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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.<br>First in man study of intravitreal tripeptidyl peptidase 1 for CLN2 retinopathy<br>Eye (Lond) (2024) 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.<br>Extracellular Vesicles Released by Genetically Modified Macrophages Activate Autophagy and Produce Potent Neuroprotection in Mouse Model of Lysosomal Storage Disorder, Batten Disease<br>Cells (2023) 12 PubMed  Europe PubMed DOI  PMC  EPMC
  3. Liu,R.H., Wang,X.Y., Jia,Y.Y., Wang,X.C., Xia,M., Nie,Q., Guo,J. and Kong,Q.X.<br>Compound heterozygous mutations in tripeptidyl peptidase 1 cause rare autosomal recessive spinocerebellar ataxia type 7: A case report<br>World J Clin Cases (2023) 11, 6618-6623. PubMed  Europe PubMed DOI  PMC  EPMC
  4. Steigerwald,C., Borsuk,J., Pappas,J., Galey,M., Scott,A., Devaney,J.M., Miller,D.E. and Abreu,N.J.<br>CLN2 disease resulting from a novel homozygous deep intronic splice variant in TPP1 discovered using long-read sequencing<br>Mol Genet Metab (2023) 140, 107713-107713. PubMed  Europe PubMed DOI
    5. 2022
  5. Baranzehi,T., Kordi-Tamandani,D.M., Najafi,M., Khajeh,A. and Schmidts,M.<br>Identification of a TPP1 Q278X Mutation in an Iranian Patient with Neuronal Ceroid Lipofuscinosis 2: Literature Review and Mutations Update<br>J Clin Med (2022) 11 PubMed  Europe PubMed DOI  PMC  EPMC
    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.<br>Immune deficiency, autoimmune disease and intellectual disability: A pleiotropic disorder caused by biallelic variants in the TPP2 gene<br>Clin Genet (2021) 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.<br>Intracerebroventricular Cerliponase Alfa for Neuronal Ceroid Lipofuscinosis Type 2 Disease: Clinical Practice Considerations From US Clinics<br>Pediatr Neurol (2020) 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.<br>Clinical Pharmacokinetics and Pharmacodynamics of Cerliponase Alfa, Enzyme Replacement Therapy for CLN2 Disease by Intracerebroventricular Administration<br>Clin Transl Sci (2020) PubMed  Europe PubMed DOI
  9. Lewis,G., Morrill,A.M., Conway-Allen,S.L. and Kim,B.<br>Review of cerliponase alfa: recombinant human enzyme replacement therapy for late-infantile neuronal ceroid lipofuscinosis type 2<br>J Child Neurol (2020) 35, 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.<br>Enzymatic diagnosis of neuronal lipofuscinoses in dried blood spots using substrates for concomitant tandem mass spectrometry and fluorimetry<br>J Mass Spectrom (2020) , e4675-e4675. PubMed  Europe PubMed DOI
  11. Santos,L.L., Ling,C.K. and Dimitriadis,E.<br>Tripeptidyl peptidase I promotes human endometrial epithelial cell adhesive capacity implying a role in receptivity<br>Reprod Biol Endocrinol (2020) 18, 124-124. PubMed  Europe PubMed DOI  PMC  EPMC  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.<br>Intravitreal enzyme replacement inhibits progression of retinal degeneration in canine CLN2 neuronal ceroid lipofuscinosis<br>Exp Eye Res (2020) , 108135-108135. PubMed  Europe PubMed DOI
    13. 2019
  13. Chakrabarti,S., Chandra,S., Roy,A., Dasarathi,S., Kundu,M. and Pahan,K.<br>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<br>Neurobiol Dis (2019) 127, 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.<br>Global brain transcriptome analysis of a Tpp1 neuronal ceroid lipofuscinoses mouse model<br>ASN Neuro (2019) 11, 1759091419843393-1759091419843393. PubMed  Europe PubMed DOI  PMC  EPMC  M
  15. Gardner,E., Bailey,M., Schulz,A., Aristorena,M., Miller,N. and Mole,S.E.<br>Mutation update: Review of TPP1 gene variants associated with neuronal ceroid lipofuscinosis CLN2 disease<br>Hum Mutat (2019) 40, 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.<br>Validity of a rapid and simple fluorometric tripeptidyl peptidase 1 (TPP1) assay using dried blood specimens to diagnose CLN2 disease<br>Clin Chim Acta (2019) 492, 69-71. PubMed  Europe PubMed DOI  A
