265 related articles for article (PubMed ID: 29079454)
21. Presymptomatic Alterations in Amino Acid Metabolism and DNA Methylation in the Cerebellum of a Murine Model of Niemann-Pick Type C Disease.
Kennedy BE; Hundert AS; Goguen D; Weaver IC; Karten B
Am J Pathol; 2016 Jun; 186(6):1582-97. PubMed ID: 27083515
[TBL] [Abstract][Full Text] [Related]
22. Repurposing Dipyridamole in Niemann Pick Type C Disease: A Proof of Concept Study.
Pepponi R; De Simone R; De Nuccio C; Visentin S; Matteucci A; Bernardo A; Popoli P; Ferrante A
Int J Mol Sci; 2022 Mar; 23(7):. PubMed ID: 35408815
[TBL] [Abstract][Full Text] [Related]
23. Decreased estradiol release from astrocytes contributes to the neurodegeneration in a mouse model of Niemann-Pick disease type C.
Chen G; Li HM; Chen YR; Gu XS; Duan S
Glia; 2007 Nov; 55(15):1509-18. PubMed ID: 17705200
[TBL] [Abstract][Full Text] [Related]
24. Mitochondrial GSH replenishment as a potential therapeutic approach for Niemann Pick type C disease.
Torres S; Matías N; Baulies A; Nuñez S; Alarcon-Vila C; Martinez L; Nuño N; Fernandez A; Caballeria J; Levade T; Gonzalez-Franquesa A; Garcia-Rovés P; Balboa E; Zanlungo S; Fabrías G; Casas J; Enrich C; Garcia-Ruiz C; Fernández-Checa JC
Redox Biol; 2017 Apr; 11():60-72. PubMed ID: 27888692
[TBL] [Abstract][Full Text] [Related]
25. The natural history of cerebellar degeneration of Niemann-Pick C mice monitored in vitro.
Marschalek N; Albert F; Meske V; Ohm TG
Neuropathol Appl Neurobiol; 2014 Dec; 40(7):933-45. PubMed ID: 24889722
[TBL] [Abstract][Full Text] [Related]
26. A murine Niemann-Pick C1 I1061T knock-in model recapitulates the pathological features of the most prevalent human disease allele.
Praggastis M; Tortelli B; Zhang J; Fujiwara H; Sidhu R; Chacko A; Chen Z; Chung C; Lieberman AP; Sikora J; Davidson C; Walkley SU; Pipalia NH; Maxfield FR; Schaffer JE; Ory DS
J Neurosci; 2015 May; 35(21):8091-106. PubMed ID: 26019327
[TBL] [Abstract][Full Text] [Related]
27. The susceptibility of cochlear outer hair cells to cyclodextrin is not related to their electromotile activity.
Zhou Y; Takahashi S; Homma K; Duan C; Zheng J; Cheatham MA; Zheng J
Acta Neuropathol Commun; 2018 Sep; 6(1):98. PubMed ID: 30249300
[TBL] [Abstract][Full Text] [Related]
28. Identification of Novel Pathways Associated with Patterned Cerebellar Purkinje Neuron Degeneration in Niemann-Pick Disease, Type C1.
Martin KB; Williams IM; Cluzeau CV; Cougnoux A; Dale RK; Iben JR; Cawley NX; Wassif CA; Porter FD
Int J Mol Sci; 2019 Dec; 21(1):. PubMed ID: 31906248
[No Abstract] [Full Text] [Related]
29. Conditional Niemann-Pick C mice demonstrate cell autonomous Purkinje cell neurodegeneration.
Elrick MJ; Pacheco CD; Yu T; Dadgar N; Shakkottai VG; Ware C; Paulson HL; Lieberman AP
Hum Mol Genet; 2010 Mar; 19(5):837-47. PubMed ID: 20007718
[TBL] [Abstract][Full Text] [Related]
30. Reduced cerebellar neurodegeneration after combined therapy with cyclodextrin/allopregnanolone and miglustat in NPC1: a mouse model of Niemann-Pick type C1 disease.
