Merops Switch to
Peptidases

Inhibitor

Family Clan Small-molecule
inhibitor SEARCHES BLAST
MEROPS

Other
information

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 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 ONE20, e0316822-e0316822. PubMed  Europe PubMed DOI
  3. 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 Oncol32, PubMed  Europe PubMed DOI  K
    4. 2024
  4. Singh,S. and Hamalainen,R.H.
    The Roles of Cystatin B in the Brain and Pathophysiological Mechanisms of Progressive Myoclonic Epilepsy Type 1
    Cells13, PubMed  Europe PubMed DOI  V
    5. 2023
  5. 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 Commun11, 132-132. PubMed  Europe PubMed DOI
    6. 2022
  6. 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 Immunol132, 104383-104383. PubMed  Europe PubMed DOI  K
    7. 2020
  7. 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 Mede11419-e11419. PubMed  Europe PubMed DOI  M
  8. 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 Sci134, 12-18. PubMed  Europe PubMed DOI
    9. 2019
  9. 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 Neurosci12, 195-195. PubMed  Europe PubMed DOI
  10. Zerovnik,E.
    Possible mechanisms by which stefin B could regulate proteostasis and oxidative stress
    Cells8, PubMed  Europe PubMed DOI
    11. 2018
  11. 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 J59, 798-800. PubMed  Europe PubMed DOI  M
    12. 2016
  12. Chauhan,S. and Tomar,R.S.
    Efficient expression and purification of biologically active human cystatin proteins
    Protein Expr Purif118, 10-17. PubMed  Europe PubMed DOI  E
  13. 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 Disord18, 28-37. PubMed  Europe PubMed DOI  K  M
  14. Mascarenhas,N.M. and Gosavi,S.
    Protein domain-swapping can be a consequence of functional residues
    J Phys Chem B120, 6929-6938. PubMed  Europe PubMed DOI
  15. 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 Neurovirol22, 666-673. PubMed  Europe PubMed DOI
  16. Zerovnik,E.
    Putative alternative functions of human stefin B (cystatin B): binding to amyloid-beta, membranes, and copper
    J Mol Recognit30, PubMed  Europe PubMed DOI
    17. 2015
  17. 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 Rep3, 76-82. PubMed  Europe PubMed DOI  K
  18. 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
    Epilepsia56, e40-e43. PubMed  Europe PubMed DOI
  19. 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 Commun465, 213-217. PubMed  Europe PubMed DOI
    20. 2014
  20. 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 Chem289, 31736-31750. PubMed  Europe PubMed DOI  K
  21. 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 Res12, 111-120. PubMed  Europe PubMed DOI
  22. 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 Neurobiol34, 205-213. PubMed  Europe PubMed DOI
    23. 2013
  23. 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 Biol165, 211-218. PubMed  Europe PubMed DOI
  24. 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 Chem394, 783-790. PubMed  Europe PubMed DOI
  25. 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 Immunol34, 1505-1513. PubMed  Europe PubMed DOI
  26. 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 Rep31, 323-330.
    27. 2012
  27. 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 Dis47, 216-224. PubMed  Europe PubMed DOI  K
  28. 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 Proteomics75, 908-913. PubMed  Europe PubMed DOI
  29. 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 Neurosci5, 94-94. PubMed  Europe PubMed DOI
  30. 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 Res99, 187-190. PubMed  Europe PubMed DOI  M
  31. 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 Biol163, 138-146. PubMed  Europe PubMed DOI
  32. 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 Immunol38, 495-504. PubMed  Europe PubMed DOI
  33. 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 Neurosci5, 93-93. PubMed  Europe PubMed DOI
  34. 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
    Bone51, 1016-1024. PubMed  Europe PubMed DOI
    35. 2011
  35. 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 Med49, 151-155. PubMed  Europe PubMed DOI  A
    36. 2010
