Restoration of muscle functionality by genetic suppression of glycogen synthesis in a murine model of Pompe disease. - sprookjes - yvandenbroek - TriplyDB

Restoration of muscle functionality by genetic suppression of glycogen synthesis in a murine model of Pompe disease.

Restoration of muscle functionality by genetic suppression of glycogen synthesis in a murine model of Pompe disease.

http://www.wikidata.org/entity/Q43230362

about

New insights into therapeutic options for Pompe disease PTG depletion removes Lafora bodies and rescues the fatal epilepsy of Lafora disease Dysregulation of multiple facets of glycogen metabolism in a murine model of Pompe disease Pompe disease: from pathophysiology to therapy and back again Progress and problems in muscle glycogenoses Antisense Oligonucleotide-mediated Suppression of Muscle Glycogen Synthase 1 Synthesis as an Approach for Substrate Reduction Therapy of Pompe Disease Suppression of autophagy permits successful enzyme replacement therapy in a lysosomal storage disorder--murine Pompe disease.Hypertransaminasemia and fatal lung disease: a case report."Get the Balance Right": Pathological Significance of Autophagy Perturbation in Neuromuscular Disorders Pharmaceutical Chaperones and Proteostasis Regulators in the Therapy of Lysosomal Storage Disorders: Current Perspective and Future Promises.Using Gene Essentiality and Synthetic Lethality Information to Correct Yeast and CHO Cell Genome-Scale Models.13C/31P MRS Metabolic Biomarkers of Disease Progression and Response to AAV Delivery of hGAA in a Mouse Model of Pompe Disease Polyglucosan neurotoxicity caused by glycogen branching enzyme deficiency can be reversed by inhibition of glycogen synthase.

P2860

Q26851649-A1297606-FA55-4AE1-A39A-BC37B822F263 Q28477920-4D722532-7454-4F88-9AEE-6CC676855684 Q28486167-D8D6F476-241C-4193-8B70-399DC40F01A6 Q34054935-712078C1-F33C-40DF-892F-ED375C79E967 Q34235074-15D31329-2245-444C-A33F-693C113D2723 Q34440333-FD7905AB-6FED-4B94-8367-A99846457BF4 Q34577206-EEAD9A82-28D2-4021-9965-C01E7E7393FC Q36686820-85D6B764-D40A-4CB1-849C-28BA3AA8D209 Q37610585-A99316B1-1438-4DC9-8DAB-F5590BB0E2B7 Q38652950-1CE6A8F7-135E-4585-B8AB-7C04570653C3 Q40478667-E67EF93E-648E-4D71-A816-646393C59722 Q41919490-2CC7B2A3-BEA0-4F8B-AF49-16872BB5C095 Q51052738-22A1A75E-C8EC-4A2E-81D8-AABB80340A63

P2860

Restoration of muscle functionality by genetic suppression of glycogen synthesis in a murine model of Pompe disease.

http://www.wikidata.org/entity/Q43230362

description

2009 nî lūn-bûn

@nan

2009年の論文

@ja

2009年学术文章

@wuu

2009年学术文章

@zh

2009年学术文章

@zh-cn

2009年学术文章

@zh-hans

2009年学术文章

@zh-my

2009年学术文章

@zh-sg

2009年學術文章

@yue

2009年學術文章

@zh-hant

name

Restoration of muscle function ...... murine model of Pompe disease.

@en

Restoration of muscle function ...... murine model of Pompe disease.

@nl

type

label

Restoration of muscle function ...... murine model of Pompe disease.

@en

Restoration of muscle function ...... murine model of Pompe disease.

@nl

prefLabel

Restoration of muscle function ...... murine model of Pompe disease.

@en

Restoration of muscle function ...... murine model of Pompe disease.

