traits in pigs
    Anim Sci Pap Rep (2013) 31, 323-330.
    28. 2012
  28. Buzzi,A., Chikhladze,M., Falcicchia,C., Paradiso,B., Lanza,G., Soukupova,M., Marti,M., Morari,M., Franceschetti,S. and Simonato,M.
    Loss of cortical GABA terminals in Unverricht-Lundborg disease
    Neurobiol Dis (2012) 47, 216-224. PubMed  Europe PubMed DOI  K
  29. Cabras,T., Manconi,B., Iavarone,F., Fanali,C., Nemolato,S., Fiorita,A., Scarano,E., Passali,G.C., Manni,A., Cordaro,M., Paludetti,G., Faa,G., Messana,I. and Castagnola,M.
    RP-HPLC-ESI-MS evidenced that salivary cystatin B is detectable in adult human whole saliva mostly as S-modified derivatives: S-Glutathionyl, S-cysteinyl and S-S 2-mer
    J Proteomics (2012) 75, 908-913. PubMed  Europe PubMed DOI
  30. Paramore,R., Morgan,G.J., Davis,P.J., Sharma,C.A., Hounslow,A., Taler-Vercic,A., Zerovnik,E., Waltho,J.P., Cliff,M.J. and Staniforth,R.A.
    Mapping local structural perturbations in the native state of stefin B (cystatin B) under amyloid forming conditions
    Front Mol Neurosci (2012) 5, 94-94. PubMed  Europe PubMed DOI  PMC  EPMC
  31. Pinto,E., Freitas,J., Duarte,A.J., Ribeiro,I., Ribeiro,D., Lima,J.L., Chaves,J. and Amaral,O.
    Unverricht-Lundborg disease: homozygosity for a new splicing mutation in the cystatin B gene
    Epilepsy Res (2012) 99, 187-190. PubMed  Europe PubMed DOI  M
  32. Premachandra,H.K., Whang,I., Lee,Y.D., Lee,S., De Zoysa,M., Oh,M.J., Jung,S.J., Lim,B.S., Noh,J.K., Park,H.C. and Lee,J.
    Cystatin B homolog from rock bream Oplegnathus fasciatus: genomic characterization, transcriptional profiling and protease-inhibitory activity of recombinant protein
    Comp Biochem Physiol B Biochem Mol Biol (2012) 163, 138-146. PubMed  Europe PubMed DOI
  33. Premachandra,H.K., Wan,Q., Elvitigala,D.A., De Zoysa,M., Choi,C.Y., Whang,I. and Lee,J.
    Genomic characterization and expression profiles upon bacterial infection of a novel cystatin B homologue from disk abalone (Haliotis discus discus)
    Dev Comp Immunol (2012) 38, 495-504. PubMed  Europe PubMed DOI
  34. Sun,T., Turk,V., Turk,B. and Kopitar-Jerala,N.
    Increased expression of stefin B in the nucleus of T98G astrocytoma cells delays caspase activation
    Front Mol Neurosci (2012) 5, 93-93. PubMed  Europe PubMed DOI
  35. Suoranta,S., Manninen,H., Koskenkorva,P., Kononen,M., Laitinen,R., Lehesjoki,A.E., Kalviainen,R. and Vanninen,R.
    Thickened skull, scoliosis and other skeletal findings in Unverricht-Lundborg disease link cystatin B function to bone metabolism
    Bone (2012) 51, 1016-1024. PubMed  Europe PubMed DOI
    36. 2011
  36. Ji,N.Y., Kang,Y.H., Park,M.Y., Lee,C.I., Kim,M.K., Kim,D.G., Kim,J.W. and Song,E.Y.
    Development of a fluorescent microsphere immunoassay for cystatin B (CSTB) in serum of patients with hepatocellular carcinoma
    Clin Chem Lab Med (2011) 49, 151-155. PubMed  Europe PubMed DOI  A
    37. 2010
  37. Ceru,S., Konjar,S., Maher,K., Repnik,U., Krizaj,I., Bencina,M., Renko,M., Nepveu,A., Zerovnik,E., Turk,B. and Kopitar-Jerala,N.