  36. 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 Chem285, 10078-10086. PubMed  Europe PubMed DOI
  37. 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 Turk27, 1-11.  M
  38. 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 Pathol177, 2256-2267. PubMed  Europe PubMed DOI
  39. 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 Biotechnol20, 1243-1250. PubMed  Europe PubMed DOI
  40. Xiao,P.P., Hu,Y.H. and Sun,L.
    Scophthalmus maximus cystatin B enhances head kidney macrophage-mediated bacterial killing
    Dev Comp Immunol34, 1237-1241. PubMed  Europe PubMed DOI
    41. 2009
  41. 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 Res15, 1024-1031. PubMed  Europe PubMed DOI
  42. 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 Neurosci29, 5910-5915. PubMed  Europe PubMed DOI
  43. 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
    Proteins74, 425-436. PubMed  Europe PubMed DOI
    44. 2008
  44. 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 Acta1783, 312-322. PubMed  Europe PubMed DOI
  45. Joensuu,T., Lehesjoki,A.E. and Kopra,O.
    Molecular background of EPM1-Unverricht-Lundborg disease
    Epilepsia49, 557-563. PubMed  Europe PubMed DOI  M  V
  46. 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 Res14, 1080-1089. PubMed  Europe PubMed DOI
  47. 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
    Placenta29, 1016-1023. PubMed  Europe PubMed DOI
    48. 2007
  48. 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 Dis25, 675-685. PubMed  Europe PubMed DOI
  49. 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 Biol366, 1569-1579. PubMed  Europe PubMed DOI  S
  50. 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 Genet15, 185-193. PubMed  Europe PubMed DOI
  51. Kaasik,A., Kuum,M., Aonurm,A., Kalda,A., Vaarmann,A. and Zharkovsky,A.
    Seizures, ataxia, and neuronal loss in cystatin B heterozygous mice
    Epilepsia48, 752-757. PubMed  Europe PubMed DOI
  52. 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
    Chembiochem8, 1950-1956. PubMed  Europe PubMed DOI
    53. 2006
  53. 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 Res72, 75-79. PubMed  Europe PubMed DOI
  54. [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 Biol25, 149-157. PubMed  Europe PubMed DOI
  55. 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 J273, 4250-4263. PubMed  Europe PubMed DOI
    56. 2005
  56. [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 Genet13, 208-215. PubMed  Europe PubMed DOI
  57. [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 Lett579, 2149-2155. PubMed  Europe PubMed DOI
  58. [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 Sci14, 2713-2722. PubMed  Europe PubMed DOI  M
  59. 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 Dis20, 104-114. PubMed  Europe PubMed DOI  M
    60. 2003
  60. 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 Chem384, 447-455. PubMed  Europe PubMed DOI
  61. [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 Commun308, 369-374. PubMed  Europe PubMed DOI
  62. [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 Neurobiol56, 315-327. PubMed  Europe PubMed DOI
  63. 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 Genet100, 213-223. PubMed  Europe PubMed DOI
  64. [YEAR:15-7-2003]Lehesjoki,A.E.
    Molecular background of progressive myoclonus epilepsy
    EMBO J22, 3473-3478. PubMed  Europe PubMed DOI
  65. 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
    Genomics81, 78-84. PubMed  Europe PubMed DOI  M
  66. [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 Acta1645, 105-112. PubMed  Europe PubMed DOI
    67. 2002
  67. [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 Genet11, 2941-2950. PubMed  Europe PubMed
  68. Kagitani-Shimono,K., Imai,K., Okamoto,N., Ono,J. and Okada,S.
    Unverricht-Lundborg disease with cystatin B gene abnormalities
    Pediatr Neurol26, 55-60. PubMed  Europe PubMed DOI
  69. Rinne,R., Saukko,P., Jarvinen,M. and Lehesjoki,A.E.
    Reduced cystatin B activity correlates with enhanced cathepsin activity in progressive myoclonus epilepsy
    Ann Med34, 380-385. PubMed  Europe PubMed DOI
  70. 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 Neurol61, 1085-1091. PubMed  Europe PubMed
  71. [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 Acta1594, 1-5. PubMed  Europe PubMed DOI
