Pathophysiology of neuropathic lysosomal storage disorders. - Wikidata - awgldk - TriplyDB

Pathophysiology of neuropathic lysosomal storage disorders.

Pathophysiology of neuropathic lysosomal storage disorders.

http://www.wikidata.org/entity/Q37739318

about

The cell biology of disease: lysosomal storage disorders: the cellular impact of lysosomal dysfunction Clarifying lysosomal storage diseases Lysosome and Cytoskeleton Pathways Are Robustly Enriched in the Blood of Septic Patients: A Meta-Analysis of Transcriptomic Data The lysosome: from waste bag to potential therapeutic target Autophagy and lysosomal dysfunction as emerging mechanisms of nanomaterial toxicity Iminosugar-based inhibitors of glucosylceramide synthase increase brain glycosphingolipids and survival in a mouse model of Sandhoff disease A canine Arylsulfatase G (ARSG) mutation leading to a sulfatase deficiency is associated with neuronal ceroid lipofuscinosis.Impaired prosaposin lysosomal trafficking in frontotemporal lobar degeneration due to progranulin mutations Factors influencing the measurement of lysosomal enzymes activity in human cerebrospinal fluid Development of targeted therapies for Parkinson's disease and related synucleinopathies Therapeutic approaches for lysosomal storage diseases Sensitivity to lysosome-dependent cell death is directly regulated by lysosomal cholesterol content Gene therapy for lysosomal storage disorders: a good start.Lysosomal protein turnover contributes to the acquisition of TGFβ-1 induced invasive properties of mammary cancer cells.Prosaposin facilitates sortilin-independent lysosomal trafficking of progranulin.Personalised medicine in paediatrics: individualising treatment in children with rare neurological diseases.The ubiquitin-proteasome system and the autophagic-lysosomal system in Alzheimer disease.Gemfibrozil and fenofibrate, Food and Drug Administration-approved lipid-lowering drugs, up-regulate tripeptidyl-peptidase 1 in brain cells via peroxisome proliferator-activated receptor α: implications for late infantile Batten disease therapy.Pathogenesis, Emerging therapeutic targets and Treatment in Sialidosis.Immunologic privilege in the central nervous system and the blood-brain barrier.Immune dysfunction in Niemann-Pick disease type C.Intrapleural administration of AAV9 improves neural and cardiorespiratory function in Pompe disease.A biological perspective of CSF lipids as surrogate markers for cognitive status in HIV Factors and processes modulating phenotypes in neuronopathic lysosomal storage diseases.Strategies for delivery of therapeutics into the central nervous system for treatment of lysosomal storage disorders.Sustained correction of motoneuron histopathology following intramuscular delivery of AAV in pompe mice.Amyloid-independent mechanisms in Alzheimer's disease pathogenesis Extracellular matrix components: an intricate network of possible biomarkers for lysosomal storage disorders?Blood-brain barrier structure and function and the challenges for CNS drug delivery.The respiratory neuromuscular system in Pompe disease.The dark sides of amyloid in Alzheimer's disease pathogenesis.The biological clock and the molecular basis of lysosomal storage diseases.Current molecular genetics strategies for the diagnosis of lysosomal storage disorders.Nanomedicine and nanotoxicology: the pros and cons for neurodegeneration and brain cancer.Induced pluripotent stem cell-based modeling of neurodegenerative diseases: a focus on autophagy.Insulin-Like Growth Factor-II/Cation-Independent Mannose 6-Phosphate Receptor in Neurodegenerative Diseases.Adeno-Associated Virus-Based Gene Therapy for CNS Diseases.Amyloid precursor protein and endosomal-lysosomal dysfunction in Alzheimer's disease: inseparable partners in a multifactorial disease.Mucopolysacccharidoses: From understanding to treatment, a century of discoveries.Neuropathology in respiratory-related motoneurons in young Pompe (Gaa(-/-)) mice.