    Stefin B interacts with histones and cathepsin L in the nucleus
    J Biol Chem (2010) 285, 10078-10086. PubMed  Europe PubMed DOI
  38. Erdinc,O.O., Joensuu,T., Ilgen-Uslu,F., Bebek,N., Ozkara,C., Tutkavul,K., Gunduz,A., Lehesjoki,A.E. and Baykan,B.
    Unverricht-Lundborg disease in Turkey: delineating the phenotype between cystatin B mutation positive and negative cases
    J Neurol Sci Turk (2010) 27, 1-11.  M
  39. Kaur,G., Mohan,P., Pawlik,M., Derosa,S., Fajiculay,J., Che,S., Grubb,A., Ginsberg,S.D., Nixon,R.A. and Levy,E.
    Cystatin C rescues degenerating neurons in a cystatin B-knockout mouse model of progressive myoclonus epilepsy
    Am J Pathol (2010) 177, 2256-2267. PubMed  Europe PubMed DOI
  40. Li,S., Guo,D., Zhao,B., Ye,J., Tian,J., Ren,W., Ju,Y., Cui,P. and Li,R.
    Cloning and expression of cDNA encoding a cysteine protease inhibitor from clamworm and its possible use in managing Anoplophora glabripennis Motschulsky (Coleoptera: Cerambycidae)
    J Microbiol Biotechnol (2010) 20, 1243-1250. PubMed  Europe PubMed DOI
  41. Xiao,P.P., Hu,Y.H. and Sun,L.
    Scophthalmus maximus cystatin B enhances head kidney macrophage-mediated bacterial killing
    Dev Comp Immunol (2010) 34, 1237-1241. PubMed  Europe PubMed DOI
    42. 2009
  42. Feldman,A.S., Banyard,J., Wu,C.L., McDougal,W.S. and Zetter,B.R.
    Cystatin B as a tissue and urinary biomarker of bladder cancer recurrence and disease progression
    Clin Cancer Res (2009) 15, 1024-1031. PubMed  Europe PubMed DOI  PMC  EPMC
  43. Lehtinen,M.K., Tegelberg,S., Schipper,H., Su,H., Zukor,H., Manninen,O., Kopra,O., Joensuu,T., Hakala,P., Bonni,A. and Lehesjoki,A.E.
    Cystatin B deficiency sensitizes neurons to oxidative stress in progressive myoclonus epilepsy, EPM1
    J Neurosci (2009) 29, 5910-5915. PubMed  Europe PubMed DOI  PMC  EPMC
  44. Skerget,K., Vilfan,A., Pompe-Novak,M., Turk,V., Waltho,J.P., Turk,D. and Zerovnik,E.
    The mechanism of amyloid-fibril formation by stefin B: temperature and protein concentration dependence of the rates
    Proteins (2009) 74, 425-436. PubMed  Europe PubMed DOI
    45. 2008
  45. Cipollini,E., Riccio,M., Di Giaimo,R., Dal Piaz,F., Pulice,G., Catania,S., Caldarelli,I., Dembic,M., Santi,S. and Melli,M.
    Cystatin B and its EPM1 mutants are polymeric and aggregate prone in vivo
    Biochim Biophys Acta (2008) 1783, 312-322. PubMed  Europe PubMed DOI
  46. Joensuu,T., Lehesjoki,A.E. and Kopra,O.
    Molecular background of EPM1-Unverricht-Lundborg disease
    Epilepsia (2008) 49, 557-563. PubMed  Europe PubMed DOI  M  V
  47. Lee,M.J., Yu,G.R., Park,S.H., Cho,B.H., Ahn,J.S., Park,H.J., Song,E.Y. and Kim,D.G.
    Identification of cystatin B as a potential serum marker in hepatocellular carcinoma
    Clin Cancer Res (2008) 14, 1080-1089. PubMed  Europe PubMed DOI
  48. Luciano-Montalvo,C., Ciborowski,P., Duan,F., Gendelman,H.E. and Melendez,L.M.
    Proteomic analyses associate cystatin B with restricted HIV-1 replication in placental macrophages
    Placenta (2008) 29, 1016-1023. PubMed  Europe PubMed DOI
    49. 2007
  49. Franceschetti,S., Sancini,G., Buzzi,A., Zucchini,S., Paradiso,B., Magnaghi,G., Frassoni,C., Chikhladze,M., Avanzini,G. and Simonato,M.
    A pathogenetic hypothesis of Unverricht-Lundborg disease onset and progression
    Neurobiol Dis (2007) 25, 675-685. PubMed  Europe PubMed DOI
  50. Jenko Kokalj,S., Guncar,G., Stern,I., Morgan,G., Rabzelj,S., Kenig,M., Staniforth,R.A., Waltho,J.P., Zerovnik,E. and Turk,D.
    Essential role of proline isomerization in stefin B tetramer formation
    J Mol Biol (2007) 366, 1569-1579. PubMed  Europe PubMed DOI  S
  51. Joensuu,T., Kuronen,M., Alakurtti,K., Tegelberg,S., Hakala,P., Aalto,A., Huopaniemi,L., Aula,N., Michellucci,R., Eriksson,K. and Lehesjoki,A.E.
