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内部出版物
Wiley电子期刊(1)
SpringerLink电子期刊(4)
Elsevier电子期刊(25)
在“
Elsevier电子期刊
”中,
命中:
25
条,耗时:小于0.01 秒
在所有数据库中总计命中:
30
条
1.
Induction of autophagy improves hepatic lipid metabolism in glucose-6-phosphatase deficiency
作者:
Benjamin L. Farah
1
;
Dustin J. Landau
2
;
Rohit A. Sinha
1
;
Elizabeth D. Brooks
2
;
3
;
Yajun Wu
4
;
Suet Yin Sarah Fung
5
;
Tomohiro Tanaka
6
;
Masahiro Hirayama
6
;
Boon-Huat Bay
4
;
Dwight
D.
Koeberl
2
;
7
;
dwight
.
koeberl
@duke.edu" class="auth_mail" title="E-mail the corresponding author
;
Paul M. Yen
1
;
8
;
paul.yen@duke-nus.edu.sg" class="auth_mail" title="E-mail the corresponding author
关键词:
G6Pase α
;
glucose-6-phosphatase α
;
G6PC
;
glucose-6-phosphatase α catalytic subunit
;
GSDIa
;
glycogen storage disease type Ia
;
G6P
;
glucose-6-phosphate
;
mTOR
;
mammalian target of rapamycin
;
AMPK
;
5&rsquo
;
AMP-activated protein kinase
;
ULK1
;
Unc51-like kinase 1
;
NAFLD
;
non-alcoholic fatty liver disease
;
GSDII
;
glycogen storage disease type II
;
LC3
;
microtubule-associated protein 1A/1B-light chain 3
;
C-C3
;
cleaved caspase 3
;
Rap
;
rapamycin
;
AAV
;
adeno-associated virus
;
GFP
;
green fluorescent protein
刊名:Journal of Hepatology
出版年:2016
2.
Salmeterol enhances the cardiac response to gene therapy in Pompe disease
作者:
Sang-oh Han
;
Songtao Li
;
Dwight
D.
Koeberl
关键词:
Pompe disease
;
Glycogen storage disease
;
Adeno-associated virus vector
;
Mannose-6-phosphate receptor
;
Enzyme replacement therapy
刊名:Molecular Genetics and Metabolism
出版年:2016
3.
Pilot study of adjunctive albuterol in late-onset Pompe disease reveals safety and efficacy from upregulated Mannose-6-phosphate receptor expression
作者:
Dwight
Koeberl
;
Stephanie Austin
;
Laura Case
;
Edward C. Smith
;
Anne Buckley
;
Sarah P. Young
;
Deeksha Bali
;
Priya S. Kishnani
刊名:Molecular Genetics and Metabolism
出版年:February, 2014
4.
The upstream enhancer elements of the G6PC promoter are critical for optimal G6PC expression in murine glycogen storage disease type Ia
作者:
Young Mok Lee
;
Chi-Jiunn Pan
;
Dwight
D.
Koeberl
;
Brian C. Mansfield
;
Janice Y. Chou
关键词:
GSD-Ia
;
glycogen storage disease type Ia
;
G6Pase
;
glucose-6-phosphatase
;
G6P
;
glucose-6-phosphate
;
HCA
;
hepatocellular adenoma
;
AAV
;
adeno-associated virus
;
GPE
;
G6PC promoter/enhancer
刊名:Molecular Genetics and Metabolism
出版年:2013
5.
Adjunctive 2-agonist therapy enhanced biochemical correction and biomarker reduction from an AAV vector expressing acid alpha-glucosidase in the skeletal muscle of mice with Pompe disease
作者:
Dwight
Koeberl
;
Songtao Li
;
Andrew Bird
;
Kristen Gazda
;
Haoyue Zhang
;
Sarah Young
刊名:Molecular Genetics and Metabolism
出版年:2013
6.
Metabolic Myopathies: Clinical Features and Diagnostic Approach
作者:
Edward C. Smith
;
MD
a
;
Areeg El-Gharbawy
;
MD
b
;
Dwight
D.
Koeberl
;
MD
;
PhD
b
;
dwight
.
koeberl
@duke.edu
关键词:
Inherited disorders of metabolism
;
Metabolic myopathy
;
Glycogen storage disease
;
Mitochondrial disorder
;
Fatty acid oxidation
刊名:Rheumatic Disease Clinics of North America
出版年:2011
7.
Cross-reactive immunologic material status affects treatment outcomes in Pompe disease infants
作者:
Priya S. Kishnani
;
Paula C. Goldenberg
;
Stephanie L. DeArmey
;
James Heller
;
Danny Benjamin
;
Sarah Young
;
Deeksha Bali
;
Sue Ann Smith
;
Jennifer S. Li
;
Hanna M
;
el
;
Dwight
Koeberl
;
Amy Rosenberg
;
Y-T Che
关键词:
Pompe disease
;
Glycogen storage disease
;
Enzyme replacement therapy
;
Lysosomal storage disease
;
Acid alpha glucosidase
;
GAA
;
Cross-reactive immunologic material
;
CRIM
;
Antibody
刊名:Molecular Genetics and Metabolism
出版年:2010
8.
Liver transplantation for glycogen storage disease type Ia
作者:
Srinevas K. Reddy
;
Stephanie L. Austin
;
Michele Spencer-Manzon
;
Dwight
D.
Koeberl
;
Bryan M. Clary
;
Dev M. Desai
;
Alastair D. Smith
;
Priya S. Kishnani
关键词:
Glycogen storage disease Ia
;
Liver transplantation
;
Hepatocellular adenoma
;
Hepatocellular carcinoma
刊名:Journal of Hepatology
出版年:2009
9.
Activation of glycolysis and apoptosis in glycogen storage disease type Ia
作者:
Baodong Sun
;
Songtao Li
;
Liu Yang
;
Tirupapuliyur Damodaran
;
Dev Desai
;
Anna Mae Diehl
;
Oscar Alzate
;
Dwight
D.
Koeberl
关键词:
Glucose-6-phosphatase
;
von Gierke disease
;
Inherited disorder of metabolism
;
Glyceraldehyde-3-phosphate dehydrogenase
;
Apoptosis
;
Glycogen storage disease
;
Fatty liver
;
Gene therapy
刊名:Molecular Genetics and Metabolism
出版年:2009
10.
74. Immunological aspects of treatment of Pompe disease
作者:
Priya Kishnani
;
Suhrad Banugaria
;
Stephanie DeArmey
;
Joanne Mackey
;
Sarah Young
;
Deeksha Bali
;
Dwight
Koeberl
;
Amy Rosenberg
;
Yoav Messinger
;
Nancy Mendelsohn
;
William Rhead
;
Y.T. Chen
刊名:Molecular Genetics and Metabolism
出版年:2010
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