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Literature for peptidase S53.003: tripeptidyl-peptidase I

Summary Gene structure Alignment Tree Sequences Sequence features Distribution Structure Literature Substrates

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

    2024
  1. Wawrzynski,J., Martinez,A.R., Thompson,D.A., Ram,D., Bowman,R., Whiteley,R., Gan,C., Harding,L., Mortensen,A., Mills,P., Gissen,P. and Henderson,R.H.
    First in man study of intravitreal tripeptidyl peptidase 1 for CLN2 retinopathy
    Eye (Lond)38, 1176-1182. PubMed  Europe PubMed DOI
    2. 2023
  2. El-Hage,N., Haney,M.J., Zhao,Y., Rodriguez,M., Wu,Z., Liu,M., Swain,C.J., Yuan,H. and Batrakova,E.V.
    Extracellular Vesicles Released by Genetically Modified Macrophages Activate Autophagy and Produce Potent Neuroprotection in Mouse Model of Lysosomal Storage Disorder, Batten Disease
    Cells12, PubMed  Europe PubMed DOI
  3. Liu,R.H., Wang,X.Y., Jia,Y.Y., Wang,X.C., Xia,M., Nie,Q., Guo,J. and Kong,Q.X.
    Compound heterozygous mutations in tripeptidyl peptidase 1 cause rare autosomal recessive spinocerebellar ataxia type 7: A case report
    World J Clin Cases11, 6618-6623. PubMed  Europe PubMed DOI
  4. Steigerwald,C., Borsuk,J., Pappas,J., Galey,M., Scott,A., Devaney,J.M., Miller,D.E. and Abreu,N.J.
    CLN2 disease resulting from a novel homozygous deep intronic splice variant in TPP1 discovered using long-read sequencing
    Mol Genet Metab140, 107713-107713. PubMed  Europe PubMed DOI
    5. 2022
  5. Baranzehi,T., Kordi-Tamandani,D.M., Najafi,M., Khajeh,A. and Schmidts,M.
    Identification of a TPP1 Q278X Mutation in an Iranian Patient with Neuronal Ceroid Lipofuscinosis 2: Literature Review and Mutations Update
    J Clin Med11, PubMed  Europe PubMed DOI
    6. 2021
  6. Atallah,I., Quinodoz,M., Campos-Xavier,B., Peter,V.G., Fouriki,A., Bonvin,C., Bottani,A., Kumps,C., Angelini,F., Bellutti Enders,F., Christen-Zaech,S., Rizzi,M., Renella,R., Beck-Popovic,M., Poloni,C., Frossard,V., Blouin,J.L., Rivolta,C., Riccio,O., Candotti,F., Hofer,M., Unger,S. and Superti-Furga,A.
    Immune deficiency, autoimmune disease and intellectual disability: A pleiotropic disorder caused by biallelic variants in the TPP2 gene
    Clin Genet PubMed  Europe PubMed DOI  M
    7. 2020
  7. de Los Reyes,E., Lehwald,L., Augustine,E.F., Berry-Kravis,E., Butler,K., Cormier,N., Demarest,S., Lu,S., Madden,J., Olaya,J., See,S., Vierhile,A., Wheless,J.W., Yang,A., Cohen-Pfeffer,J., Chu,D., Leal-Pardinas,F. and Wang,R.Y.
    Intracerebroventricular Cerliponase Alfa for Neuronal Ceroid Lipofuscinosis Type 2 Disease: Clinical Practice Considerations From US Clinics
    Pediatr Neurol PubMed  Europe PubMed DOI
  8. Kim,A., Grover,A., Hammon,K., de Hart,G., Slasor,P., Cherukuri,A., Ajayi,T., Jacoby,D., Schulz,A., Specchio,N., de Los Reyes,E., Gissen,P. and Henshaw,J.W.