    Cystatin B: mutation detection, alternative splicing and expression in progressive myclonus epilepsy of Unverricht-Lundborg type (EPM1) patients
    Eur J Hum Genet (2007) 15, 185-193. PubMed  Europe PubMed DOI
  52. Kaasik,A., Kuum,M., Aonurm,A., Kalda,A., Vaarmann,A. and Zharkovsky,A.
    Seizures, ataxia, and neuronal loss in cystatin B heterozygous mice
    Epilepsia (2007) 48, 752-757. PubMed  Europe PubMed DOI
  53. van Zoelen,D.J., Egmond,M.R., Pieters,R.J. and Liskamp,R.M.
    Synthesis and evaluation of TAC-based inhibitors of papain as mimics of cystatin B
    Chembiochem (2007) 8, 1950-1956. PubMed  Europe PubMed DOI
    54. 2006
  54. Andrade,D.M., Scherer,S.W. and Minassian,B.A.
    Protein therapy for Unverricht-Lundborg disease using cystatin B transduction by TAT-PTD. Is it that simple?
    Epilepsy Res (2006) 72, 75-79. PubMed  Europe PubMed DOI
  55. [YEAR:28-11-2006]Laitala-Leinonen,T., Rinne,R., Saukko,P., Vaananen,H.K. and Rinne,A.
    Cystatin B as an intracellular modulator of bone resorption
    Matrix Biol (28-11-2006) 25, 149-157. PubMed  Europe PubMed DOI
  56. Zerovnik,E., Skerget,K., Tusek-Znidaric,M., Loeschner,C., Brazier,M.W. and Brown,D.R.
    High affinity copper binding by stefin B (cystatin B) and its role in the inhibition of amyloid fibrillation
    FEBS J (2006) 273, 4250-4263. PubMed  Europe PubMed DOI
    57. 2005
  57. [YEAR:13-10-2005]Alakurtti,K., Weber,E., Rinne,R., Theil,G., de Haan,G.J., Lindhout,D., Salmikangas,P., Saukko,P., Lahtinen,U. and Lehesjoki,A.E.
    Loss of lysosomal association of cystatin B proteins representing progressive myoclonus epilepsy, EPM1, mutations
    Eur J Hum Genet (13-10-2005) 13, 208-215. PubMed  Europe PubMed DOI
  58. [YEAR:11-4-2005]Kopitar-Jerala,N., Schweiger,A., Myers,R.M., Turk,V. and Turk,B.
    Sensitization of stefin B-deficient thymocytes towards staurosporin-induced apoptosis is independent of cysteine cathepsins
    FEBS Lett (11-4-2005) 579, 2149-2155. PubMed  Europe PubMed DOI
  59. [YEAR:9-9-2005]Rabzelj,S., Turk,V. and Zerovnik,E.
    In vitro study of stability and amyloid-fibril formation of two mutants of human stefin B (cystatin B) occurring in patients with EPM1
    Protein Sci (9-9-2005) 14, 2713-2722. PubMed  Europe PubMed DOI  PMC  EPMC  M
  60. Riccio,M., Santi,S., Dembic,M., Di Giaimo,R., Cipollini,E., Costantino-Ceccarini,E., Ambrosetti,D., Maraldi,N.M. and Melli,M.
    Cell-specific expression of the epm1 (cystatin B) gene in developing rat cerebellum
    Neurobiol Dis (2005) 20, 104-114. PubMed  Europe PubMed DOI  M
    61. 2003
  61. Bervar,A., Zajc,I., Sever,N., Katunuma,N., Sloane,B.F. and Lah,T.T.
    Invasiveness of transformed human breast epithelial cell lines is related to cathepsin B and inhibited by cysteine proteinase inhibitors
    Biol Chem (2003) 384, 447-455. PubMed  Europe PubMed DOI
  62. [YEAR:22-8-2003]Brannvall,K., Hjelm,H., Korhonen,L., Lahtinen,U., Lehesjoki,A.E. and Lindholm,D.
    Cystatin-B is expressed by neural stem cells and by differentiated neurons and astrocytes
    Biochem Biophys Res Commun (22-8-2003) 308, 369-374. PubMed  Europe PubMed DOI
  63. [YEAR:15-9-2003]Houseweart,M.K., Pennacchio,L.A., Vilaythong,A., Peters,C., Noebels,J.L. and Myers,R.M.
