Neural deficits contribute to respiratory insufficiency in Pompe disease. - Wikidata - wikimedia - TriplyDB

Neural deficits contribute to respiratory insufficiency in Pompe disease.

Neural deficits contribute to respiratory insufficiency in Pompe disease.

http://www.wikidata.org/entity/Q37224150

about

Targeted approaches to induce immune tolerance for Pompe disease therapy New insights into therapeutic options for Pompe disease Practical Recommendations for Diagnosis and Management of Respiratory Muscle Weakness in Late-Onset Pompe Disease The pharmacological chaperone AT2220 increases the specific activity and lysosomal delivery of mutant acid alpha-glucosidase, and promotes glycogen reduction in a transgenic mouse model of Pompe disease Retrograde gene delivery to hypoglossal motoneurons using adeno-associated virus serotype 9.Gel-mediated delivery of AAV1 vectors corrects ventilatory function in Pompe mice with established disease Lactate produced by glycogenolysis in astrocytes regulates memory processing.The pharmacological chaperone AT2220 increases recombinant human acid α-glucosidase uptake and glycogen reduction in a mouse model of Pompe disease.Suppression of mTORC1 activation in acid-α-glucosidase-deficient cells and mice is ameliorated by leucine supplementation.Pax6 interactions with chromatin and identification of its novel direct target genes in lens and forebrain B-Cell Depletion is Protective Against Anti-AAV Capsid Immune Response: A Human Subject Case Study Peripheral nerve and neuromuscular junction pathology in Pompe disease Pompe disease gene therapy.Hypoglossal neuropathology and respiratory activity in pompe mice Altered activation of the tibialis anterior in individuals with Pompe disease: Implications for motor unit dysfunction Small-fiber neuropathy in pompe disease: first reported cases and prospective screening of a clinic cohort.A chaperone enhances blood α-glucosidase activity in Pompe disease patients treated with enzyme replacement therapy.Comparative impact of AAV and enzyme replacement therapy on respiratory and cardiac function in adult Pompe mice Spinal delivery of AAV vector restores enzyme activity and increases ventilation in Pompe mice.Correcting Neuromuscular Deficits With Gene Therapy in Pompe Disease In Vivo Selection Yields AAV-B1 Capsid for Central Nervous System and Muscle Gene Therapy Stimulation of Respiratory Motor Output and Ventilation in a Murine Model of Pompe Disease by Ampakines.Oral delivery of Acid Alpha Glucosidase epitopes expressed in plant chloroplasts suppresses antibody formation in treatment of Pompe mice.Gene therapy approaches for lysosomal storage disease: next-generation treatment.Evaluation of Readministration of a Recombinant Adeno-Associated Virus Vector Expressing Acid Alpha-Glucosidase in Pompe Disease: Preclinical to Clinical Planning.Pompe disease: Shared and unshared features of lysosomal storage disorders Copackaged AAV9 Vectors Promote Simultaneous Immune Tolerance and Phenotypic Correction of Pompe Disease.Power spectral analysis of hypoglossal nerve activity during intermittent hypoxia-induced long-term facilitation in mice.Diaphragm Pacing as a Rehabilitative Tool for Patients With Pompe Disease Who Are Ventilator-Dependent: Case Series.Altered activation of the diaphragm in late-onset Pompe disease.Diaphragm and ventilatory dysfunction during cancer cachexia.Phase I/II trial of adeno-associated virus-mediated alpha-glucosidase gene therapy to the diaphragm for chronic respiratory failure in Pompe disease: initial safety and ventilatory outcomes.Necrotizing myositis causes restrictive hypoventilation in a mouse model for human enterovirus 71 infection Intrapleural administration of AAV9 improves neural and cardiorespiratory function in Pompe disease.Adeno-associated virus-mediated gene therapy for metabolic myopathy.B-Cell depletion and immunomodulation before initiation of enzyme replacement therapy blocks the immune response to acid alpha-glucosidase in infantile-onset Pompe disease.Sustained correction of motoneuron histopathology following intramuscular delivery of AAV in pompe mice.Preclinical toxicology and biodistribution studies of recombinant adeno-associated virus 1 human acid α-glucosidase.Animal models for metabolic, neuromuscular and ophthalmological rare diseases.The respiratory neuromuscular system in Pompe disease.