P2860

Q24603159-0DDAB88A-3A40-4261-BBD3-AD7C91D9AADC Q24620532-C046E1C9-53A7-46A8-A42F-31F4F30D5562 Q26823225-EB78ED68-142E-4773-8E25-6B6305E144EF Q27022248-8B8085E0-1B46-4683-A0ED-BFBA0F1C3278 Q28388552-A4FD0814-9D11-4E85-8980-6610E7ACE4C6 Q28478879-0A463382-7FEF-4840-87DE-0D1445BE6C29 Q30496353-1A7D89B4-C412-4458-9849-C232DD9541AF Q33815765-46543EF1-154B-4745-901D-71DFF347E005 Q33831118-2389817D-C87D-483F-B05A-99B7675910F2 Q34238616-C2757560-19A2-47DD-B852-A28B607FCA08 Q34311420-92C972AB-F788-467A-A296-6D6CA63C77F3 Q34483105-51BCBE47-3BDA-4DC7-A14D-1A678BB96EC9 Q34502978-3B7A32EF-0653-4F4B-8FCD-5E4D9E9762D7 Q35116175-240BC594-2588-4322-9B5E-6288017007DC Q36078240-F86157EA-98F5-4C70-A85E-2B53DD246FD7 Q36117928-E665700A-58BC-44FC-9CAA-B833475CC358 Q36119393-9AA7F4BD-AED4-4C06-8B23-8753A3749447 Q36385912-65464358-3A52-4C19-9A76-51B8C54DB9A9 Q36649026-B34B4440-4F22-4C7E-B9BE-DF1CCA359861 Q36684162-715F8CC5-5D78-4D4C-A273-35462867B815 Q36802183-A84D7F4F-5A61-45BB-ACC8-0C5208B3D83E Q37181416-39BF6C9E-3B26-44B8-A984-823A3453563B Q37541514-BD2F0F08-FCB0-4A2A-869D-DE4890011B54 Q37595934-7B4F19EA-C528-4D36-B51D-FCFB9F559C57 Q37672410-AC1157FC-300C-49C7-9EB8-E279C8EBE7A4 Q37696025-D014BC7B-D124-4BFA-9EA1-BA79F8883D94 Q37808579-083A4331-CB90-453C-9000-6C9344D93F6D Q38085972-69E3DA78-014E-4BF9-B1F0-A36EBB4AC8B1 Q38100970-450862D5-470A-469C-9E2D-3131E08CAFCA Q38116744-AE89ACA8-DE57-4722-A03F-0A77B06DD6D0 Q38184783-EA1901CD-DD9E-47F7-9FFB-79F1409338F3 Q38316379-8B3BEE80-42E1-4280-9030-2FEF869FFAB4 Q38633334-37943325-1BC5-45A0-93E2-F58FD66D1AFB Q38665015-5F697D3F-6382-41DC-B9AD-80A9DB7652F8 Q38737027-D3C281E5-8C16-40C8-8DFB-13E14CBC25CA Q38780921-147B8D5F-1EDB-471D-8BE9-91C701520278 Q38856794-189C38C7-B98E-4A4F-9AE0-AB027ED84FA0 Q39405747-EE294F0D-5FC8-4381-BA3D-3C8930842B86 Q39466972-AC11520F-13E1-481D-90B1-6AE363EBD43A Q39963807-41EB5C5D-4121-4E27-AFFD-D67BEEF02C7A

P2860

Pathophysiology of neuropathic lysosomal storage disorders.

http://www.wikidata.org/entity/Q37739318

description

article científic

@ca

article scientifique

@fr

articolo scientifico

@it

artigo científico

@pt

bilimsel makale

@tr

scientific article published on 29 April 2010

@en

vedecký článok

@sk

vetenskaplig artikel

@sv

videnskabelig artikel

@da

vědecký článek

@cs

name

Pathophysiology of neuropathic lysosomal storage disorders.