  17. Smith,P.K., Sen,M.G., Fisher,P.R. and Annesley,S.J.<br>Modelling of neuronal ceroid lipofuscinosis type 2 in Dictyostelium discoideum suggests that cytopathological outcomes result from altered TOR signalling<br>Cells (2019) 8 PubMed  Europe PubMed DOI  K
    18. 2018
  18. Nemtsova,Y., Wiseman,J.A., El-Banna,M., Lobel,P. and Sleat,D.E.<br>Inducible transgenic expression of tripeptidyl peptidase 1 in a mouse model of late-infantile neuronal ceroid lipofuscinosis<br>PLoS ONE (2018) 13, 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.<br>Study of intraventricular cerliponase alfa for CLN2 disease<br>N Engl J Med (2018) 378, 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.<br>Batten disease: biochemical and molecular characterization revealing novel PPT1 and TPP1 gene mutations in Indian patients<br>BMC Neurol (2018) 18, 203-203. PubMed  Europe PubMed DOI  PMC  EPMC  M
  21. Sole-Domenech,S., Rojas,A.V., Maisuradze,G.G., Scheraga,H.A., Lobel,P. and Maxfield,F.R.<br>Lysosomal enzyme tripeptidyl peptidase 1 destabilizes fibrillar Abeta by multiple endoproteolytic cleavages within the beta-sheet domain<br>Proc Natl Acad Sci U S A (2018) 115, 1493-1498. PubMed  Europe PubMed DOI
    22. 2017
  22. Ghosh,A., Rangasamy,S.B., Modi,K.K. and Pahan,K.<br>Gemfibrozil, Food and Drug Administration-approved lipid-lowering drug, increases longevity in mouse model of Late Infantile Neuronal Ceroid Lipofuscinosis<br>J Neurochem (2017) 141, 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.<br>Extraneuronal pathology in a canine model of CLN2 neuronal ceroid lipofuscinosis after intracerebroventricular gene therapy that delays neurological disease progression<br>Gene Ther (2017) 24, 215-223. PubMed  Europe PubMed DOI  U
  24. Markham,A.<br>Cerliponase alfa: first global approval<br>Drugs (2017) 77, 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.<br>Diagnosis of neuronal ceroid lipofuscinosis type 2 (CLN2 disease): expert recommendations for early detection and laboratory diagnosis<br>Mol Genet Metab (2016) 119, 160-167. PubMed  Europe PubMed DOI  M
  26. Kohlschutter,A. and Schulz,A.<br>CLN2 Disease (classic late infantile neuronal ceroid lipofuscinosis)<br>Pediatr Endocrinol Rev (2016) 13 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.<br>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<br>Peptides (2016) 76, 80-86. PubMed  Europe PubMed DOI  A  P
  28. Shibata,M., Koike,M., Kusumi,S., Sato,N. and Uchiyama,Y.<br>A specific tripeptidyl substrate for tripeptidyl peptidase activity is effectively hydrolyzed by alanyl aminopeptidase/aminopeptidase N/CD13 in the rat kidney<br>Arch Histol Cytol (2016) 76, 1-8. DOI
  29. Whiting,R.E., Jensen,C.A., Pearce,J.W., Gillespie,L.E., Bristow,D.E. and Katz,M.L.<br>Intracerebroventricular gene therapy that delays neurological disease progression is associated with selective preservation of retinal ganglion cells in a canine model of CLN2 disease<br>Exp Eye Res (2016) 146, 276-282. PubMed  Europe PubMed DOI  M
    30. 2015
  30. Atanasova,D. and Lazarov,N.<br>Histochemical demonstration of tripeptidyl aminopeptidase I in the rat carotid body<br>Acta Histochem (2015) 117, 219-222. PubMed  Europe PubMed DOI  L
  31. Carcel-Trullols,J., Kovacs,A.D. and Pearce,D.A.<br>Cell biology of the NCL proteins: what they do and don't do<br>Biochim Biophys Acta (2015) 1852, 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.<br>AAV gene transfer delays disease onset in a TPP1-deficient canine model of the late infantile form of Batten disease<br>Sci Transl Med (2015) 7, 313ra180-313ra. PubMed  Europe PubMed DOI  K
  33. Phillips,J.E. and Gomer,R.H.<br>Partial genetic suppression of a loss-of-function mutant of the neuronal ceroid lipofuscinosis-associated protease TPP1 in Dictyostelium discoideum<br>Dis Model Mech (2015) 8, 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.<br>Nonclinical evaluation of CNS-administered TPP1 enzyme replacement in canine CLN2 neuronal ceroid lipofuscinosis<br>Mol Genet Metab (2015) 114, 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.<br>SIRT1 ameliorates age-related senescence of mesenchymal stem cells via modulating telomere shelterin<br>Front Aging Neurosci (2014) 6, 103-103. PubMed  Europe PubMed DOI  PMC  EPMC