    72. 2001
  72. [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
    Proteins42, 512-522. PubMed  Europe PubMed DOI
  73. 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 Sci10, 1729-1738. PubMed  Europe PubMed DOI
  74. [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 Res262, 84-94. PubMed  Europe PubMed DOI
    75. 2000
  75. [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
    Gene242, 65-73. PubMed  Europe PubMed DOI
  76. 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
    Eur J Neurosci12, 1687-1695. PubMed  Europe PubMed DOI
    77. 1999
  77. Lalioti,M.D., Scott,H.S. and Antonarakis,S.E.
    Altered spacing of promoter elements due to the dodecamer repeat expansion contributes to reduced expression of the cystatin B gene in EPM1
    Hum Mol Genet8, 1791-1798. PubMed  Europe PubMed DOI
  78. Lehesjoki,A.E. and Koskiniemi,M.
    Progressive myoclonus epilepsy of Unverricht-Lundborg type
    Epilepsia40 Suppl 3, 23-28. PubMed  Europe PubMed
  79. [YEAR:10-8-1999]Pol,E. and Bjork,I.
    Importance of the second binding loop and the C-terminal end of cystatin B (stefin B) for inhibition of cysteine proteinases
    Biochemistry38, 10519-10526. PubMed  Europe PubMed DOI
  80. [YEAR:1-8-1999]Zerovnik,E., Virden,R., Jerala,R., Kroon-Zitko,L., Turk,V. and Waltho,J.P.
    Differences in the effects of TFE on the folding pathways of human stefins A and B
    Proteins36, 205-216. PubMed  Europe PubMed DOI
    81. 1998
  81. Lehesjoki,A.E. and Koskiniemi,M.
    Clinical features and genetics of progressive myoclonus epilepsy of the Univerricht-Lundborg type
    Ann Med30, 474-480. PubMed  Europe PubMed
  82. Pennacchio,L.A., Bouley,D.M., Higgins,K.M., Scott,M.P., Noebels,J.L. and Myers,R.M.
    Progressive ataxia, myoclonic epilepsy and cerebellar apoptosis in cystatin B-deficient mice
    Nat Genet20, 251-258. PubMed  Europe PubMed DOI
    83. 1997
  83. Bespalova,I.N., Adkins,S., Pranzatelli,M. and Burmeister,M.
    Novel cystatin B mutation and diagnostic PCR assay in an Unverricht-Lundborg progressive myoclonus epilepsy patient
    Am J Med Genet74, 467-471. PubMed  Europe PubMed DOI  K  M
  84. Labauge,P., Ouazzani,R., M'Rabet,A., Grid,D., Genton,P., Dravet,C., Chkili,T., Beck,C., Buresi,C., Baldy-Moulinier,M. and Malafosse,A.
    Allelic heterogeneity of Mediterranean myoclonus and the cystatin B gene
    Ann Neurol41, 686-689. PubMed  Europe PubMed DOI
  85. Lafreniere,R.G., Rochefort,D.L., Chretien,N., Rommens,J.M., Cochius,J.I., Kalviainen,R., Nousiainen,U., Patry,G., Farrell,K., Soderfeldt,B., Federico,A., Hale,B.R., Cossio,O.H., Sorensen,T., Pouliot,M.A., Kmiec,T., Uldall,P., Janszky,J., Pranzatelli,M.R., Andermann,F., Andermann,E. and Rouleau,G.A.
    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 Genet15, 298-302. PubMed  Europe PubMed DOI
  86. Lalioti,M.D., Mirotsou,M., Buresi,C., Peitsch,M.C., Rossier,C., Ouazzani,R., Baldy-Moulinier,M., Bottani,A., Malafosse,A. and Antonarakis,S.E.
    Identification of mutations in Cystatin B, the gene responsible for the Unverricht-Lundborg type of progressive myoclonus epilepsy (EPM1)
    Am J Hum Genet60, 342-351. PubMed  Europe PubMed
    87. 1996
  87. Lenarcic,B., Krizaj,I., Zunec,P. and Turk,V.
    Differences in specificity for the interactions of stefins A, B and D with cysteine proteinases
    FEBS Lett395, 113-118. PubMed  Europe PubMed DOI
  88. Pennacchio,L.A. and Myers,R.M.
    Isolation and characterization of the mouse cystatin B gene
    Genome Res6, 1103-1109. PubMed  Europe PubMed
  89. Ritonja,A., Coetzer,T.H., Pike,R.N. and Dennison,C.
    The amino acid sequences, structure comparisons and inhibition kinetics of sheep cathepsin L and sheep stefin B
    Comp Biochem Physiol B Biochem Mol Biol114, 193-198. PubMed  Europe PubMed DOI
    90. 1994
  90. [YEAR:14-2-1994]Turk,B., Colic,A., Stoka,V. and Turk,V.
    Kinetics of inhibition of bovine cathepsin S by bovine stefin B
    FEBS Lett339, 155-159. PubMed  Europe PubMed DOI
    91. 1992
  91. Pike,R.N., Coetzer,T.H. and Dennison,C.
    Proteolytically active complexes of cathepsin L and a cysteine proteinase inhibitor; purification and demonstration of their formation in vitro