    Clinical Pharmacokinetics and Pharmacodynamics of Cerliponase Alfa, Enzyme Replacement Therapy for CLN2 Disease by Intracerebroventricular Administration
    Clin Transl Sci PubMed  Europe PubMed DOI
  9. Lewis,G., Morrill,A.M., Conway-Allen,S.L. and Kim,B.
    Review of cerliponase alfa: recombinant human enzyme replacement therapy for late-infantile neuronal ceroid lipofuscinosis type 2
    J Child Neurol35, 348-353. PubMed  Europe PubMed DOI  U
  10. Maeser,S., Petre,B.A., Ion,L., Rawer,S., Kohlschutter,A., Santorelli,F.M., Simonati,A., Schulz,A. and Przybylski,M.
    Enzymatic diagnosis of neuronal lipofuscinoses in dried blood spots using substrates for concomitant tandem mass spectrometry and fluorimetry
    J Mass Spectrome4675-e4675. PubMed  Europe PubMed DOI
  11. Santos,L.L., Ling,C.K. and Dimitriadis,E.
    Tripeptidyl peptidase I promotes human endometrial epithelial cell adhesive capacity implying a role in receptivity
    Reprod Biol Endocrinol18, 124-124. PubMed  Europe PubMed DOI  K
  12. Whiting,R.E.H., Robinson,G.O., Ota-Kuroki,J., Lim,S., Castaner,L.J., Jensen,C.A., Kowal,J., Nguyen,A., Corado,C., O'Neill,C.A. and Katz,M.L.
    Intravitreal enzyme replacement inhibits progression of retinal degeneration in canine CLN2 neuronal ceroid lipofuscinosis
    Exp Eye Res108135-108135. PubMed  Europe PubMed DOI
    13. 2019
  13. Chakrabarti,S., Chandra,S., Roy,A., Dasarathi,S., Kundu,M. and Pahan,K.
    Upregulation of tripeptidyl-peptidase 1 by 3-hydroxy-(2,2)-dimethyl butyrate, a brain endogenous ligand of PPARalpha: Implications for late-infantile Batten disease therapy
    Neurobiol Dis127, 362-373. PubMed  Europe PubMed DOI
  14. Domowicz,M.S., Chan,W.C., Claudio-Vazquez,P., Henry,J.G., Ware,C.B., Andrade,J., Dawson,G. and Schwartz,N.B.
    Global brain transcriptome analysis of a Tpp1 neuronal ceroid lipofuscinoses mouse model
    ASN Neuro11, 1759091419843393-1759091419843393. PubMed  Europe PubMed DOI  M
  15. Gardner,E., Bailey,M., Schulz,A., Aristorena,M., Miller,N. and Mole,S.E.
    Mutation update: Review of TPP1 gene variants associated with neuronal ceroid lipofuscinosis CLN2 disease
    Hum Mutat40, 1924-1938. PubMed  Europe PubMed DOI  M  V
  16. Lukacs,Z., Nickel,M., Murko,S., Nieves Cobos,P., Schulz,A., Santer,R. and Kohlschutter,A.
    Validity of a rapid and simple fluorometric tripeptidyl peptidase 1 (TPP1) assay using dried blood specimens to diagnose CLN2 disease
    Clin Chim Acta492, 69-71. PubMed  Europe PubMed DOI  A
  17. Smith,P.K., Sen,M.G., Fisher,P.R. and Annesley,S.J.
    Modelling of neuronal ceroid lipofuscinosis type 2 in Dictyostelium discoideum suggests that cytopathological outcomes result from altered TOR signalling
    Cells8, PubMed  Europe PubMed DOI  K
    18. 2018
  18. Nemtsova,Y., Wiseman,J.A., El-Banna,M., Lobel,P. and Sleat,D.E.
    Inducible transgenic expression of tripeptidyl peptidase 1 in a mouse model of late-infantile neuronal ceroid lipofuscinosis
    PLoS ONE13, e0192286-e0192286. PubMed  Europe PubMed DOI
  19. Schulz,A., Ajayi,T., Specchio,N., de Los Reyes,E., Gissen,P., Ballon,D., Dyke,J.P., Cahan,H., Slasor,P., Jacoby,D. and Kohlschutter,A.
