Hepatic mitochondrial dysfunction is a feature of Glycogen Storage Disease Type Ia (GSDIa)

Thyroid Hormone Stimulation of Autophagy Is Essential for Mitochondrial Biogenesis and Activity in Skeletal Muscle

(2016) Ronny Lesmana et al.   ENDOCRINOLOGY

Oxidative stress, mitochondrial dysfunction and neurodegenerative diseases; a mechanistic insight

(2015) Aashiq Hussain Bhat et al.   BIOMEDICINE & PHARMACOTHERAPY

Maintaining Ancient Organelles: Mitochondrial Biogenesis and Maturation

(2015) R. B. Vega et al.   CIRCULATION RESEARCH

Mitochondrial biology, targets, and drug delivery

(2015) Lara Milane et al.   JOURNAL OF CONTROLLED RELEASE

Therapeutic Strategies for Mitochondrial Disorders

(2015) Josef Finsterer et al.   PEDIATRIC NEUROLOGY

Mutations causing mitochondrial disease: What is new and what challenges remain?

(2015) R. N. Lightowlers et al.   SCIENCE

The mitochondrial O-linked N-acetylglucosamine transferase (mOGT) in the diabetic patient could be the initial trigger to develop Alzheimer disease

(2014) Liliana Lozano et al.   EXPERIMENTAL GERONTOLOGY

Long-Term Efficacy Following Readministration of an Adeno-Associated Virus Vector in Dogs with Glycogen Storage Disease Type Ia

(2011) Amanda Demaster et al.   HUMAN GENE THERAPY

SIRT3 Deficiency and Mitochondrial Protein Hyperacetylation Accelerate the Development of the Metabolic Syndrome

(2011) Matthew D. Hirschey et al.   MOLECULAR CELL

Glucose-6-phosphatase deficiency

(2011) Roseline Froissart et al.   Orphanet Journal of Rare Diseases

Carbohydrate-response-element-binding protein (ChREBP) and not the liver X receptor α (LXRα) mediates elevated hepatic lipogenic gene expression in a mouse model of glycogen storage disease type 1

(2010) Aldo Grefhorst et al.   BIOCHEMICAL JOURNAL

Lack of glucose recycling between endoplasmic reticulum and cytoplasm underlies cellular dysfunction in glucose-6-phosphatase- -deficient neutrophils in a congenital neutropenia syndrome

(2010) H. S. Jun et al.   BLOOD

Glycogen storage disease type I and G6Pase-β deficiency: etiology and therapy

(2010) Janice Y. Chou et al.   Nature Reviews Endocrinology

Phosphorylation of ULK1 (hATG1) by AMP-Activated Protein Kinase Connects Energy Sensing to Mitophagy

(2010) D. F. Egan et al.   SCIENCE

Liver transplantation for glycogen storage disease type Ia

(2009) Srinevas K. Reddy et al.   JOURNAL OF HEPATOLOGY

Hepatic anaplerotic outflow fluxes are redirected from gluconeogenesis to lactate synthesis in patients with Type 1a glycogen storage disease

(2009) John G. Jones et al.   METABOLIC ENGINEERING

Activation of glycolysis and apoptosis in glycogen storage disease type Ia

(2009) Baodong Sun et al.   MOLECULAR GENETICS AND METABOLISM

The lysosomal-mitochondrial axis in free fatty acid-induced hepatic lipotoxicity

(2008) ZhengZheng Li et al.   HEPATOLOGY

Increased de novo Lipogenesis and Delayed Conversion of Large VLDL into Intermediate Density Lipoprotein Particles Contribute to Hyperlipidemia in Glycogen Storage Disease Type 1a

(2008) Robert H J Bandsma et al.   PEDIATRIC RESEARCH

Nonalcoholic fatty liver disease and mitochondrial dysfunction

(2008) Yongzhong Wei et al.   WORLD JOURNAL OF GASTROENTEROLOGY

Angiotensin mediates renal fibrosis in the nephropathy of glycogen storage disease type Ia

(2007) W.H. Yiu et al.   KIDNEY INTERNATIONAL