Secondary accumulation of gangliosides in lysosomal storage disorders.
about
Chronic cyclodextrin treatment of murine Niemann-Pick C disease ameliorates neuronal cholesterol and glycosphingolipid storage and disease progressionSanfilippo syndrome: causes, consequences, and treatmentsAtaxia is the major neuropathological finding in arylsulfatase G-deficient mice: similarities and dissimilarities to Sanfilippo disease (mucopolysaccharidosis type III).Pathology of GM2 gangliosidosis in Jacob sheepEarly neurodegeneration progresses independently of microglial activation by heparan sulfate in the brain of mucopolysaccharidosis IIIB miceCerebellar alterations and gait defects as therapeutic outcome measures for enzyme replacement therapy in α-mannosidosis.Retroviral-vector-mediated gene therapy to mucopolysaccharidosis I mice improves sensorimotor impairments and other behavioral deficits.Cell- and gene-based therapeutic approaches for neurological deficits in mucopolysaccharidosesBlood-brain barrier impairment in an animal model of MPS III BMulti-system disorders of glycosphingolipid and ganglioside metabolismCommon and uncommon pathogenic cascades in lysosomal storage diseasesSafe, efficient, and reproducible gene therapy of the brain in the dog models of Sanfilippo and Hurler syndromes.Neuropathology in mouse models of mucopolysaccharidosis type I, IIIA and IIIBSecondary storage of dermatan sulfate in Sanfilippo disease.Brain pathology in Niemann Pick disease type A: insights from the acid sphingomyelinase knockout miceBlood-brain barrier impairment in MPS III patients.Filipin recognizes both GM1 and cholesterol in GM1 gangliosidosis mouse brain.Glycosaminoglycan storage in neuroanatomical regions of mucopolysaccharidosis I dogs following intrathecal recombinant human iduronidase.Endosomal/lysosomal processing of gangliosides affects neuronal cholesterol sequestration in Niemann-Pick disease type C.Secondary lipid accumulation in lysosomal disease.Gene therapy for neurologic manifestations of mucopolysaccharidoses.X-linked Angelman-like syndrome caused by Slc9a6 knockout in mice exhibits evidence of endosomal-lysosomal dysfunction.The designer aminoglycoside NB84 significantly reduces glycosaminoglycan accumulation associated with MPS I-H in the Idua-W392X mouse.Lysosomal accumulation of SCMAS (subunit c of mitochondrial ATP synthase) in neurons of the mouse model of mucopolysaccharidosis III B.Neurocognition across the spectrum of mucopolysaccharidosis type I: Age, severity, and treatment.Multimodal detection of GM2 and GM3 lipid species in the brain of mucopolysaccharidosis type II mouse by serial imaging mass spectrometry and immunohistochemistry.Storage solutions: treating lysosomal disorders of the brain.Cellular lipidomicsGangliosides as apoptotic signals in ER stress response.New developments in treating glycosphingolipid storage diseases.Genetic diseases of sphingolipid metabolism: pathological mechanisms and therapeutic options.The mucopolysaccharidoses: a success of molecular medicine.Substrate reduction therapy.Glycosphingolipid storage leads to the enhanced degradation of the B cell receptor in Sandhoff disease mice.Pathogenic cascades in lysosomal disease-Why so complex?Altered distribution and function of natural killer cells in murine and human Niemann-Pick disease type C1.Secondary alterations of sphingolipid metabolism in lysosomal storage diseases.Lysosomal storage disorders and Parkinson's disease: Gaucher disease and beyond.Epilepsy in children--when should we think neurometabolic disease?Mucopolysaccharidosis type I: current knowledge on its pathophysiological mechanisms.