    Study of intraventricular cerliponase alfa for CLN2 disease
    N Engl J Med378, 1898-1907. PubMed  Europe PubMed DOI  U
  20. Sheth,J., Mistri,M., Bhavsar,R., Pancholi,D., Kamate,M., Gupta,N., Kabra,M., Mehta,S., Nampoothiri,S., Thakker,A., Jain,V., Shah,R. and Sheth,F.
    Batten disease: biochemical and molecular characterization revealing novel PPT1 and TPP1 gene mutations in Indian patients
    BMC Neurol18, 203-203. PubMed  Europe PubMed DOI  M
  21. Sole-Domenech,S., Rojas,A.V., Maisuradze,G.G., Scheraga,H.A., Lobel,P. and Maxfield,F.R.
    Lysosomal enzyme tripeptidyl peptidase 1 destabilizes fibrillar Abeta by multiple endoproteolytic cleavages within the beta-sheet domain
    Proc Natl Acad Sci U S A115, 1493-1498. PubMed  Europe PubMed DOI
    22. 2017
  22. Ghosh,A., Rangasamy,S.B., Modi,K.K. and Pahan,K.
    Gemfibrozil, Food and Drug Administration-approved lipid-lowering drug, increases longevity in mouse model of Late Infantile Neuronal Ceroid Lipofuscinosis
    J Neurochem141, 423-435. PubMed  Europe PubMed DOI  M
  23. Katz,M.L., Johnson,G.C., Leach,S.B., Williamson,B.G., Coates,J.R., Whiting,R.E.H., Vansteenkiste,D.P. and Whitney,M.S.
    Extraneuronal pathology in a canine model of CLN2 neuronal ceroid lipofuscinosis after intracerebroventricular gene therapy that delays neurological disease progression
    Gene Ther24, 215-223. PubMed  Europe PubMed DOI  U
  24. Markham,A.
    Cerliponase alfa: first global approval
    Drugs77, 1247-1249. PubMed  Europe PubMed DOI  U
    25. 2016
  25. Fietz,M., AlSayed,M., Burke,D., Cohen-Pfeffer,J., Cooper,J.D., Dvorakova,L., Giugliani,R., Izzo,E., Jahnova,H., Lukacs,Z., Mole,S.E., Noher de Halac,I., Pearce,D.A., Poupetova,H., Schulz,A., Specchio,N., Xin,W. and Miller,N.
    Diagnosis of neuronal ceroid lipofuscinosis type 2 (CLN2 disease): expert recommendations for early detection and laboratory diagnosis
    Mol Genet Metab119, 160-167. PubMed  Europe PubMed DOI  M
  26. Kohlschutter,A. and Schulz,A.
    CLN2 Disease (classic late infantile neuronal ceroid lipofuscinosis)
    Pediatr Endocrinol Rev13 Suppl 1, 682-688. PubMed  Europe PubMed  M  V
  27. Kondo,M.Y., Gouvea,I.E., Okamoto,D.N., Santos,J.A., Souccar,C., Oda,K., Juliano,L. and Juliano,M.A.
    Analysis of catalytic properties of tripeptidyl peptidase I (TTP-I), a serine carboxyl lysosomal protease, and its detection in tissue extracts using selective FRET peptide substrate
    Peptides76, 80-86. PubMed  Europe PubMed DOI  A  P
  28. Shibata,M., Koike,M., Kusumi,S., Sato,N. and Uchiyama,Y.
    A specific tripeptidyl substrate for tripeptidyl peptidase activity is effectively hydrolyzed by alanyl aminopeptidase/aminopeptidase N/CD13 in the rat kidney
    Arch Histol Cytol76, 1-8. DOI
  29. Whiting,R.E., Jensen,C.A., Pearce,J.W., Gillespie,L.E., Bristow,D.E. and Katz,M.L.
    Intracerebroventricular gene therapy that delays neurological disease progression is associated with selective preservation of retinal ganglion cells in a canine model of CLN2 disease
    Exp Eye Res146, 276-282. PubMed  Europe PubMed DOI  M
    30. 2015
  30. Atanasova,D. and Lazarov,N.
    Histochemical demonstration of tripeptidyl aminopeptidase I in the rat carotid body
    Acta Histochem117, 219-222. PubMed  Europe PubMed DOI  L
  31. Carcel-Trullols,J., Kovacs,A.D. and Pearce,D.A.
    Cell biology of the NCL proteins: what they do and don't do
    Biochim Biophys Acta1852, 2242-2255. PubMed  Europe PubMed DOI
  32. Katz,M.L., Tecedor,L., Chen,Y., Williamson,B.G., Lysenko,E., Wininger,F.A., Young,W.M., Johnson,G.C., Whiting,R.E., Coates,J.R. and Davidson,B.L.
