Insulin-resistance in glycogen storage disease type Ia: linking carbohydrates and mitochondria?

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

(2017) Benjamin L. Farah et al.   Scientific Reports

Reduced bone mineral density in glycogen storage disease type III: evidence for a possible connection between metabolic imbalance and bone homeostasis

(2016) Daniela Melis et al.   BONE

“Classical organic acidurias”: diagnosis and pathogenesis

(2016) Guglielmo RD Villani et al.   CLINICAL AND EXPERIMENTAL MEDICINE

Liver and Muscle Contribute Differently to the Plasma Acylcarnitine Pool During Fasting and Exercise in Humans

(2016) G. Xu et al.   JOURNAL OF CLINICAL ENDOCRINOLOGY & METABOLISM

Transorgan fluxes in a porcine model reveal a central role for liver in acylcarnitine metabolism

(2015) Marieke G. Schooneman et al.   AMERICAN JOURNAL OF PHYSIOLOGY-ENDOCRINOLOGY AND METABOLISM

Acylcarnitines: potential implications for skeletal muscle insulin resistance

(2015) Céline Aguer et al.   FASEB JOURNAL

Lipids in hepatic glycogen storage diseases: pathophysiology, monitoring of dietary management and future directions

(2015) Terry G. J. Derks et al.   JOURNAL OF INHERITED METABOLIC DISEASE

Targeted metabolomics in the expanded newborn screening for inborn errors of metabolism

(2015) Emanuela Scolamiero et al.   Molecular BioSystems

Acylcarnitines—old actors auditioning for new roles in metabolic physiology

(2015) Colin S. McCoin et al.   Nature Reviews Endocrinology

A pilot longitudinal study of the use of waxy maize heat modified starch in the treatment of adults with glycogen storage disease type I: a randomized double-blind cross-over study

(2015) Kaustuv Bhattacharya et al.   Orphanet Journal of Rare Diseases

Glycogen storage disease type Ia (GSDIa) but not Glycogen storage disease type Ib (GSDIb) is associated to an increased risk of metabolic syndrome: possible role of microsomal glucose 6-phosphate accumulation

(2015) Daniela Melis et al.   Orphanet Journal of Rare Diseases

Molecular mechanisms of neutrophil dysfunction in glycogen storage disease type Ib

(2014) H. S. Jun et al.   BLOOD

Diagnosis and management of glycogen storage disease type I: a practice guideline of the American College of Medical Genetics and Genomics

(2014) Priya S. Kishnani et al.   GENETICS IN MEDICINE

Insulin-like growth factor 1 receptor signaling induced by supraphysiological doses of IGF-1 in human peripheral blood lymphocytes

(2014) Sara Spaziani et al.   PROTEOMICS

Visceral Adiposity Index: An Indicator of Adipose Tissue Dysfunction

(2014) Marco Calogero Amato et al.   International Journal of Endocrinology

Hepatic glucose sensing and integrative pathways in the liver

(2013) Maaike H. Oosterveer et al.   CELLULAR AND MOLECULAR LIFE SCIENCES

Glycogen storage disease type 1 and diabetes: Learning by comparing and contrasting the two disorders

(2013) F. Rajas et al.   DIABETES & METABOLISM

Dietary treatment of glycogen storage disease type Ia: Uncooked cornstarch and/or continuous nocturnal gastric drip-feeding?

(2013) Terry G.J. Derks et al.   MOLECULAR GENETICS AND METABOLISM

Elevated TCA cycle function in the pathology of diet-induced hepatic insulin resistance and fatty liver

(2012) Santhosh Satapati et al.   JOURNAL OF LIPID RESEARCH

Dietary dilemmas in the management of glycogen storage disease type I

(2011) Kaustuv Bhattacharya   JOURNAL OF INHERITED METABOLIC DISEASE

Correlation between measures of insulin resistance in fasting and non-fasting blood

(2011) Robert J Hancox et al.   Diabetology & Metabolic Syndrome

Glycogen Storage Disease Type 1: Impact of Medium-Chain Triglycerides on Metabolic Control and Growth

(2010) Anibh M. Das et al.   ANNALS OF NUTRITION AND METABOLISM

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

Mitochondrial Overload and Incomplete Fatty Acid Oxidation Contribute to Skeletal Muscle Insulin Resistance

(2008) Timothy R. Koves et al.   Cell Metabolism

Molecular and metabolic mechanisms of insulin resistance and β-cell failure in type 2 diabetes

(2008) Deborah M. Muoio et al.   NATURE REVIEWS MOLECULAR CELL BIOLOGY

Current approaches for assessing insulin sensitivity and resistance in vivo: advantages, limitations, and appropriate usage

(2007) Ranganath Muniyappa et al.   AMERICAN JOURNAL OF PHYSIOLOGY-ENDOCRINOLOGY AND METABOLISM