Cyclodextrin overcomes the transport defect in nearly every organ of NPC1 mice leading to excretion of sequestered cholesterol as bile acid - Test2 - elhamkhani - TriplyDB

Cyclodextrin overcomes the transport defect in nearly every organ of NPC1 mice leading to excretion of sequestered cholesterol as bile acid

Cyclodextrin overcomes the transport defect in nearly every organ of NPC1 mice leading to excretion of sequestered cholesterol as bile acid

http://www.wikidata.org/entity/Q33784624

about

2-hydroxypropyl-beta-cyclodextrin raises hearing threshold in normal cats and in cats with Niemann-Pick type C disease Diagnostic workup and management of patients with suspected Niemann-Pick type C disease Collaborative development of 2-hydroxypropyl-β-cyclodextrin for the treatment of Niemann-Pick type C1 disease Hearing loss and hair cell death in mice given the cholesterol-chelating agent hydroxypropyl-β-cyclodextrin Cyclodextrins as Emerging Therapeutic Tools in the Treatment of Cholesterol-Associated Vascular and Neurodegenerative Diseases Cyclodextrin induces calcium-dependent lysosomal exocytosis Protein replacement therapy partially corrects the cholesterol-storage phenotype in a mouse model of Niemann-Pick type C2 disease Activation mobilizes the cholesterol in the late endosomes-lysosomes of Niemann Pick type C cells The small GTPase Cdc42 interacts with Niemann-Pick C1-like 1 (NPC1L1) and controls its movement from endocytic recycling compartment to plasma membrane in a cholesterol-dependent manner Efficacy and ototoxicity of different cyclodextrins in Niemann-Pick C disease Visual evoked potentials of Niemann-Pick type C1 mice reveal an impairment of the visual pathway that is rescued by 2-hydroxypropyl-ß-cyclodextrin Intracisternal cyclodextrin prevents cerebellar dysfunction and Purkinje cell death in feline Niemann-Pick type C1 disease.Pluronic based β-cyclodextrin polyrotaxanes for treatment of Niemann-Pick Type C disease.Development and validation of sensitive LC-MS/MS assays for quantification of HP-β-CD in human plasma and CSF.Niemann-Pick C disease and mobilization of lysosomal cholesterol by cyclodextrin Cholesterol, the central lipid of mammalian cells Genetic connections between neurological disorders and cholesterol metabolism Therapeutic potential of cyclodextrins in the treatment of Niemann-Pick type C disease.Cell cholesterol homeostasis: mediation by active cholesterol Hepatic entrapment of esterified cholesterol drives continual expansion of whole body sterol pool in lysosomal acid lipase-deficient mice Psychosine, the cytotoxic sphingolipid that accumulates in globoid cell leukodystrophy, alters membrane architecture.Cholesterol homeostatic responses provide biomarkers for monitoring treatment for the neurodegenerative disease Niemann-Pick C1 (NPC1)Sensitivity to lysosome-dependent cell death is directly regulated by lysosomal cholesterol content Synthesis, characterization, and evaluation of pluronic-based β-cyclodextrin polyrotaxanes for mobilization of accumulated cholesterol from Niemann-Pick type C fibroblasts.Pulmonary abnormalities in animal models due to Niemann-Pick type C1 (NPC1) or C2 (NPC2) disease Filipin recognizes both GM1 and cholesterol in GM1 gangliosidosis mouse brain.Rescue of an in vitro neuron phenotype identified in Niemann-Pick disease, type C1 induced pluripotent stem cell-derived neurons by modulating the WNT pathway and calcium signaling Cholesterol oxidation products are sensitive and specific blood-based biomarkers for Niemann-Pick C1 disease Amino acid substitution in NPC1 that abolishes cholesterol binding reproduces phenotype of complete NPC1 deficiency in mice.Effects of cyclodextrins on GM1-gangliosides in fibroblasts from GM1-gangliosidosis patients.A murine Niemann-Pick C1 I1061T knock-in model recapitulates the pathological features of the most prevalent human disease allele.Quantitative role of LAL, NPC2, and NPC1 in lysosomal cholesterol processing defined by genetic and pharmacological manipulations.Normalization of cholesterol homeostasis by 2-hydroxypropyl-β-cyclodextrin in neurons and glia from Niemann-Pick C1 (NPC1)-deficient mice Systemic administration of 2-hydroxypropyl-β-cyclodextrin to symptomatic Npc1-deficient mice slows cholesterol sequestration in the major organs and improves liver function Neuronal and epithelial cell rescue resolves chronic systemic inflammation in the lipid storage disorder Niemann-Pick C.Microarray expression analysis and identification of serum biomarkers for Niemann-Pick disease, type C1.Weekly Treatment of 2-Hydroxypropyl-β-cyclodextrin Improves Intracellular Cholesterol Levels in LDL Receptor Knockout Mice Intrathecal 2-hydroxypropyl-beta-cyclodextrin in a single patient with Niemann-Pick C1.Cyclodextrin mediates rapid changes in lipid balance in Npc1-/- mice without carrying cholesterol through the bloodstream.Neuroprotection by cyclodextrin in cell and mouse models of Alzheimer disease