    AAV gene transfer delays disease onset in a TPP1-deficient canine model of the late infantile form of Batten disease
    Sci Transl Med7, 313ra180-313ra. PubMed  Europe PubMed DOI  K
  33. Phillips,J.E. and Gomer,R.H.
    Partial genetic suppression of a loss-of-function mutant of the neuronal ceroid lipofuscinosis-associated protease TPP1 in Dictyostelium discoideum
    Dis Model Mech8, 147-156. PubMed  Europe PubMed DOI
  34. Vuillemenot,B.R., Kennedy,D., Cooper,J.D., Wong,A.M., Sri,S., Doeleman,T., Katz,M.L., Coates,J.R., Johnson,G.C., Reed,R.P., Adams,E.L., Butt,M.T., Musson,D.G., Henshaw,J., Keve,S., Cahayag,R., Tsuruda,L.S. and O'Neill,C.A.
    Nonclinical evaluation of CNS-administered TPP1 enzyme replacement in canine CLN2 neuronal ceroid lipofuscinosis
    Mol Genet Metab114, 281-293. PubMed  Europe PubMed DOI  U
    35. 2014
  35. Chen,H., Liu,X., Zhu,W., Chen,H., Hu,X., Jiang,Z., Xu,Y., Wang,L., Zhou,Y., Chen,P., Zhang,N., Hu,D., Zhang,L., Wang,Y., Xu,Q., Wu,R., Yu,H. and Wang,J.
    SIRT1 ameliorates age-related senescence of mesenchymal stem cells via modulating telomere shelterin
    Front Aging Neurosci6, 103-103. PubMed  Europe PubMed DOI
  36. Vuillemenot,B.R., Kennedy,D., Reed,R.P., Boyd,R.B., Butt,M.T., Musson,D.G., Keve,S., Cahayag,R., Tsuruda,L.S. and O'Neill,C.A.
    Recombinant human tripeptidyl peptidase-1 infusion to the monkey CNS: Safety, pharmacokinetics, and distribution
    Toxicol Appl Pharmacol277, 49-57. PubMed  Europe PubMed DOI  U
  37. Whiting,R.E., Narfstrom,K., Yao,G., Pearce,J.W., Coates,J.R., Castaner,L.J., Jensen,C.A., Dougherty,B.N., Vuillemenot,B.R., Kennedy,D., O'Neill,C.A. and Katz,M.L.
    Enzyme replacement therapy delays pupillary light reflex deficits in a canine model of late infantile neuronal ceroid lipofuscinosis
    Exp Eye Res125, 164-172. PubMed  Europe PubMed DOI  U
    38. 2013
  38. Kohan,R., Carabelos,M.N., Xin,W., Sims,K., Guelbert,N., Cismondi,I.A., Pons,P., Alonso,G.I., Troncoso,M., Witting,S., Pearce,D.A., Dodelson de Kremer,R., Oller-Ramirez,A.M. and Noher de Halac,I.
    Neuronal ceroid lipofuscinosis type CLN2: a new rationale for the construction of phenotypic subgroups based on a survey of 25 cases in South America
    Gene516, 114-121. PubMed  Europe PubMed DOI  M
  39. Mahmood,F., Fu,S., Cooke,J., Wilson,S.W., Cooper,J.D. and Russell,C.
    A zebrafish model of CLN2 disease is deficient in tripeptidyl peptidase 1 and displays progressive neurodegeneration accompanied by a reduction in proliferation
    Brain136, 1488-1507. PubMed  Europe PubMed DOI  K
  40. Sun,Y., Almomani,R., Breedveld,G.J., Santen,G.W., Aten,E., Lefeber,D.J., Hoff,J.I., Brusse,E., Verheijen,F.W., Verdijk,R.M., Kriek,M., Oostra,B., Breuning,M.H., Losekoot,M., den Dunnen,J.T., van de Warrenburg,B.P. and Maat-Kievit,A.J.