Maass F; Petersen J; Hovakimyan M; Schmitt O; Witt M; Hawlitschka A; Lukas J; Rolfs A; Wree A
J Neurosci Res; 2015 Mar; 93(3):433-42. PubMed ID: 25400034
[TBL] [Abstract][Full Text] [Related]
31. AAV9-NPC1 significantly ameliorates Purkinje cell death and behavioral abnormalities in mouse NPC disease.
Xie C; Gong XM; Luo J; Li BL; Song BL
J Lipid Res; 2017 Mar; 58(3):512-518. PubMed ID: 28053186
[TBL] [Abstract][Full Text] [Related]
32. Gastrointestinal Tract Pathology in a BALB/c Niemann-Pick Disease Type C1 Null Mouse Model.
Cougnoux A; Movassaghi M; Picache JA; Iben JR; Navid F; Salman A; Martin K; Farhat NY; Cluzeau C; Tseng WC; Burkert K; Sojka C; Wassif CA; Cawley NX; Bonnet R; Porter FD
Dig Dis Sci; 2018 Apr; 63(4):870-880. PubMed ID: 29357083
[TBL] [Abstract][Full Text] [Related]
33. BACE1-cleavage of Sez6 and Sez6L is elevated in Niemann-Pick type C disease mouse brains.
Causevic M; Dominko K; Malnar M; Vidatic L; Cermak S; Pigoni M; Kuhn PH; Colombo A; Havas D; Flunkert S; McDonald J; Gunnersen JM; Hutter-Paier B; Tahirovic S; Windisch M; Krainc D; Lichtenthaler SF; Hecimovic S
PLoS One; 2018; 13(7):e0200344. PubMed ID: 29979789
[TBL] [Abstract][Full Text] [Related]
34. Imatinib therapy blocks cerebellar apoptosis and improves neurological symptoms in a mouse model of Niemann-Pick type C disease.
Alvarez AR; Klein A; Castro J; Cancino GI; Amigo J; Mosqueira M; Vargas LM; Yévenes LF; Bronfman FC; Zanlungo S
FASEB J; 2008 Oct; 22(10):3617-27. PubMed ID: 18591368
[TBL] [Abstract][Full Text] [Related]
35. Altered levels and distribution of amyloid precursor protein and its processing enzymes in Niemann-Pick type C1-deficient mouse brains.
Kodam A; Maulik M; Peake K; Amritraj A; Vetrivel KS; Thinakaran G; Vance JE; Kar S
Glia; 2010 Aug; 58(11):1267-81. PubMed ID: 20607864
[TBL] [Abstract][Full Text] [Related]
36. Evaluation of the Potential Role of Proprotein Convertase Subtilisin/Kexin Type 9 (PCSK9) in Niemann-Pick Disease, Type C1.
Cawley NX; Lyons AT; Abebe D; Wassif CA; Porter FD
Int J Mol Sci; 2020 Mar; 21(7):. PubMed ID: 32244519
[TBL] [Abstract][Full Text] [Related]
37. Increased activity and altered subcellular distribution of lysosomal enzymes determine neuronal vulnerability in Niemann-Pick type C1-deficient mice.
Amritraj A; Peake K; Kodam A; Salio C; Merighi A; Vance JE; Kar S
Am J Pathol; 2009 Dec; 175(6):2540-56. PubMed ID: 19893049
[TBL] [Abstract][Full Text] [Related]
38. Normalization of Hepatic Homeostasis in the
Munkacsi AB; Hammond N; Schneider RT; Senanayake DS; Higaki K; Lagutin K; Bloor SJ; Ory DS; Maue RA; Chen FW; Hernandez-Ono A; Dahlson N; Repa JJ; Ginsberg HN; Ioannou YA; Sturley SL
J Biol Chem; 2017 Mar; 292(11):4395-4410. PubMed ID: 28031458
[TBL] [Abstract][Full Text] [Related]
39. Loss of amyloid precursor protein exacerbates early inflammation in Niemann-Pick disease type C.
Shin SD; Shin A; Mayagoitia K; Siebold L; Rubini M; Wilson CG; Bellinger DL; Soriano S
J Neuroinflammation; 2019 Dec; 16(1):269. PubMed ID: 31847862
[TBL] [Abstract][Full Text] [Related]
40. Ultrastructure of spinal anterior horn cells in human Niemann-Pick type C (NPC) patient and mouse model of NPC with retroposon insertion in NPC1 genes.
Kusunoki-Ii M; Kohama H; Kato K; Nomura Y; Nagashima K; Ninomiya H; Kato M; Kato S
Pathol Int; 2020 Jul; 70(7):422-432. PubMed ID: 32342600
[TBL] [Abstract][Full Text] [Related]
[Previous] [Next] [New Search]
