228 related articles for article (PubMed ID: 23804102)
1. Lysosomal membrane permeability stimulates protein aggregate formation in neurons of a lysosomal disease.
Micsenyi MC; Sikora J; Stephney G; Dobrenis K; Walkley SU
J Neurosci; 2013 Jun; 33(26):10815-27. PubMed ID: 23804102
[TBL] [Abstract][Full Text] [Related]
2. Lysosomal degradation of cholecystokinin-(29-33)-amide in mouse brain is dependent on tripeptidyl peptidase-I: implications for the degradation and storage of peptides in classical late-infantile neuronal ceroid lipofuscinosis.
Bernardini F; Warburton MJ
Biochem J; 2002 Sep; 366(Pt 2):521-9. PubMed ID: 12038963
[TBL] [Abstract][Full Text] [Related]
3. Human iPSC models of neuronal ceroid lipofuscinosis capture distinct effects of TPP1 and CLN3 mutations on the endocytic pathway.
Lojewski X; Staropoli JF; Biswas-Legrand S; Simas AM; Haliw L; Selig MK; Coppel SH; Goss KA; Petcherski A; Chandrachud U; Sheridan SD; Lucente D; Sims KB; Gusella JF; Sondhi D; Crystal RG; Reinhardt P; Sterneckert J; Schöler H; Haggarty SJ; Storch A; Hermann A; Cotman SL
Hum Mol Genet; 2014 Apr; 23(8):2005-22. PubMed ID: 24271013
[TBL] [Abstract][Full Text] [Related]
4. Glial fibrillary acidic protein is elevated in the lysosomal storage disease classical late-infantile neuronal ceroid lipofuscinosis, but is not a component of the storage material.
Xu S; Sleat DE; Jadot M; Lobel P
Biochem J; 2010 May; 428(3):355-62. PubMed ID: 20370715
[TBL] [Abstract][Full Text] [Related]
5. Extracellular Vesicles Released by Genetically Modified Macrophages Activate Autophagy and Produce Potent Neuroprotection in Mouse Model of Lysosomal Storage Disorder, Batten Disease.
El-Hage N; Haney MJ; Zhao Y; Rodriguez M; Wu Z; Liu M; Swain CJ; Yuan H; Batrakova EV
Cells; 2023 May; 12(11):. PubMed ID: 37296618
[TBL] [Abstract][Full Text] [Related]
6. Mutation update: Review of TPP1 gene variants associated with neuronal ceroid lipofuscinosis CLN2 disease.
Gardner E; Bailey M; Schulz A; Aristorena M; Miller N; Mole SE
Hum Mutat; 2019 Nov; 40(11):1924-1938. PubMed ID: 31283065
[TBL] [Abstract][Full Text] [Related]
7. Tripeptidyl peptidases: enzymes that count.
Tomkinson B
Trends Biochem Sci; 1999 Sep; 24(9):355-9. PubMed ID: 10470035
[TBL] [Abstract][Full Text] [Related]
8. Untargeted Metabolite Profiling of Cerebrospinal Fluid Uncovers Biomarkers for Severity of Late Infantile Neuronal Ceroid Lipofuscinosis (CLN2, Batten Disease).
Sindelar M; Dyke JP; Deeb RS; Sondhi D; Kaminsky SM; Kosofsky BE; Ballon DJ; Crystal RG; Gross SS
Sci Rep; 2018 Oct; 8(1):15229. PubMed ID: 30323181
[TBL] [Abstract][Full Text] [Related]
9. Neural stem cells for disease modeling and evaluation of therapeutics for infantile (CLN1/PPT1) and late infantile (CLN2/TPP1) neuronal ceroid lipofuscinoses.
Sima N; Li R; Huang W; Xu M; Beers J; Zou J; Titus S; Ottinger EA; Marugan JJ; Xie X; Zheng W
Orphanet J Rare Dis; 2018 Apr; 13(1):54. PubMed ID: 29631617
[TBL] [Abstract][Full Text] [Related]
10. Diagnosis of neuronal ceroid lipofuscinosis type 2 (CLN2 disease): Expert recommendations for early detection and laboratory diagnosis.
Fietz M; AlSayed M; Burke D; Cohen-Pfeffer J; Cooper JD; Dvořáková L; Giugliani R; Izzo E; Jahnová H; Lukacs Z; Mole SE; Noher de Halac I; Pearce DA; Poupetova H; Schulz A; Specchio N; Xin W; Miller N
Mol Genet Metab; 2016 Sep; 119(1-2):160-7. PubMed ID: 27553878
[TBL] [Abstract][Full Text] [Related]
11. Gemfibrozil, food and drug administration-approved lipid-lowering drug, increases longevity in mouse model of late infantile neuronal ceroid lipofuscinosis.