    Autosomal recessive spinocerebellar ataxia 7 (SCAR7) is caused by variants in TPP1, the gene involved in classic late-infantile neuronal ceroid lipofuscinosis 2 disease (CLN2 Disease)
    Hum Mutat34, 706-713. PubMed  Europe PubMed DOI  M
  41. Van Beersel,G., Tihon,E., Demine,S., Hamer,I., Jadot,M. and Arnould,T.
    Different molecular mechanisms involved in spontaneous and oxidative stress-induced mitochondrial fragmentation in tripeptidyl peptidase-1 (TPP-1)-deficient fibroblasts
    Biosci Rep33, e00023-e00023. PubMed  Europe PubMed DOI  M
    42. 2012
  42. Meng,Y., Sohar,I., Wang,L., Sleat,D.E. and Lobel,P.
    Systemic administration of tripeptidyl peptidase I in a mouse model of late infantile neuronal ceroid lipofuscinosis: effect of glycan modification
    PLoS ONE7, e40509-e40509. PubMed  Europe PubMed DOI  U
  43. Shacka,J.J.
    Mouse models of neuronal ceroid lipofuscinoses: useful pre-clinical tools to delineate disease pathophysiology and validate therapeutics
    Brain Res Bull88, 43-57. PubMed  Europe PubMed DOI  M
  44. Sohar,I., Sleat,D.E. and Lobel,P.
    Tripeptidyl peptidase I
    [ISSN:978-0-12-407742-3]3, 3350-3356. DOI
  45. Sondhi,D., Johnson,L., Purpura,K., Monette,S., Souweidane,M.M., Kaplitt,M.G., Kosofsky,B., Yohay,K., Ballon,D., Dyke,J., Kaminksy,S.M., Hackett,N.R. and Crystal,R.G.
    Long-term expression and safety of administration of AAVrh.10hCLN2 to the brain of rats and nonhuman primates for the treatment of late infantile neuronal ceroid lipofuscinosis
    Hum Gene Ther Methods23, 324-335. PubMed  Europe PubMed DOI  U
    46. 2011
  46. Dimitrova,M., Deleva,D., Pavlova,V. and Ivanov,I.
    Developmental study of tripeptidyl peptidase I activity in the mouse central nervous system and peripheral organs
    Cell Tissue Res346, 141-149. PubMed  Europe PubMed DOI
  47. Vuillemenot,B.R., Katz,M.L., Coates,J.R., Kennedy,D., Tiger,P., Kanazono,S., Lobel,P., Sohar,I., Xu,S., Cahayag,R., Keve,S., Koren,E., Bunting,S., Tsuruda,L.S. and O'Neill,C.A.
    Intrathecal tripeptidyl-peptidase 1 reduces lysosomal storage in a canine model of late infantile neuronal ceroid lipofuscinosis
    Mol Genet Metab104, 325-337. PubMed  Europe PubMed DOI  U
  48. Xu,S., Wang,L., El-Banna,M., Sohar,I., Sleat,D.E. and Lobel,P.
    Large-volume intrathecal enzyme delivery increases survival of a mouse model of late infantile neuronal ceroid lipofuscinosis
    Mol Ther19, 1842-1848. PubMed  Europe PubMed DOI  U
    49. 2010
  49. Kuizon,S., Dimaiuta,K., Walus,M., Jenkins,E.C., Kuizon,M., Kida,E., Golabek,A.A., Espinoza,D.O., Pullarkat,R.K. and Junaid,M.A.
    A critical tryptophan and Ca2+ in activation and catalysis of TPPI, the enzyme deficient in classic late-infantile neuronal ceroid lipofuscinosis
    PLoS ONE5, e11929-e11929. PubMed  Europe PubMed DOI  I
  50. Walus,M., Kida,E. and Golabek,A.A.
    Functional consequences and rescue potential of pathogenic missense mutations in tripeptidyl peptidase I
    Hum Mutat31, 710-721. PubMed  Europe PubMed DOI  M
    51. 2009
  51. Autefage,H., Albinet,V., Garcia,V., Berges,H., Nicolau,M.L., Therville,N., Altie,M.F., Caillaud,C., Levade,T. and Andrieu-Abadie,N.