P2860

Q26767363-B2D65384-9275-4F21-90D2-6D58F4F72099 Q26851649-57EAD331-F550-4C76-A1A5-241AA8995F83 Q28067387-32B1158E-9D14-49AC-AA8B-CF40DEB55D0D Q28540925-3C66B1F9-9042-4785-8D4D-AD01891A81D5 Q33586940-5010F142-E561-4C5F-8C35-72624821417A Q33731154-759BED5A-4710-4DAA-B46C-3F2E8F0979A5 Q34105200-18AA811A-2FC2-4D13-B4AB-4C699C34D9BA Q34345151-77FE6600-65AF-4A62-82F4-E2EF8F708076 Q34519309-CF843A83-17D0-4018-A37E-E4797C151DF6 Q34559489-742B9F70-8E58-486F-BD14-4269F4FF8F58 Q34763584-43ABEA8F-DA94-48A5-BC1D-7AD3870DF0C5 Q34920690-FA5BD5F7-E25F-47A9-8DD8-086EE3BDA09C Q34982843-EBF8BBC7-4EA5-4298-85C7-B0FBB9A0D68D Q35084926-98A329C8-78A1-49F8-B3BE-64AE87507013 Q35143360-8431CBC2-0908-4F70-BD0B-717778E75593 Q35296758-58D6B271-603B-493E-99E3-20DA935D5087 Q35600430-FADDB7CE-E2CF-4C20-91C6-221D1D092E9D Q35651103-E3325B5D-065B-4A20-8CD5-CF2EC2C1E94A Q35665212-915CE026-AAF5-4735-86A3-2DAE975F7FF8 Q35903220-53E810A8-E56C-4DFC-8BF6-49382212C996 Q35999976-61AE0B30-BB6B-4B05-A90A-C0626D99ECE3 Q36049311-5D0827EA-0306-48C1-A9B1-E5B8DBE4F345 Q36083396-C4B30696-40A8-4FA1-9B9C-40AEEE340ABA Q36147475-70352FB7-A0F2-426A-8807-B1C735C5BAAC Q36164927-1E054B89-90A9-419E-84ED-9296695A8789 Q36207744-A83FCCE3-996C-40F9-B5E2-380B7E953005 Q36543422-D391A18A-6A44-4B39-BD78-E20BD2404C77 Q36729720-430A1D6D-C930-4A13-9001-B6595405B99F Q36875150-ED6540D0-6FBE-48EF-8855-9740E274C518 Q36908177-C61E4F10-CED8-4A91-A716-FA9A02E52A32 Q36945130-8D82F3CC-4877-473D-9057-0ED0D0BE155F Q36945774-E8721C9D-62E3-41C3-B2A3-DEF6771E39CD Q37008702-9CCF6222-3FC3-4F0E-A753-996DAEDA96F6 Q37181416-68A33D50-B135-40BD-AFA6-08FF270CE424 Q37272261-3F520A9F-82AD-4F0D-B5AF-64FB5E3D17A6 Q37694653-0A0956EA-DD1C-4A95-A06B-62A1D646241C Q37696025-CBA582CE-D352-4D31-9C30-26E78D161BC5 Q37730125-C70A0CFB-26AE-4311-8F4A-B70B2993B138 Q38089518-9AC3B684-E37C-47E9-8D35-641AE9AF0D7F Q38116744-B194BA34-569D-413A-B3B6-BB86111A5416

P2860

Neural deficits contribute to respiratory insufficiency in Pompe disease.

http://www.wikidata.org/entity/Q37224150

description

article científic

@ca

article scientifique

@fr

articolo scientifico

@it

artigo científico

@pt

bilimsel makale

@tr

scientific article published on 27 May 2009

@en

vedecký článok

@sk

vetenskaplig artikel

@sv

videnskabelig artikel

@da

vědecký článek

@cs

name

Neural deficits contribute to respiratory insufficiency in Pompe disease.

@en

Neural deficits contribute to respiratory insufficiency in Pompe disease.

@nl

type

label

Neural deficits contribute to respiratory insufficiency in Pompe disease.

@en

Neural deficits contribute to respiratory insufficiency in Pompe disease.

@nl

prefLabel

Neural deficits contribute to respiratory insufficiency in Pompe disease.

@en

Neural deficits contribute to respiratory insufficiency in Pompe disease.