  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.<br>Recombinant human tripeptidyl peptidase-1 infusion to the monkey CNS: Safety, pharmacokinetics, and distribution<br>Toxicol Appl Pharmacol (2014) 277, 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.<br>Enzyme replacement therapy delays pupillary light reflex deficits in a canine model of late infantile neuronal ceroid lipofuscinosis<br>Exp Eye Res (2014) 125, 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.<br>Neuronal ceroid lipofuscinosis type CLN2: a new rationale for the construction of phenotypic subgroups based on a survey of 25 cases in South America<br>Gene (2013) 516, 114-121. PubMed  Europe PubMed DOI  M
  39. Mahmood,F., Fu,S., Cooke,J., Wilson,S.W., Cooper,J.D. and Russell,C.<br>A zebrafish model of CLN2 disease is deficient in tripeptidyl peptidase 1 and displays progressive neurodegeneration accompanied by a reduction in proliferation<br>Brain (2013) 136, 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.<br>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)<br>Hum Mutat (2013) 34, 706-713. PubMed  Europe PubMed DOI  M
  41. Van Beersel,G., Tihon,E., Demine,S., Hamer,I., Jadot,M. and Arnould,T.<br>Different molecular mechanisms involved in spontaneous and oxidative stress-induced mitochondrial fragmentation in tripeptidyl peptidase-1 (TPP-1)-deficient fibroblasts<br>Biosci Rep (2013) 33, e00023-e00023. PubMed  Europe PubMed DOI  M
    42. 2012
  42. Meng,Y., Sohar,I., Wang,L., Sleat,D.E. and Lobel,P.<br>Systemic administration of tripeptidyl peptidase I in a mouse model of late infantile neuronal ceroid lipofuscinosis: effect of glycan modification<br>PLoS ONE (2012) 7, e40509-e40509. PubMed  Europe PubMed DOI  PMC  EPMC  U
  43. Shacka,J.J.<br>Mouse models of neuronal ceroid lipofuscinoses: useful pre-clinical tools to delineate disease pathophysiology and validate therapeutics<br>Brain Res Bull (2012) 88, 43-57. PubMed  Europe PubMed DOI  M
  44. Sohar,I., Sleat,D.E. and Lobel,P.<br>Tripeptidyl peptidase I<br>[ISSN:978-0-12-407742-3] (2012) 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.<br>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<br>Hum Gene Ther Methods (2012) 23, 324-335. PubMed  Europe PubMed DOI  U
    46. 2011
  46. Dimitrova,M., Deleva,D., Pavlova,V. and Ivanov,I.<br>Developmental study of tripeptidyl peptidase I activity in the mouse central nervous system and peripheral organs<br>Cell Tissue Res (2011) 346, 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.<br>Intrathecal tripeptidyl-peptidase 1 reduces lysosomal storage in a canine model of late infantile neuronal ceroid lipofuscinosis<br>Mol Genet Metab (2011) 104, 325-337. PubMed  Europe PubMed DOI  U
  48. Xu,S., Wang,L., El-Banna,M., Sohar,I., Sleat,D.E. and Lobel,P.<br>Large-volume intrathecal enzyme delivery increases survival of a mouse model of late infantile neuronal ceroid lipofuscinosis<br>Mol Ther (2011) 19, 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.<br>A critical tryptophan and Ca2+ in activation and catalysis of TPPI, the enzyme deficient in classic late-infantile neuronal ceroid lipofuscinosis<br>PLoS ONE (2010) 5, e11929-e11929. PubMed  Europe PubMed DOI  PMC  EPMC  I
  50. Walus,M., Kida,E. and Golabek,A.A.<br>Functional consequences and rescue potential of pathogenic missense mutations in tripeptidyl peptidase I<br>Hum Mutat (2010) 31, 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.<br>Lysosomal serine protease CLN2 regulates tumor necrosis factor-alpha-mediated apoptosis in a bid-dependent manner<br>J Biol Chem (2009) 284, 11507-11516. PubMed  Europe PubMed DOI  PMC  EPMC
  52. Goldberg-Stern,H., Halevi,A., Marom,D., Straussberg,R. and Mimouni-Bloch,A.<br>Late infantile neuronal ceroid lipofuscinosis: a new mutation in Arabs<br>Pediatr Neurol (2009) 41, 297-300. PubMed  Europe PubMed DOI  M