    Lysosomal serine protease CLN2 regulates tumor necrosis factor-alpha-mediated apoptosis in a bid-dependent manner
    J Biol Chem284, 11507-11516. PubMed  Europe PubMed DOI
  52. Goldberg-Stern,H., Halevi,A., Marom,D., Straussberg,R. and Mimouni-Bloch,A.
    Late infantile neuronal ceroid lipofuscinosis: a new mutation in Arabs
    Pediatr Neurol41, 297-300. PubMed  Europe PubMed DOI  M
  53. Guhaniyogi,J., Sohar,I., Das,K., Stock,A.M. and Lobel,P.
    Crystal structure and autoactivation pathway of the precursor form of human tripeptidyl-peptidase 1, the enzyme deficient in late infantile ceroid lipofuscinosis
    J Biol Chem284, 3985-3997. PubMed  Europe PubMed DOI  S
  54. Kim,K.H., Sleat,D.E., Bernard,O. and Lobel,P.
    Genetic modulation of apoptotic pathways fails to alter disease course in tripeptidyl-peptidase 1 deficient mice
    Neurosci Lett453, 27-30. PubMed  Europe PubMed DOI  K
  55. Pal,A., Kraetzner,R., Gruene,T., Grapp,M., Schreiber,K., Gronborg,M., Urlaub,H., Becker,S., Asif,A.R., Gartner,J., Sheldrick,G.M. and Steinfeld,R.
    Structure of tripeptidyl-peptidase I provides insight into the molecular basis of late infantile neuronal ceroid lipofuscinosis
    J Biol Chem284, 3976-3984. PubMed  Europe PubMed DOI  S
    56. 2008
  56. Bessa,C., Teixeira,C.A., Dias,A., Alves,M., Rocha,S., Lacerda,L., Loureiro,L., Guimaraes,A. and Ribeiro,M.G.
    CLN2/TPP1 deficiency: The novel mutation IVS7-10A>G causes intron retention and is associated with a mild disease phenotype
    Mol Genet Metab93, 66-73. PubMed  Europe PubMed DOI  M
  57. Chang,M., Cooper,J.D., Sleat,D.E., Cheng,S.H., Dodge,J.C., Passini,M.A., Lobel,P. and Davidson,B.L.
    Intraventricular enzyme replacement improves disease phenotypes in a mouse model of late infantile neuronal ceroid lipofuscinosis
    Mol Ther16, 649-656. PubMed  Europe PubMed DOI  U
  58. Golabek,A.A., Dolzhanskaya,N., Walus,M., Wisniewski,K.E. and Kida,E.
    Prosegment of tripeptidyl peptidase I is a potent, slow-binding inhibitor of its cognate enzyme
    J Biol Chem283, 16497-16504. PubMed  Europe PubMed DOI
  59. Sleat,D.E., El-Banna,M., Sohar,I., Kim,K.H., Dobrenis,K., Walkley,S.U. and Lobel,P.
    Residual levels of tripeptidyl-peptidase I activity dramatically ameliorate disease in late-infantile neuronal ceroid lipofuscinosis
    Mol Genet Metab94, 222-233. PubMed  Europe PubMed DOI
  60. Sondhi,D., Peterson,D.A., Edelstein,A.M., del Fierro,K., Hackett,N.R. and Crystal,R.G.
    Survival advantage of neonatal CNS gene transfer for late infantile neuronal ceroid lipofuscinosis
    Exp Neurol213, 18-27. PubMed  Europe PubMed DOI  U
    61. 2007
  61. Cabrera-Salazar,M.A., Roskelley,E.M., Bu,J., Hodges,B.L., Yew,N., Dodge,J.C., Shihabuddin,L.S., Sohar,I., Sleat,D.E., Scheule,R.K., Davidson,B.L., Cheng,S.H., Lobel,P. and Passini,M.A.