P2860

Q24598867-2E9E6659-7A8E-4BD0-9907-22D341463841 Q26748586-1C77EB75-2519-4942-9F72-529B7674146A Q27006029-D160B6A1-9BCA-4810-97CB-16D53D83DB9D Q27322555-D54A001F-21AF-4DEF-83D9-63863DF772B8 Q28071254-C836A542-7025-4FB2-BB5F-09A8EFE1AF4F Q28476365-8F7F7A3E-1984-43DB-9D60-847483D7A38D Q28477793-95ED9664-80CF-4C60-BE44-DF2CB030553B Q28479001-2B9E0C70-4394-48CB-8DA0-2CCF2F0F3412 Q28580370-8BFE0903-D4E7-4F9E-B6E0-647A3EB92448 Q28834418-1F8BE06F-3E5A-4EF6-BB3D-B915C0237ABF Q30401160-2E38DDB4-702E-44E7-A7C8-7AFD7D761F2F Q30645985-66A7E3CF-AE47-491D-858D-F646B15C63FF Q33612118-DD7FB5F7-8887-4581-BFA3-5F13744D1B27 Q33825055-AE693606-4BC9-4228-BB01-C37A0C9FFFC9 Q33945068-724C9AF7-5954-4E7B-B67B-75FD62F5D0F2 Q34021021-35C3EF99-952B-410B-B5C2-D4B7F512480E Q34053056-898143B9-B3A5-47AD-8FD3-A219FA3C8D78 Q34069183-F0CFC2B9-AAF5-4ADC-8480-BC778E10368D Q34258914-4C5B17CB-785B-428F-A806-4DE949B24576 Q34356070-6521AE8F-BCE9-45F2-83B8-62F1841C2F03 Q34368767-A3CDE8EB-7F56-481C-A65C-36FC54D55C1A Q34379062-C0116510-7023-49B9-BA89-143DE730E7A0 Q34483105-6BC5A7E2-D3E1-4A1A-BD43-AB1AA3F29228 Q34656648-A4337FE5-302C-4136-B30F-6DF65FE02F4E Q34812413-25D109DD-7072-4CF5-9601-71D8ACD9AACB Q35067305-DAFE37B0-C734-4941-9682-27E6BC720349 Q35119670-3CA1B82E-361F-4EC3-BACB-FC3D487D5A84 Q35205918-A5627768-3F4D-448B-9637-A4F60D542078 Q35217180-0AE29312-54D0-49C0-A37B-E7B3E69E988E Q35597034-5510898F-E75C-42F3-808B-94BB7A6D0264 Q35649458-F1C677ED-7416-4F40-A045-4303E4EE9958 Q35771137-236A939B-3861-40E5-AC2A-9EFF1958FE75 Q35841853-5868BE32-D657-4303-9360-A4E2EEB7DFE6 Q35920269-3A9CB3DE-C80C-43FF-9392-7606025CAF71 Q36026905-DBFFB37A-09B4-479C-9116-5905F561D600 Q36122424-D10C961E-5F03-488B-843B-4BBDE009C71A Q36184055-8588C868-2D15-44C1-A980-16F4B9267B83 Q36248727-6AD08585-2E6C-42EF-AF8D-A1949E39919C Q36302736-FEB13C5F-0681-46C5-A370-3A154A6D5D94 Q36478244-7AD577B9-F7D1-4D10-8DDE-D700C21655A6

P2860

Cyclodextrin overcomes the transport defect in nearly every organ of NPC1 mice leading to excretion of sequestered cholesterol as bile acid