@nl

P1433

Q37224150-7286F787-011C-4535-9E99-1FE10670BDF4

P1476

Q37224150-3F124033-EA4E-496D-9408-DBD8A142131C

P2093

Q37224150-020183F5-746B-40CC-BB9B-7EE295795469

Q37224150-264A3B85-5A8C-4996-B2FE-7CC44F1CB897

Q37224150-8774217B-0758-4015-BD4B-8DA5CB914280

Q37224150-A090CEE2-D9CD-4F90-A6AC-A152CD31B14B

Q37224150-AD5843FE-DD4A-4661-9755-40F28FF3DE02

Q37224150-B3B81FFE-6E22-4D90-B9A3-54076AE4DBCD

Q37224150-B3BFDF8C-714A-4A0B-84D9-B50C64CB11F9

Q37224150-F88A6C6F-DA8E-49D5-A1CF-FDA30599E821

P2860

Q37224150-103735C4-56F0-4F12-8D0F-460B7D8E26D7

Q37224150-15606D39-972B-4446-8CF7-71C2F50E564B

Q37224150-1AF8ED18-535B-46F4-9F92-C01356DA9A76

Q37224150-1BD68B11-EAC2-4395-B35B-2F53B63E0BB2

Q37224150-20F9FB45-132B-4572-8EE5-59CED3DCE2F9

Q37224150-2308E371-254A-4A74-8E6D-699716DF9F0F

Q37224150-2D574B5D-EA9D-427C-B90B-1ACD454E51A2

Q37224150-36DE07E6-6B79-4C41-91AF-34BAA9A341A1

Q37224150-3A0E0467-B8B4-4943-80AB-E6709DEE2702

Q37224150-3CBFA046-EE2F-42B8-986D-DAAE2A2E6744

P304

Q37224150-A5B72E50-D302-4886-B256-F55D7E459A84

P31

Q37224150-9D88ED07-912C-42EC-92BA-F2402890FCD6

P356

Q37224150-CBACCD6B-10D6-4BED-9D42-34429F95F247

P407

Q37224150-A10C15E3-3948-40CC-A4FC-82A8C3A1E6AD

P433

Q37224150-16305B04-B57A-4247-B2DC-905994A9E7A5

P478

Q37224150-FF58EA07-C277-48AF-9BBB-AFA0C784BDB2

P577

Q37224150-E2A844E9-FA4F-436E-8798-007179D46944

P5875

Q37224150-01059414-C98B-40FB-8B93-394FF68ADA42

P698

Q37224150-857F4EF2-EF40-4B2A-92CD-6851E3C5F511

P932

Q37224150-09868BCC-BC1A-4DF5-AD83-6E1898B558B1

P356

PNAS.0902534106

P1433

Proceedings of the National Academy of Sciences of the United States of America

P1476

Neural deficits contribute to respiratory insufficiency in Pompe disease.

@en

P2093

Barry J Byrne

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

Cathryn Mah

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

David D Fuller

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

Kai Qiu

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

Keith C DeRuisseau

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

Lara R DeRuisseau

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

Paul J Reier

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

William H Donnelly

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

P2860

Clinical and metabolic correction of pompe disease by enzyme therapy in acid maltase-deficient quail

Studies of lysosomal alpha-glucosidase. I. Purification and properties of the rat liver enzyme

Hes1 and Hes5 as notch effectors in mammalian neuronal differentiation.

Conditional tissue-specific expression of the acid alpha-glucosidase (GAA) gene in the GAA knockout mice: implications for therapy

Targeted disruption of the acid alpha-glucosidase gene in mice causes an illness with critical features of both infantile and adult human glycogen storage disease type II

On the barometric method for measurements of ventilation, and its use in small animals.

Episodic but not continuous hypoxia elicits long-term facilitation of phrenic motor output in rats.

Development of obstruction to ventricular outflow and impairment of inflow in glycogen storage disease of the heart: serial echocardiographic studies from birth to death at 6 months.

Glycogen disease of skeletal muscle; report of two cases and review of literature.

Murine acid alpha-glucosidase: cell-specific mRNA differential expression during development and maturation

P304

9419-9424

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

P31

scholarly article

P356

10.1073/PNAS.0902534106

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

P407

P433

23

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

P478

106

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

P577

2009-05-27T00:00:00Z

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

P5875

26245653

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

P698

19474295

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

P932

2695054

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