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Items: 1 to 20 of 53

1.
2.

A Basic ApoE-Based Peptide Mediator to Deliver Proteins across the Blood-Brain Barrier: Long-Term Efficacy, Toxicity, and Mechanism.

Meng Y, Wiseman JA, Nemtsova Y, Moore DF, Guevarra J, Reuhl K, Banks WA, Daneman R, Sleat DE, Lobel P.

Mol Ther. 2017 Jul 5;25(7):1531-1543. doi: 10.1016/j.ymthe.2017.03.037. Epub 2017 Apr 26.

PMID:
28456380
3.

Oral Health Status of Patients with Lysosomal Storage Diseases in Poland.

Drążewski D, Grzymisławska M, Korybalska K, Czepulis N, Grzymisławski M, Witowski J, Surdacka A.

Int J Environ Res Public Health. 2017 Mar 9;14(3). pii: E281. doi: 10.3390/ijerph14030281.

4.

Two factor-based reprogramming of rodent and human fibroblasts into Schwann cells.

Mazzara PG, Massimino L, Pellegatta M, Ronchi G, Ricca A, Iannielli A, Giannelli SG, Cursi M, Cancellieri C, Sessa A, Del Carro U, Quattrini A, Geuna S, Gritti A, Taveggia C, Broccoli V.

Nat Commun. 2017 Feb 7;8:14088. doi: 10.1038/ncomms14088.

5.

Lysosomal dysfunction disrupts presynaptic maintenance and restoration of presynaptic function prevents neurodegeneration in lysosomal storage diseases.

Sambri I, D'Alessio R, Ezhova Y, Giuliano T, Sorrentino NC, Cacace V, De Risi M, Cataldi M, Annunziato L, De Leonibus E, Fraldi A.

EMBO Mol Med. 2017 Jan;9(1):112-132. doi: 10.15252/emmm.201606965.

6.

Efficacy of phosphodiesterase-4 inhibitors in juvenile Batten disease (CLN3).

Aldrich A, Bosch ME, Fallet R, Odvody J, Burkovetskaya M, Rama Rao KV, Cooper JD, Drack AV, Kielian T.

Ann Neurol. 2016 Dec;80(6):909-923. doi: 10.1002/ana.24815. Epub 2016 Nov 23.

7.

The Parkinson's disease-associated genes ATP13A2 and SYT11 regulate autophagy via a common pathway.

Bento CF, Ashkenazi A, Jimenez-Sanchez M, Rubinsztein DC.

Nat Commun. 2016 Jun 9;7:11803. doi: 10.1038/ncomms11803.

8.

The Nuclear Zinc Finger Protein Zfat Maintains FoxO1 Protein Levels in Peripheral T Cells by Regulating the Activities of Autophagy and the Akt Signaling Pathway.

Ishikura S, Iwaihara Y, Tanaka Y, Luo H, Nishi K, Doi K, Koyanagi M, Okamura T, Tsunoda T, Shirasawa S.

J Biol Chem. 2016 Jul 15;291(29):15282-91. doi: 10.1074/jbc.M116.723734. Epub 2016 May 20.

9.

Deacetylation of TFEB promotes fibrillar Aβ degradation by upregulating lysosomal biogenesis in microglia.

Bao J, Zheng L, Zhang Q, Li X, Zhang X, Li Z, Bai X, Zhang Z, Huo W, Zhao X, Shang S, Wang Q, Zhang C, Ji J.

Protein Cell. 2016 Jun;7(6):417-33. doi: 10.1007/s13238-016-0269-2. Epub 2016 May 21.

10.

Heat Shock Protein Beta-1 Modifies Anterior to Posterior Purkinje Cell Vulnerability in a Mouse Model of Niemann-Pick Type C Disease.

Chung C, Elrick MJ, Dell'Orco JM, Qin ZS, Kalyana-Sundaram S, Chinnaiyan AM, Shakkottai VG, Lieberman AP.

PLoS Genet. 2016 May 6;12(5):e1006042. doi: 10.1371/journal.pgen.1006042. eCollection 2016 May.

11.

Disease models for the development of therapies for lysosomal storage diseases.

Xu M, Motabar O, Ferrer M, Marugan JJ, Zheng W, Ottinger EA.

Ann N Y Acad Sci. 2016 May;1371(1):15-29. doi: 10.1111/nyas.13052. Epub 2016 May 4. Review.

12.

Age-dependent development of liver fibrosis in Glmp (gt/gt) mice.

Nesset CK, Kong XY, Damme M, Schjalm C, Roos N, Løberg EM, Eskild W.

Fibrogenesis Tissue Repair. 2016 Apr 28;9:5. doi: 10.1186/s13069-016-0042-4. eCollection 2016.

13.

Moving towards effective therapeutic strategies for Neuronal Ceroid Lipofuscinosis.

Geraets RD, Koh Sy, Hastings ML, Kielian T, Pearce DA, Weimer JM.

Orphanet J Rare Dis. 2016 Apr 16;11:40. doi: 10.1186/s13023-016-0414-2. Review.

14.

Lysosomal Storage Diseases-Regulating Neurodegeneration.

Onyenwoke RU, Brenman JE.

J Exp Neurosci. 2016 Apr 5;9(Suppl 2):81-91. doi: 10.4137/JEN.S25475. eCollection 2015. Review.

15.

Arf-like GTPase Arl8: Moving from the periphery to the center of lysosomal biology.

Khatter D, Sindhwani A, Sharma M.

Cell Logist. 2015 Sep 21;5(3):e1086501. eCollection 2015 Jul-Sep. Review.

16.

Lysosome and endoplasmic reticulum quality control pathways in Niemann-Pick type C disease.

Schultz ML, Krus KL, Lieberman AP.

Brain Res. 2016 Oct 15;1649(Pt B):181-188. doi: 10.1016/j.brainres.2016.03.035. Epub 2016 Mar 26. Review.

17.

Genetic and pharmacological evidence implicates cathepsins in Niemann-Pick C cerebellar degeneration.

Chung C, Puthanveetil P, Ory DS, Lieberman AP.

Hum Mol Genet. 2016 Apr 1;25(7):1434-46. doi: 10.1093/hmg/ddw025. Epub 2016 Jan 28.

18.

Distinct Entry Mechanisms for Nonenveloped and Quasi-Enveloped Hepatitis E Viruses.

Yin X, Ambardekar C, Lu Y, Feng Z.

J Virol. 2016 Mar 28;90(8):4232-42. doi: 10.1128/JVI.02804-15. Print 2016 Apr.

19.

MAP1LC3B overexpression protects against Hermansky-Pudlak syndrome type-1-induced defective autophagy in vitro.

Ahuja S, Knudsen L, Chillappagari S, Henneke I, Ruppert C, Korfei M, Gochuico BR, Bellusci S, Seeger W, Ochs M, Guenther A, Mahavadi P.

Am J Physiol Lung Cell Mol Physiol. 2016 Mar 15;310(6):L519-31. doi: 10.1152/ajplung.00213.2015. Epub 2015 Dec 30.

20.

Activities of genes controlling sphingolipid metabolism in human fibroblasts treated with flavonoids.

Moskot M, Jakóbkiewicz-Banecka J, Smolińska E, Banecki B, Węgrzyn G, Gabig-Cimińska M.

Metab Brain Dis. 2015 Oct;30(5):1257-67. doi: 10.1007/s11011-015-9705-x. Epub 2015 Jul 26. Erratum in: Metab Brain Dis. 2017 Feb 22;:. Metab Brain Dis. 2017 Apr;32(2):649.

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