http://www.wikidata.org/entity/Q33784624

description

2009 nî lūn-bûn

@nan

2009 թուականի Նոյեմբերին հրատարակուած գիտական յօդուած

@hyw

2009 թվականի նոյեմբերին հրատարակված գիտական հոդված

@hy

2009年の論文

@ja

2009年論文

@yue

2009年論文

@zh-hant

2009年論文

@zh-hk

2009年論文

@zh-mo

2009年論文

@zh-tw

2009年论文

@wuu

name

Cyclodextrin overcomes the tra ...... tered cholesterol as bile acid

@ast

Cyclodextrin overcomes the tra ...... tered cholesterol as bile acid

@en

type

label

Cyclodextrin overcomes the tra ...... tered cholesterol as bile acid

@ast

Cyclodextrin overcomes the tra ...... tered cholesterol as bile acid

@en

prefLabel

Cyclodextrin overcomes the tra ...... tered cholesterol as bile acid

@ast

Cyclodextrin overcomes the tra ...... tered cholesterol as bile acid

@en

P1433

Q33784624-34E30961-F6F1-4AA1-B236-C5BE3A05A49C

P1476

Q33784624-FA02F3DF-0D75-4C0D-BD0F-A9D0D3BC5CC2

P2093

Q33784624-74FF93E4-FA95-43EF-88DF-EC003A2441CD

Q33784624-79AC8DCD-2B35-4185-919E-FA243E972A41

Q33784624-8F410650-129A-47C0-B231-25BA99B819F4

Q33784624-D25E5453-B75C-4A0C-8754-5BE3321C7F94

Q33784624-E02CB746-ED28-477F-B016-A707A9084971

Q33784624-F62AAAA9-E619-49AB-9CFC-1945CDDE7506

P2860

Q33784624-0FFC67FA-63D4-432E-8387-6DFA50FDA699

Q33784624-1011D28C-E62B-4630-BD74-93201C96A2F4

Q33784624-18FF6641-A2F0-46CE-BA62-E415A86FB760

Q33784624-1956F420-3F10-458F-9915-F75DCD9CE3EC

Q33784624-272741ED-3AF6-482D-ACC5-9F13D0F107B4

Q33784624-2E064575-37C6-4169-8F5E-F26AF36A787F

Q33784624-2E7B4A00-5F88-4D9E-AB1C-2542FD700816

Q33784624-3CBFA8A3-BF4B-4739-A902-08DCF541D3F0

Q33784624-411D8516-0C2B-41A6-9C24-ACAAF2333560

Q33784624-4E84687D-9D34-4634-B532-F6C8110298C4

P304

Q33784624-62209FF7-FCDD-4C7A-ACED-91767008B477

P31

Q33784624-B8481A16-C4E2-49FB-B20F-D3D9639AB381

P356

Q33784624-E0B3035F-A77F-4BA7-A050-9DB397C3FA9C

P433

Q33784624-F0658F34-BB6A-49DD-AB62-287F8A3E4DED

P478

Q33784624-434A3588-6C0D-4FB6-85F9-0550B2EDD822

P577

Q33784624-74EB250C-2AA6-4AFB-AF79-C965631AFB0E

P5875

Q33784624-DD046CDE-67DD-4171-AA77-7078A0C01B7D

P698

Q33784624-4C59AA73-FDE9-46C4-A460-21B8F437F011

P932

Q33784624-96E9F788-950E-402A-A6B1-295B59B48621

P356

P1433

Journal of Lipid Research

P1476

Cyclodextrin overcomes the tra ...... tered cholesterol as bile acid

@en

P2093

Anna M Miller

http://www.w3.org/2001/XMLSchema#string

Benny Liu

http://www.w3.org/2001/XMLSchema#string

Charina M Ramirez

http://www.w3.org/2001/XMLSchema#string

John M Dietschy

http://www.w3.org/2001/XMLSchema#string

Joyce J Repa

http://www.w3.org/2001/XMLSchema#string

Stephen D Turley

http://www.w3.org/2001/XMLSchema#string

P2860

Identification of HE1 as the second gene of Niemann-Pick C disease

Niemann-Pick C1 disease gene: homology to mediators of cholesterol homeostasis

Sterol-regulated transport of SREBPs from endoplasmic reticulum to Golgi: Insig renders sorting signal in Scap inaccessible to COPII proteins

Analyzing real-time PCR data by the comparative C(T) method

A receptor-mediated pathway for cholesterol homeostasis

Protein sensors for membrane sterols

Quantitative real-time polymerase chain reaction measurement of regulators of G-protein signaling mRNA levels in mouse tissues.

Neurons in Niemann-Pick disease type C accumulate gangliosides as well as unesterified cholesterol and undergo dendritic and axonal alterations.

Nuclear receptor regulation of cholesterol and bile acid metabolism.

The Niemann-Pick C1 protein resides in a vesicular compartment linked to retrograde transport of multiple lysosomal cargo.

P304

933-944

http://www.w3.org/2001/XMLSchema#string

P31

scholarly article

P356

10.1194/JLR.M000257

http://www.w3.org/2001/XMLSchema#string

P433

5

http://www.w3.org/2001/XMLSchema#string

P478

51

http://www.w3.org/2001/XMLSchema#string

P577

2009-11-18T00:00:00Z

http://www.w3.org/2001/XMLSchema#dateTime

P5875

40452570

http://www.w3.org/2001/XMLSchema#string

P698

19965601

http://www.w3.org/2001/XMLSchema#string

P932

2853461

http://www.w3.org/2001/XMLSchema#string