Ghosh A; Rangasamy SB; Modi KK; Pahan K
J Neurochem; 2017 May; 141(3):423-435. PubMed ID: 28199020
[TBL] [Abstract][Full Text] [Related]
12. Upregulation of tripeptidyl-peptidase 1 by 3-hydroxy-(2,2)-dimethyl butyrate, a brain endogenous ligand of PPARα: Implications for late-infantile Batten disease therapy.
Chakrabarti S; Chandra S; Roy A; Dasarathi S; Kundu M; Pahan K
Neurobiol Dis; 2019 Jul; 127():362-373. PubMed ID: 30928643
[TBL] [Abstract][Full Text] [Related]
13. A mouse model of classical late-infantile neuronal ceroid lipofuscinosis based on targeted disruption of the CLN2 gene results in a loss of tripeptidyl-peptidase I activity and progressive neurodegeneration.
Sleat DE; Wiseman JA; El-Banna M; Kim KH; Mao Q; Price S; Macauley SL; Sidman RL; Shen MM; Zhao Q; Passini MA; Davidson BL; Stewart GR; Lobel P
J Neurosci; 2004 Oct; 24(41):9117-26. PubMed ID: 15483130
[TBL] [Abstract][Full Text] [Related]
14. A zebrafish model of CLN2 disease is deficient in tripeptidyl peptidase 1 and displays progressive neurodegeneration accompanied by a reduction in proliferation.
Mahmood F; Fu S; Cooke J; Wilson SW; Cooper JD; Russell C
Brain; 2013 May; 136(Pt 5):1488-507. PubMed ID: 23587805
[TBL] [Abstract][Full Text] [Related]
15. A novel CLN2/TPP1 mutation in a patient with late infantile neuronal ceroid lipofuscinosis.
Yu F; Liu XM; Chen YH; Zhang SQ; Wang K
Neurol Sci; 2015 Oct; 36(10):1917-9. PubMed ID: 26032578
[No Abstract] [Full Text] [Related]
16. Intrathecal tripeptidyl-peptidase 1 reduces lysosomal storage in a canine model of late infantile neuronal ceroid lipofuscinosis.
Vuillemenot BR; Katz ML; Coates JR; Kennedy D; Tiger P; Kanazono S; Lobel P; Sohar I; Xu S; Cahayag R; Keve S; Koren E; Bunting S; Tsuruda LS; O'Neill CA
Mol Genet Metab; 2011 Nov; 104(3):325-37. PubMed ID: 21784683
[TBL] [Abstract][Full Text] [Related]
17. Analysis of Brain and Cerebrospinal Fluid from Mouse Models of the Three Major Forms of Neuronal Ceroid Lipofuscinosis Reveals Changes in the Lysosomal Proteome.
Sleat DE; Wiseman JA; El-Banna M; Zheng H; Zhao C; Soherwardy A; Moore DF; Lobel P
Mol Cell Proteomics; 2019 Nov; 18(11):2244-2261. PubMed ID: 31501224
[TBL] [Abstract][Full Text] [Related]
18. Tripeptidyl peptidase I, the late infantile neuronal ceroid lipofuscinosis gene product, initiates the lysosomal degradation of subunit c of ATP synthase.
Ezaki J; Takeda-Ezaki M; Kominami E
J Biochem; 2000 Sep; 128(3):509-16. PubMed ID: 10965052
[TBL] [Abstract][Full Text] [Related]
19. Multifocal retinopathy in Dachshunds with CLN2 neuronal ceroid lipofuscinosis.
Whiting RE; Pearce JW; Castaner LJ; Jensen CA; Katz RJ; Gilliam DH; Katz ML
Exp Eye Res; 2015 May; 134():123-32. PubMed ID: 25697710
[TBL] [Abstract][Full Text] [Related]
20. A lysosomal proteinase, the late infantile neuronal ceroid lipofuscinosis gene (CLN2) product, is essential for degradation of a hydrophobic protein, the subunit c of ATP synthase.
Ezaki J; Tanida I; Kanehagi N; Kominami E
J Neurochem; 1999 Jun; 72(6):2573-82. PubMed ID: 10349869
[TBL] [Abstract][Full Text] [Related]
[Next] [New Search]