mTOR dysfunction contributes to vacuolar pathology and weakness in valosin-containing protein associated inclusion body myopathy. - Wikidata - awgldk - TriplyDB

mTOR dysfunction contributes to vacuolar pathology and weakness in valosin-containing protein associated inclusion body myopathy.

mTOR dysfunction contributes to vacuolar pathology and weakness in valosin-containing protein associated inclusion body myopathy.

http://www.wikidata.org/entity/Q36853801

about

Impaired Autophagy and Defective Mitochondrial Function: Converging Paths on the Road to Motor Neuron Degeneration Altered cofactor regulation with disease-associated p97/VCP mutations Lipin-1 regulates autophagy clearance and intersects with statin drug effects in skeletal muscle.A genetic screen identifies Tor as an interactor of VAPB in a Drosophila model of amyotrophic lateral sclerosis.Gene expression profiling in tibial muscular dystrophy reveals unfolded protein response and altered autophagy.Rapamycin and chloroquine: the in vitro and in vivo effects of autophagy-modifying drugs show promising results in valosin containing protein multisystem proteinopathy.IBMPFD Disease-Causing Mutant VCP/p97 Proteins Are Targets of Autophagic-Lysosomal Degradation.TDP-43 loss of function increases TFEB activity and blocks autophagosome-lysosome fusion.VCP and ATL1 regulate endoplasmic reticulum and protein synthesis for dendritic spine formation Glycolytic-to-oxidative fiber-type switch and mTOR signaling activation are early-onset features of SBMA muscle modified by high-fat diet Rapamycin increases survival in ALS mice lacking mature lymphocytes."Get the Balance Right": Pathological Significance of Autophagy Perturbation in Neuromuscular Disorders Modulation of mTOR signaling as a strategy for the treatment of Pompe disease.Monitoring autophagy in the treatment of protein aggregate diseases: steps toward identifying autophagic biomarkers.Autophagic cellular responses to physical exercise in skeletal muscle.ADSC therapy in neurodegenerative disorders.Autophagy in neuronal cells: general principles and physiological and pathological functions.Adipose tissue-derived stem cells in neural regenerative medicine.Dysfunction of autophagy as the pathological mechanism of motor neuron disease based on a patient-specific disease model.Ubiquitin- and ATP-dependent unfoldase activity of P97/VCP•NPLOC4•UFD1L is enhanced by a mutation that causes multisystem proteinopathy.Sporadic inclusion body myositis - a myodegenerative disease or an inflammatory myopathy.Increased autophagy accelerates colchicine-induced muscle toxicity.215th ENMC International Workshop VCP-related multi-system proteinopathy (IBMPFD) 13-15 November 2015, Heemskerk, The Netherlands.Is amyotrophic lateral sclerosis/frontotemporal dementia an autophagy disease?Dysregulated molecular pathways in amyotrophic lateral sclerosis-frontotemporal dementia spectrum disorder.

P2860

Q26766040-81B256D6-C93F-4B9B-A5A3-4D21D5C90CFF Q28258936-9F27F89E-99B0-4C3B-97B0-0D70038143D6 Q34221540-B1941962-A599-4F1E-8EF1-54B236E5A5FE Q34515862-743F249B-2CC1-467E-92D0-6CF439073FAB Q35117475-9446293A-1BAC-4234-9906-11C13F35059F Q35462835-5E50AA51-C2B0-4445-8C71-8B3491F5DB03 Q36170792-D24F78E0-C5F6-4583-8509-FBBFC8622C28 Q36478806-F22FD1CA-C2AE-46D8-8CA4-57C9479255ED Q36708176-A26171E6-2543-4014-B39B-FF1D7D011F2E Q37014362-D2DBB474-9936-40CE-9C72-4D46B36E6B28 Q37357775-60C12301-82D6-468A-9118-9EE71FDC4AAE Q37610585-C7CD22BF-6109-4093-9049-4C4C734B855F Q37672756-02FB769F-A52B-4630-8E83-E17CA815F939 Q38081388-0FC33034-51FF-474C-8DC6-A51BCD7177AD Q38189267-1436CB2F-7C3B-43A9-B722-697D108C2970 Q38210872-1B8D7840-A080-46E2-9AAA-ADB955C749DF Q38264793-CD1307DC-57EC-49A0-A7C5-1B6598DC6865 Q38342453-CDC021F4-7FFD-4E5A-A97A-B65463DB0394 Q38556198-7784A5DC-9BD7-4467-9A7F-238D6405C478 Q38682988-7A203071-4E60-4FD6-9B99-FB22BC38F948 Q38767200-4B6FBF69-A428-4720-9AFE-D541827A72CA Q43821019-3A8FF61C-100B-4057-B937-39D9443E420D Q46501146-A73C39CD-184C-434A-A9A9-F6D1FAD9776F Q47108927-9FCF218E-480F-45AE-8DAD-050807FC189B Q47946104-4C9FF918-BED5-4155-BC11-D569E11BAC94