    Arch Biochem Biophys294, 623-629. PubMed  Europe PubMed DOI
    92. 1991
  92. Bromme,D., Rinne,R. and Kirschke,H.
    Tight-binding inhibition of cathepsin S by cystatins
    Biomed Biochim Acta50, 631-635. PubMed  Europe PubMed
    93. 1990
  93. Stubbs,M.T., Laber,B., Bode,W., Huber,R., Jerala,R., Lenarcic,B. and Turk,V.
    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 J9, 1939-1947. PubMed  Europe PubMed  S
  94. Thiele,U., Assfalg-Machleidt,I., Machleidt,W. and Auerswald,E.A.
    N-Terminal variants of recombinant stefin B: effect on affinity for papain and cathepsin B
    Biol Chem Hoppe Seyler371 Suppl, 125-136. PubMed  Europe PubMed
    95. 1987
  95. Jarvinen,M., Rinne,A. and Hopsu-Havu,V.K.
    Human cystatins in normal and diseased tissues - a review
    Acta Histochem82, 5-18. PubMed  Europe PubMed DOI
  96. [YEAR:25-8-1986]Abrahamson,M., Barrett,A.J., Salvesen,G. and Grubb,A.
    Isolation of six cysteine proteinase inhibitors from human urine. Their physicochemical and enzyme kinetic properties and concentrations in biological fluids
    J Biol Chem261, 11282-11289. PubMed  Europe PubMed
    97. 1985
  97. Bige,L., Ouali,A. and Valin,C.
    Purification and characterization of a low molecular weight cysteine proteinase inhibitor from bovine muscle
    Biochim Biophys Acta843, 269-275. PubMed  Europe PubMed
  98. Hopsu-Havu,V.K., Joronen,I., Rinne,A. and Jarvinen,M.
    Production of acid and neutral cysteine-proteinase inhibitors by a cultured human skin epithelium cell line
    Arch Dermatol Res277, 452-456. PubMed  Europe PubMed
  99. Ritonja,A., Machleidt,W. and Barrett,A.J.
    Amino acid sequence of the intracellular cysteine proteinase inhibitor cystatin B from human liver
    Biochem Biophys Res Commun131, 1187-1192. PubMed  Europe PubMed
    100. 1984
  100. Davies,M.E. and Barrett,A.J.
    Immunolocalization of human cystatins in neutrophils and lymphocytes
    Histochemistry80, 373-377. PubMed  Europe PubMed
  101. Green,G.D., Kembhavi,A.A., Davies,M.E. and Barrett,A.J.
    Cystatin-like cysteine proteinase inhibitors from human liver
    Biochem J218, 939-946. PubMed  Europe PubMed
  102. Hopsu-Havu,V.K., Joronen,I.A., Jarvinen,M., Rinne,A. and Aalto,M.
    Cysteine proteinase inhibitors produced by mononuclear phagocytes
    Cell Tissue Res236, 161-164. PubMed  Europe PubMed
  103. Kominami,E., Bando,Y., Wakamatsu,N. and Katunuma,N.
    Different tissue distributions of two types of thiol proteinase inhibitors from rat liver and epidermis
    J Biochem96, 1437-1442. PubMed  Europe PubMed
  104. Kominami,E., Tsukahara,T., Ii,K., Hizawa,K. and Katunuma,N.
    Studies on thiol proteinase inhibitors in rat peripheral blood cells
    Biochem Biophys Res Commun123, 816-821. PubMed  Europe PubMed
  105. Nishida,Y., Sumi,H. and Mihara,H.
    A thiol protease inhibitor released from cultured human malignant melanoma cells
    Cancer Res44, 3324-3329. PubMed  Europe PubMed
    106. 1983
  106. Takeda,A., Kobayashi,S. and Samejima,T.
    Isolation and characterization of two thiol proteinase inhibitors of low molecular weight from newborn rat epidermis
    J Biochem94, 811-820. PubMed  Europe PubMed
    107. 1982
  107. Jarvinen,M. and Rinne,A.
    Human spleen cysteineproteinase inhibitor. Purification, fractionation into isoelectric variants and some properties of the variants
    Biochim Biophys Acta708, 210-217. PubMed  Europe PubMed
  108. [YEAR:25-12-1982]Kominami,E., Wakamatsu,N. and Katunuma,N.
    Purification and characterization of thiol proteinase inhibitor from rat liver
    J Biol Chem257, 14648-14652. PubMed  Europe PubMed
  109. Spanier,A.M. and Bird,J.W.
    Endogenous cathepsin B inhibitor activity in normal and myopathic red and white skeletal muscle
    Muscle Nerve5, 313-320. PubMed  Europe PubMed DOI
    110. 1981
  110. [YEAR:29-6-1981]Hirado,M., Iwata,D., Niinobe,M. and Fujii,S.
    Purification and properties of thiol protease inhibitor from rat liver cytosol
    Biochim Biophys Acta669, 21-27. PubMed  Europe PubMed
  111. [YEAR:31-3-1981]Kominami,E., Wakamatsu,N. and Katunuma,N.
    Endogenous thiol protease inhibitor from rat liver
    Biochem Biophys Res Commun99, 568-575. PubMed  Europe PubMed

feedback
Page created 8-September-2023

EMBL-EBI logo

© 2025 EBI
Terms of use