@nl

P1433

Q43230362-E17F0976-6A96-40FE-8B5B-E55A763B7C21

P1476

Q43230362-4AA28F21-9376-490D-9B76-F4AEE8496E6F

P2093

Q43230362-3739EC17-8331-42DE-8F4A-7E230010279F

Q43230362-3856467C-129C-40AC-9777-A9ADC809898B

Q43230362-510B3BF9-C132-4635-86E8-5D3EF9DD34F1

Q43230362-5CB5E336-7EA5-4789-BB4B-FD1633946ABD

Q43230362-63EC156B-B6B7-4E5F-8ADC-72656D8D3384

Q43230362-6F08A414-4004-4791-8917-8437EA21F805

Q43230362-9B635EAE-B6C5-432E-94BE-AA99DAC58A4B

Q43230362-B8273353-4651-4405-A89E-C1A20447AE59

Q43230362-BA19FF9C-124E-4FA1-94B7-55D30FF10638

Q43230362-BA4FEBEE-FDB1-4DF7-B15C-100E62AA3882

P2860

Q43230362-076FD6CE-AD18-4000-819E-A9CB9F12A3B5

Q43230362-0945B843-735A-4190-BE8B-C966301C4DA4

Q43230362-18C890C0-2C22-430F-ABE4-D92C6872A4BD

Q43230362-1F645E4E-0520-43BF-8D84-2568B73B99EF

Q43230362-2812D379-E870-4C9D-BE06-852C8F6777FB

Q43230362-2E4ECDB7-D3C9-4CB1-89F0-653958C0BA1D

Q43230362-32D2C467-851A-449F-9C04-98882F3CCBE4

Q43230362-33A1D4BA-932A-4F97-956D-7FA0C50542A0

Q43230362-37FD8CAC-813C-4513-9161-BE9F33EB39AD

Q43230362-45C0E413-2BED-474D-A2B9-5313B92A5299

P304

Q43230362-14383533-12FF-4EC4-8355-F8CA8C440DFB

P31

Q43230362-FB363A2D-C3A5-4EF1-A5E4-9B8C9148AE87

P356

Q43230362-7236B980-AF44-4C64-ABD1-B01100A40567

P433

Q43230362-8B5D25EF-C1AC-407E-ABB9-CB8D612D28C7

P478

Q43230362-457518B0-5AB5-4EED-A793-6588B13FFEEC

P577

Q43230362-C4AE1D02-20C0-48F8-9BDD-90327A3B086E

P5875

Q43230362-D840C8EE-3F8E-45A0-BAB0-C0AB3B76BEC7

P698

Q43230362-629E7F76-BC05-4AC5-AE53-559ED989BAF1

P932

Q43230362-45A6E0BB-A3AB-4EB3-87A0-D35FCE7792DA

P356

P1433

Human Molecular Genetics

P1476

Restoration of muscle function ...... murine model of Pompe disease

@en

P2093

Alban Vignaud

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

Arnaud Ferry

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

Beth L Thurberg

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

Catherine Caillaud

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

Emmanuel Richard

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

Gaelle Douillard-Guilloux

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

Isabelle Guillet-Deniau

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

Maryline Favier

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

Nina Raben

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

Peter J Roach

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

P2860

Identification of a novel mutation in GYS1 (muscle-specific glycogen synthase) resulting in sudden cardiac death, that is diagnosable from skin fibroblasts

Cardiomyopathy and exercise intolerance in muscle glycogen storage disease 0

Glucose toxicity and the development of diabetes in mice with muscle-specific inactivation of GLUT4

Generalized glycogen storage and cardiomegaly in a knockout mouse model of Pompe disease

Glycogen stored in skeletal but not in cardiac muscle in acid alpha-glucosidase mutant (Pompe) mice is highly resistant to transgene-encoded human enzyme

Abnormal cardiac development in the absence of heart glycogen

Increased glycogen accumulation in transgenic mice overexpressing glycogen synthase in skeletal muscle

Role of carbohydrate in physical activity.

Direct observation of glycogen synthesis in human muscle with 13C NMR

Enzyme therapy for pompe disease with recombinant human alpha-glucosidase from rabbit milk.

P304

684-696

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

P31

scholarly article

P356

10.1093/HMG/DDP535

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

P433

4

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

P478

19

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

P577

2009-12-03T00:00:00Z

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

P5875

40446526

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

P698

19959526

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

P932

6281383

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