    Cathepsin B but not cathepsins L or S contributes to the pathogenesis of Unverricht-Lundborg progressive myoclonus epilepsy (EPM1)
    J Neurobiol (15-9-2003) 56, 315-327. PubMed  Europe PubMed DOI
  64. Lalioti,M.D., Antonarakis,S.E. and Scott,H.S.
    The epilepsy, the protease inhibitor and the dodecamer: progressive myoclonus epilepsy, cystatin b and a 12-mer repeat expansion
    Cytogenet Cell Genet (2003) 100, 213-223. PubMed  Europe PubMed DOI
  65. [YEAR:15-7-2003]Lehesjoki,A.E.
    Molecular background of progressive myoclonus epilepsy
    EMBO J (15-7-2003) 22, 3473-3478. PubMed  Europe PubMed DOI  PMC  EPMC
  66. Osawa,M., Kaneko,M., Horiuchi,H., Kitano,T., Kawamoto,Y., Saitou,N. and Umetsu,K.
    Evolution of the cystatin B gene: implications for the origin of its variable dodecamer tandem repeat in humans
    Genomics (2003) 81, 78-84. PubMed  Europe PubMed DOI  M
  67. [YEAR:31-1-2003]Pol,E. and Bjork,I.
    Contributions of individual residues in the N-terminal region of cystatin B (stefin B) to inhibition of cysteine proteinases
    Biochim Biophys Acta (31-1-2003) 1645, 105-112. PubMed  Europe PubMed DOI
    68. 2002
  68. [YEAR:1-11-2002]Di Giaimo,R., Riccio,M., Santi,S., Galeotti,C., Ambrosetti,D.C. and Melli,M.
    New insights into the molecular basis of progressive myoclonus epilepsy: a multiprotein complex with cystatin B
    Hum Mol Genet (1-11-2002) 11, 2941-2950. PubMed  Europe PubMed
  69. Kagitani-Shimono,K., Imai,K., Okamoto,N., Ono,J. and Okada,S.
    Unverricht-Lundborg disease with cystatin B gene abnormalities
    Pediatr Neurol (2002) 26, 55-60. PubMed  Europe PubMed DOI
  70. Rinne,R., Saukko,P., Jarvinen,M. and Lehesjoki,A.E.
    Reduced cystatin B activity correlates with enhanced cathepsin activity in progressive myoclonus epilepsy
    Ann Med (2002) 34, 380-385. PubMed  Europe PubMed DOI
  71. Shannon,P., Pennacchio,L.A., Houseweart,M.K., Minassian,B.A. and Myers,R.M.
    Neuropathological changes in a mouse model of progressive myoclonus epilepsy: cystatin B deficiency and Unverricht-Lundborg disease
    J Neuropathol Exp Neurol (2002) 61, 1085-1091. PubMed  Europe PubMed
  72. [YEAR:31-1-2002]Zerovnik,E., Pompe-Novak,M., Skarabot,M., Ravnikar,M., Musevic,I. and Turk,V.
    Human stefin B readily forms amyloid fibrils in vitro
    Biochim Biophys Acta (31-1-2002) 1594, 1-5. PubMed  Europe PubMed DOI
    73. 2001
  73. [YEAR:1-3-2001]Kenig,M., Jerala,R., Kroon-Zitko,L., Turk,V. and Zerovnik,E.
    Major differences in stability and dimerization properties of two chimeric mutants of human stefins
    Proteins (1-3-2001) 42, 512-522. PubMed  Europe PubMed DOI
  74. Pol,E. and 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, 1729-1738. PubMed  Europe PubMed DOI  PMC  EPMC
  75. [YEAR:15-1-2001]Riccio,M., Di Giaimo,R., Pianetti,S., Palmieri,P.P., Melli,M. and Santi,S.
    Nuclear localization of cystatin B, the cathepsin inhibitor implicated in myoclonus epilepsy (EPM1)
    Exp Cell Res (15-1-2001) 262, 84-94. PubMed  Europe PubMed DOI
    76. 2000
  76. [YEAR:25-1-2000]Alakurtti,K., Virtaneva,K., Joensuu,T., Palvimo,J.J. and Lehesjoki,A.E.
    Characterization of the cystatin B gene promoter harboring the dodecamer repeat expanded in progressive myoclonus epilepsy, EPM1
    Gene (25-1-2000) 242, 65-73. PubMed  Europe PubMed DOI
  77. D'Amato,E., Kokaia,Z., Nanobashvili,A., Reeben,M., Lehesjoki,A.E., Saarma,M. and Lindvall,O.
    Seizures induce widespread upregulation of cystatin B, the gene mutated in progressive myoclonus epilepsy, in rat forebrain neurons
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