Kishore Alagere Krishnamurthy

Hepatocyte-specific Cas9-mediated editing of G6pc and Slc37a4 elicits comparable biochemical and regulatory responses between glycogen storage disease (GSD) type Ia and Ib mice

Kishore Alagere Krishnamurthy, Ruiqi Xiao, Martijn G. S. Rutten, Trijnie Bos et autres

BACKGROUND/OBJECTIVE: Glycogen storage disease type I (GSD I) is an autosomal recessive inborn error of carbohydrate metabolism. Patients with GSD type Ia and Ib exhibit overlapping and distinct symptoms and complications. Notably, GSD Ia patients show more severe hypertriglyceridemia and higher risk …

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Metabolic dysfunction-associated steatotic liver disease and steatohepatitis-associated hepatocarcinoma preclinical models

Jack Leslie, Kishore Alagere Krishnamurthy, Indresh K Gopalsamy, Patrícia Inácio et autres

Metabolic dysfunction-associated steatotic liver disease (MASLD) encompasses liver steatosis and metabolic dysfunction-associated steatohepatitis (MASH), which can result in fibrosis and/or cirrhosis and increase the risk of hepatocellular carcinoma (HCC). The latest Clinical Practice Guidelines acknowledge the importance of systemic metabolic dysfunction as …

gb, de, it (code pays fourni par la source)

Precision-cut liver slices as an ex vivo model to assess impaired hepatic glucose production

Ligia Akemi Kiyuna, Kishore Alagere Krishnamurthy, Esther Homan, Miriam Langelaar‐Makkinje et autres

Fasting hypoglycemia is a severe and incompletely understood symptom of various inborn errors of metabolism (IEM). Precision-cut liver slices (PCLS) represent a promising model for studying glucose production ex vivo. This study quantified the net glucose production of human and murine PCLS …

nl (code pays fourni par la source)

Exploring therapeutic targets for glycogen storage disease type 1 by modulating signal transduction pathways in hepatic in vivo and ex vivo models

Kishore Alagere Krishnamurthy

Hepatic ChREBP orchestrates intrahepatic carbohydrate metabolism to limit hepatic glucose 6-phosphate and glycogen accumulation in a mouse model for acute Glycogen Storage Disease type Ib

Kishore Alagere Krishnamurthy, Martijn G. S. Rutten, Joanne A. Hoogerland, Theo H. van Dijk et autres

OBJECTIVE: Carbohydrate Response Element Binding Protein (ChREBP) is a glucose 6-phosphate (G6P)-sensitive transcription factor that acts as a metabolic switch to maintain intracellular glucose and phosphate homeostasis. Hepatic ChREBP is well-known for its regulatory role in glycolysis, the pentose phosphate pathway, and …

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Precision-cut liver slices as an ex vivo model to assess impaired hepatic glucose production

Barbara M. Bakker, Ligia Akemi Kiyuna, Kishore Alagere Krishnamurthy, Miriam Langelaar‐Makkinje et autres

nl (code pays fourni par la source)

Normalization of hepatic ChREBP activity does not protect against liver disease progression in a mouse model for Glycogen Storage Disease type Ia

Martijn G. S. Rutten, Lei Yu, Joanne A. Hoogerland, Vincent W. Bloks et autres

BACKGROUND: Glycogen storage disease type 1a (GSD Ia) is an inborn error of metabolism caused by a defect in glucose-6-phosphatase (G6PC1) activity, which induces severe hepatomegaly and increases the risk for liver cancer. Hepatic GSD Ia is characterized by constitutive activation of …

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Normalization of hepatic ChREBP activity does not protect against liver disease progression in a mouse model for Glycogen Storage Disease type Ia

Martijn G. S. Rutten, Lei Yu, Joanne A. Hoogerland, Vincent W. Bloks et autres

Abstract Background Glycogen storage disease type 1a (GSD Ia) is an inborn error of metabolism caused by a defect in glucose-6-phosphatase (G6PC1) activity, which induces severe hepatomegaly and increases the risk for liver cancer. Hepatic GSD Ia is characterized by constitutive activation …

nl, se, fr, Mali (code pays fourni par la source)

Precision-cut liver slices as an ex vivo model to assess impaired hepatic glucose production

Kishore Alagere Krishnamurthy, Ligia Akemi Kiyuna, Miriam Langelaar, Albert Gerding et autres

Increased atherosclerosis in a mouse model of glycogen storage disease type 1a

Anouk M. La Rose, Anouk G. Groenen, Benedek Halmos, Venetia Bazioti et autres

Glycogen storage disease type 1a (GSD Ia) is an inborn error of carbohydrate metabolism. Despite severe hyperlipidemia, GSD Ia patients show limited atherogenesis compared to age-and-gender matched controls. Employing a GSD Ia mouse model that resembles the severe hyperlipidemia in patients, we …

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