@en

Pathophysiology of neuropathic lysosomal storage disorders.

@nl

type

label

Pathophysiology of neuropathic lysosomal storage disorders.

@en

Pathophysiology of neuropathic lysosomal storage disorders.

@nl

prefLabel

Pathophysiology of neuropathic lysosomal storage disorders.

@en

Pathophysiology of neuropathic lysosomal storage disorders.

@nl

P1433

Q37739318-420AF713-7B19-43A4-9E8F-82CEED1334FE

P1476

Q37739318-3B78E401-353A-4730-B2C1-F7DBAE328B21

P2093

Q37739318-E29461B5-9000-4D66-BB6C-8E43F7DC475F

Q37739318-E885B427-9AEC-4845-ACF9-E230C6530537

P2860

Q37739318-041490EE-605C-48A2-8071-13DE279D6771

Q37739318-135CEAED-AFE9-4FA4-A3D1-CF732AF3E8B3

Q37739318-2E8E5860-95B8-4955-A77E-7AFDD0A797C3

Q37739318-3687AB7C-0667-4AFC-89EF-2DE8EDF22F7B

Q37739318-3C8F043E-A3CC-47B6-A3F2-1C5C846BDEA3

Q37739318-573E4A82-A9F5-454C-8BB1-72A2F06BDB49

Q37739318-58AF970D-0A72-498A-BE0A-78D9B2A747D4

Q37739318-5BF5DE89-34A8-4C76-9367-BF2B6AB12606

Q37739318-CBEF9BAC-7976-44C6-8DD9-9486246B1138

Q37739318-E0A5393B-A356-4507-8A94-6F28E873EAE6

P2888

Q37739318-FE49A3DB-C12B-494A-995F-DABDACDA141E

P304

Q37739318-97C6F89A-4CF3-4628-B729-CB5FEAD793F9

P31

Q37739318-A982D5F6-45DA-46F6-A336-D554C233E392

P356

Q37739318-7AD180F4-5D1E-4F96-B2F3-4E964497387E

P433

Q37739318-5CC3AA0E-11A9-4C30-A583-C393899CF2B8

P478

Q37739318-A22101B7-DE00-4EF8-9FE8-A3A9C03DC633

P577

Q37739318-35FD45EC-71C2-4BDB-9B4E-06A748068DCC

P698

Q37739318-ACB9DFAA-3AF7-492F-9CA4-49268AD2FE5A

P921

Q37739318-0157B4D5-8A05-4453-B95E-3F2C85E9881A

P356

S10545-010-9075-9

P1433

Journal of Inherited Metabolic Disease

P1476

Pathophysiology of neuropathic lysosomal storage disorders.

@en

P2093

Cinzia Maria Bellettato

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

Maurizio Scarpa

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

P2860

Ryanodine receptor calcium release channels.

Selectivity and types of cell death in the neuronal ceroid lipofuscinoses.

Consequences of NPC1 and NPC2 loss of function in mammalian neurons.

Inability of tau to properly regulate neuronal microtubule dynamics: a loss-of-function mechanism by which tau might mediate neuronal cell death.

Progress towards understanding disease mechanisms in small vertebrate models of neuronal ceroid lipofuscinosis.

Autophagy in neurodegeneration and development

Blood to brain to the rescue.

Autophagy, mitochondria and cell death in lysosomal storage diseases.

Participation of autophagy in storage of lysosomes in neurons from mouse models of neuronal ceroid-lipofuscinoses (Batten disease).

Presenilin mutations in familial Alzheimer disease and transgenic mouse models accelerate neuronal lysosomal pathology.

P2888

s10545-010-9075-9

P304

347-362

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

P31

scholarly article

P356

10.1007/S10545-010-9075-9

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

P433

4

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

P478

33

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

P577

2010-04-29T00:00:00Z

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

P698

20429032

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

P921

pathophysiology