  53. Guhaniyogi,J., Sohar,I., Das,K., Stock,A.M. and Lobel,P.<br>Crystal structure and autoactivation pathway of the precursor form of human tripeptidyl-peptidase 1, the enzyme deficient in late infantile ceroid lipofuscinosis<br>J Biol Chem (2009) 284, 3985-3997. PubMed  Europe PubMed DOI  PMC  EPMC  S
  54. Kim,K.H., Sleat,D.E., Bernard,O. and Lobel,P.<br>Genetic modulation of apoptotic pathways fails to alter disease course in tripeptidyl-peptidase 1 deficient mice<br>Neurosci Lett (2009) 453, 27-30. PubMed  Europe PubMed DOI  PMC  EPMC  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.<br>Structure of tripeptidyl-peptidase I provides insight into the molecular basis of late infantile neuronal ceroid lipofuscinosis<br>J Biol Chem (2009) 284, 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.<br>CLN2/TPP1 deficiency: The novel mutation IVS7-10A>G causes intron retention and is associated with a mild disease phenotype<br>Mol Genet Metab (2008) 93, 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.<br>Intraventricular enzyme replacement improves disease phenotypes in a mouse model of late infantile neuronal ceroid lipofuscinosis<br>Mol Ther (2008) 16, 649-656. PubMed  Europe PubMed DOI  U
  58. Golabek,A.A., Dolzhanskaya,N., Walus,M., Wisniewski,K.E. and Kida,E.<br>Prosegment of tripeptidyl peptidase I is a potent, slow-binding inhibitor of its cognate enzyme<br>J Biol Chem (2008) 283, 16497-16504. PubMed  Europe PubMed DOI  PMC  EPMC
  59. Sleat,D.E., El-Banna,M., Sohar,I., Kim,K.H., Dobrenis,K., Walkley,S.U. and Lobel,P.<br>Residual levels of tripeptidyl-peptidase I activity dramatically ameliorate disease in late-infantile neuronal ceroid lipofuscinosis<br>Mol Genet Metab (2008) 94, 222-233. PubMed  Europe PubMed DOI  PMC  EPMC
  60. Sondhi,D., Peterson,D.A., Edelstein,A.M., del Fierro,K., Hackett,N.R. and Crystal,R.G.<br>Survival advantage of neonatal CNS gene transfer for late infantile neuronal ceroid lipofuscinosis<br>Exp Neurol (2008) 213, 18-27. PubMed  Europe PubMed DOI  PMC  EPMC  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.<br>Timing of therapeutic intervention determines functional and survival outcomes in a mouse model of late infantile Batten disease<br>Mol Ther (2007) 15, 1782-1788. PubMed  Europe PubMed DOI  K  U
  62. Lindas,A.C. and Tomkinson,B.<br>Characterization of the promoter of the gene encoding human tripeptidyl-peptidase II and identification of upstream silencer elements<br>Gene (2007) 393, 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.<br>Enhanced survival of the LINCL mouse following CLN2 gene transfer using the rh.10 rhesus macaque-derived adeno-associated virus vector<br>Mol Ther (2007) 15, 481-491. PubMed  Europe PubMed DOI  U
    64. 2006
  64. Golabek,A.A. and Kida,E.<br>Tripeptidyl-peptidase I in health and disease<br>Biol Chem (2006) 387, 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.<br>Intracranial delivery of CLN2 reduces brain pathology in a mouse model of classical late infantile neuronal ceroid lipofuscinosis<br>J Neurosci (1-2-2006) 26, 1334-1342. PubMed  Europe PubMed DOI
  66. [YEAR:16-6-2006]Steinfeld,R., Fuhrmann,J.C. and Gartner,J.<br>Detection of tripeptidyl peptidase I activity in living cells by fluorogenic substrates<br>J Histochem Cytochem (16-6-2006) 54, 991-996. PubMed  Europe PubMed DOI
  67. [YEAR:8-12-2006]Tian,Y., Sohar,I., Taylor,J.W. and Lobel,P.<br>Determination of the substrate specificity of tripeptidyl-peptidase I using combinatorial peptide libraries and development of improved fluorogenic substrates<br>J Biol Chem (8-12-2006) 281, 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.<br>Glycosaminoglycans modulate activation, activity, and stability of tripeptidyl-peptidase I in vitro and in vivo<br>J Biol Chem (6-12-2005) 280, 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.<br>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<br>Hum Gene Ther (2005) 16, 1484-1503. PubMed  Europe PubMed DOI
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