P2860
Q21142752-62E28E73-0C6A-4938-9BB6-6C7BA3597E3AQ26773200-E5A47433-202D-4444-B88E-EA15AD706C29Q27301077-B48794D1-DF4C-4C1E-98A1-05A2AFC934F2Q28299661-BBC82B78-08E4-44A3-89D7-891E78E3CF72Q28472880-A23C28B8-BDC4-4067-A425-1A1D23E07CF1Q30499614-85A8FDF6-8434-4F67-A43E-A5E3B7EDCCFBQ30532642-AEB4222A-F64A-4C66-A63E-E8E97E72935DQ33692991-AFF2BD85-080C-4C7E-9630-DE530043815EQ33847821-796214E0-E7F6-4201-89AA-CF93A5742729Q33902037-F4480DAB-F1C5-450E-8786-4EF6C2C9CD00Q33966917-ACBDE7F6-B48C-49B2-85E2-E113B1BE1427Q34153746-79FFD3F2-CE66-4B2C-95CB-B130F912B109Q34256752-7170E535-EB7A-4662-9BF1-7D24B6B7CE6BQ34606079-055DFCA6-E974-45AE-B35F-414AB0FE3B55Q34685652-0E12B8E2-2BE7-4DCB-B732-531AA05EFF25Q35041986-F62A47DD-A72B-44B1-A2A9-BE3FD83210FDQ35067305-94F4B435-AF2B-4CBF-A49F-2E46E0F44FADQ35108104-2397B5B5-75EF-49A8-BF24-E9AF236E9517Q35167848-1AD89A9E-D5D8-407B-AF3E-072AE80A17D1Q35240940-9698C153-B512-48A2-B285-B8F2E37DA4BAQ35362251-B965B275-C199-4819-9232-C18FAC85DD6BQ35540083-7E423227-6B68-4C25-9498-65A2DF5E3A4CQ35661233-6207536B-3501-4CBC-A770-417210ADCC1EQ35842650-D7887625-74EF-42DB-8647-E35EFDF4DADCQ36032860-EAA312B9-862F-4D30-B418-B8B501DBE367Q36200634-58E17962-6D2F-43BA-B817-19ACB10549FFQ36210235-76937B87-E8B2-45EF-B20F-E77D9926A8D3Q36247057-3A365454-6FCE-4838-B12B-2BA0B57A337EQ36362402-27BBCAF5-2007-40E3-A02C-A538776EA017Q36363837-3436792A-5425-4D29-8CFC-6C5E086FD6F7Q36592822-196C96DF-4097-4B85-A67B-1354A3BA32FFQ37059193-AFDE12FC-FF5C-4B7C-9BD0-C937AA3AC0FDQ37109614-02739E94-A925-46DB-9CD5-255D9A280E16Q37189795-478D2F88-E39E-4CC2-9E80-9713E8D7730FQ37364596-A6F10D00-EBBB-4B92-A75C-852EF27AF706Q37429710-78BA1B29-4C4F-48BD-9946-4790C3CAE0FDQ37826106-B830216B-317A-4C91-A567-38BCD6B4EF17Q37880734-2DCBD30C-33A5-46BE-BB7D-74151FE67DE2Q37989420-CE24EF93-13A1-4086-A6AD-8AB697634054Q38004963-92DAC5A5-D663-4BBC-A464-D70CA2443DAB
P2860
Secondary accumulation of gangliosides in lysosomal storage disorders.
description
2004 nî lūn-bûn
@nan
2004年の論文
@ja
2004年論文
@yue
2004年論文
@zh-hant
2004年論文
@zh-hk
2004年論文
@zh-mo
2004年論文
@zh-tw
2004年论文
@wuu
2004年论文
@zh
2004年论文
@zh-cn
name
Secondary accumulation of gangliosides in lysosomal storage disorders.
@ast
Secondary accumulation of gangliosides in lysosomal storage disorders.
@en
type
label
Secondary accumulation of gangliosides in lysosomal storage disorders.
@ast
Secondary accumulation of gangliosides in lysosomal storage disorders.
@en
prefLabel
Secondary accumulation of gangliosides in lysosomal storage disorders.
@ast
Secondary accumulation of gangliosides in lysosomal storage disorders.
@en
P1476
Secondary accumulation of gangliosides in lysosomal storage disorders.
@en
P2093
Steven U Walkley
P304
P356
10.1016/J.SEMCDB.2004.03.002
P577
2004-08-01T00:00:00Z