P2860

mTOR dysfunction contributes to vacuolar pathology and weakness in valosin-containing protein associated inclusion body myopathy.

http://www.wikidata.org/entity/Q36853801

description

2012 nî lūn-bûn

@nan

2012年の論文

@ja

2012年論文

@yue

2012年論文

@zh-hant

2012年論文

@zh-hk

2012年論文

@zh-mo

2012年論文

@zh-tw

2012年论文

@wuu

2012年论文

@zh

2012年论文

@zh-cn

name

mTOR dysfunction contributes t ...... iated inclusion body myopathy.

@en

type

label

mTOR dysfunction contributes t ...... iated inclusion body myopathy.

@en

prefLabel

mTOR dysfunction contributes t ...... iated inclusion body myopathy.

@en

P1433

Q36853801-0A5440BF-F2A5-4690-9E5B-14081AC880BA

P1476

Q36853801-F5605EFF-BE65-46FA-BEAB-79CF37DEBD83

P2093

Q36853801-938015F1-D5C2-4079-9F8A-7991F33C1297

Q36853801-AEF96ED6-6B8F-4FAB-8F07-0A0D3C464CB4

Q36853801-BC932D5D-0FA7-4F87-9FB1-B1578E663CAE

Q36853801-DA17EB4F-60EB-48F1-A571-0CCE76AB31C9

Q36853801-F651B090-714F-48B8-8134-7DECC70BDE73

P2860

Q36853801-02103100-B5B3-4C4B-BF53-8D33535E72EC

Q36853801-061D1174-00CB-4ADE-99D5-E0E80213A603

Q36853801-0665C17E-7E6B-44CE-B913-02EFFD5CB600

Q36853801-0E19697A-C817-42DD-AC0A-5F198A6EDC71

Q36853801-0FC38FBA-8A6F-4B7A-9708-17BAAFD05704

Q36853801-207046B6-31A3-4B75-98E4-21E68570CF71

Q36853801-24CFFDB8-B810-401C-A467-B6E3BFB0D71D

Q36853801-298D4585-A3A7-4657-BCD4-EA15692733E3

Q36853801-2EFF1AD3-0942-4986-BD4E-7DB40363542F

Q36853801-327D96C7-773D-497D-A600-1C366D7C4012

P304

Q36853801-41AF115C-6152-44D6-B7B5-EC404F6B56D5

P31

Q36853801-B89EBD89-4A5D-4D33-A2A5-F3F0851BD93F

P356

Q36853801-B725C01D-DD08-4F1C-8EA4-0B7BB82FEAE6

P433

Q36853801-24F0D244-A660-4454-9778-90A15148319F

P478

Q36853801-5F018812-840B-4851-885A-F3A74BA474CC

P50

Q36853801-A07373B3-8DDC-4FFF-80CC-B6B597261089

P577

Q36853801-96014CE6-BE31-4C66-857E-E660A48F8A05

P698

Q36853801-1A9D6CF6-1972-4944-BCFC-B1904C696BFA

P932

Q36853801-6FEB046E-7A32-43D7-AD10-CF9B69762B3C

P356

P1433

Human Molecular Genetics

P1476

mTOR dysfunction contributes t ...... iated inclusion body myopathy.

@en

P2093

Caleb Lusk

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

Conrad C Weihl

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

James K Ching

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

Sara K Pittman

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

Sarita V Elizabeth

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

P2860

UBXD7 binds multiple ubiquitin ligases and implicates p97 in HIF1alpha turnover

The Rag GTPases bind raptor and mediate amino acid signaling to mTORC1

Exercise-induced BCL2-regulated autophagy is required for muscle glucose homeostasis

Exome sequencing reveals VCP mutations as a cause of familial ALS

ULK-Atg13-FIP200 complexes mediate mTOR signaling to the autophagy machinery

RETRACTED: VMA21 deficiency causes an autophagic myopathy by compromising V-ATPase activity and lysosomal acidification

VCP/p97 is essential for maturation of ubiquitin-containing autophagosomes and this function is impaired by mutations that cause IBMPFD

The p97 ATPase associates with EEA1 to regulate the size of early endosomes

mTOR drives its own activation via SCF(βTrCP)-dependent degradation of the mTOR inhibitor DEPTOR

DEPTOR, an mTOR inhibitor, is a physiological substrate of SCF(βTrCP) E3 ubiquitin ligase and regulates survival and autophagy

P304

1167-1179

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

P31

scholarly article

P356

10.1093/HMG/DDS524

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

P433

6

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

P478

22

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

P50

P577

2012-12-18T00:00:00Z

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

P698

23250913

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

P932

3657474

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