    Timing of therapeutic intervention determines functional and survival outcomes in a mouse model of late infantile Batten disease
    Mol Ther15, 1782-1788. PubMed  Europe PubMed DOI  K  U
  62. Lindas,A.C. and Tomkinson,B.
    Characterization of the promoter of the gene encoding human tripeptidyl-peptidase II and identification of upstream silencer elements
    Gene393, 62-69. PubMed  Europe PubMed DOI
  63. Sondhi,D., Hackett,N.R., Peterson,D.A., Stratton,J., Baad,M., Travis,K.M., Wilson,J.M. and Crystal,R.G.
    Enhanced survival of the LINCL mouse following CLN2 gene transfer using the rh.10 rhesus macaque-derived adeno-associated virus vector
    Mol Ther15, 481-491. PubMed  Europe PubMed DOI  U
    64. 2006
  64. Golabek,A.A. and Kida,E.
    Tripeptidyl-peptidase I in health and disease
    Biol Chem387, 1091-1099. PubMed  Europe PubMed DOI  V
  65. [YEAR:1-2-2006]Passini,M.A., Dodge,J.C., Bu,J., Yang,W., Zhao,Q., Sondhi,D., Hackett,N.R., Kaminsky,S.M., Mao,Q., Shihabuddin,L.S., Cheng,S.H., Sleat,D.E., Stewart,G.R., Davidson,B.L., Lobel,P. and Crystal,R.G.
    Intracranial delivery of CLN2 reduces brain pathology in a mouse model of classical late infantile neuronal ceroid lipofuscinosis
    J Neurosci26, 1334-1342. PubMed  Europe PubMed DOI
  66. [YEAR:16-6-2006]Steinfeld,R., Fuhrmann,J.C. and Gartner,J.
    Detection of tripeptidyl peptidase I activity in living cells by fluorogenic substrates
    J Histochem Cytochem54, 991-996. PubMed  Europe PubMed DOI
  67. [YEAR:8-12-2006]Tian,Y., Sohar,I., Taylor,J.W. and Lobel,P.
    Determination of the substrate specificity of tripeptidyl-peptidase I using combinatorial peptide libraries and development of improved fluorogenic substrates
    J Biol Chem281, 6559-6572. PubMed  Europe PubMed DOI  P
    68. 2005
  68. [YEAR:6-12-2005]Golabek,A.A., Walus,M., Wisniewski,K.E. and Kida,E.
    Glycosaminoglycans modulate activation, activity, and stability of tripeptidyl-peptidase I in vitro and in vivo
    J Biol Chem280, 7550-7561. PubMed  Europe PubMed DOI
  69. Hackett,N.R., Redmond,D.E., Sondhi,D., Giannaris,E.L., Vassallo,E., Stratton,J., Qiu,J., Kaminsky,S.M., Lesser,M.L., Fisch,G.S., Rouselle,S.D. and Crystal,R.G.
    Safety of direct administration of AAV2(CU)hCLN2, a candidate treatment for the central nervous system manifestations of late infantile neuronal ceroid lipofuscinosis, to the brain of rats and nonhuman primates
    Hum Gene Ther16, 1484-1503. PubMed  Europe PubMed DOI
  70. Oyama,H., Fujisawa,T., Suzuki,T., Dunn,B.M., Wlodawer,A. and Oda,K.
    Catalytic residues and substrate specificity of recombinant human tripeptidyl peptidase I (CLN2)
    J Biochem138, 127-134. PubMed  Europe PubMed DOI  P
  71. [YEAR:28-7-2005]Sondhi,D., Peterson,D.A., Giannaris,E.L., Sanders,C.T., Mendez,B.S., De,B., Rostkowski,A.B., Blanchard,B., Bjugstad,K., Sladek,J.R., Jr., Redmond,D.E., Jr., Leopold,P.L., Kaminsky,S.M., Hackett,N.R. and Crystal,R.G.
    AAV2-mediated CLN2 gene transfer to rodent and non-human primate brain results in long-term TPP-I expression compatible with therapy for LINCL
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