citrullinemia/triglyceride

Der Link wird in der Zwischenablage gespeichert

ArtikelKlinische VersuchePatente

Wählen Sie den Sortiertyp

15 Ergebnisse

Medium-chain triglyceride supplementation under a low-carbohydrate formula is a promising therapy for adult-onset type II citrullinemia.

Nur registrierte Benutzer können Artikel übersetzen

Einloggen Anmelden

BACKGROUND Citrin, encoded by SLC25A13, is a component of the malate-aspartate shuttle, which is the main NADH-transporting system in the liver. Citrin deficiency causes neonatal intrahepatic cholestasis (NICCD), which usually resolves within the first year of life. However, small numbers of adults

Medium-chain triglycerides supplement therapy with a low-carbohydrate formula can supply energy and enhance ammonia detoxification in the hepatocytes of patients with adult-onset type II citrullinemia.

Nur registrierte Benutzer können Artikel übersetzen

Einloggen Anmelden

Citrin, encoded by SLC25A13, constitutes the malate-aspartate shuttle, the main NADH-shuttle in the liver. Citrin deficiency causes neonatal intrahepatic cholestasis (NICCD) and adult-onset type II citrullinemia (CTLN2). Citrin deficiency is predicted to impair hepatic glycolysis and de novo

Steatogenesis in adult-onset type II citrullinemia is associated with down-regulation of PPARα.

Nur registrierte Benutzer können Artikel übersetzen

Einloggen Anmelden

SLC25A13 (citrin or aspartate-glutamate carrier 2) is located in the mitochondrial membrane in the liver and its genetic deficiency causes adult-onset type II citrullinemia (CTLN2). CTLN2 is one of the urea cycle disorders characterized by sudden-onset hyperammonemia due to reduced argininosuccinate

Treatment with lactose (galactose)-restricted and medium-chain triglyceride-supplemented formula for neonatal intrahepatic cholestasis caused by citrin deficiency.

Nur registrierte Benutzer können Artikel übersetzen

Einloggen Anmelden

Citrin plays a role in the transfer of NADH-reducing equivalent from cytosol to mitochondria as part of the malate-aspartate shuttle in liver. Citrin deficiency may cause an impairment of glycolysis due to an increase in the cytosolic NADH/NAD ratio leading to an energy shortage in the liver.

[Liver transplantation in type II citrullinemia].

Nur registrierte Benutzer können Artikel übersetzen

Einloggen Anmelden

We reported a case of adult-onset citrullinemia associated with hypertrigliceridemia and diabetes mellitus. A 24-year-old female was healthy until recently. She first felt intermittent headaches and nausea. Then she noticed memory loss and tiredness. Abnormal behavior such as getting lost on the way

Impaired ketogenesis in patients with adult-type citrullinemia.

Nur registrierte Benutzer können Artikel übersetzen

Einloggen Anmelden

OBJECTIVE To clarify the mechanism causing fatty liver in adult-type citrullinemia, the effect of fasting on blood levels of free fatty acids, triglycerides, and ketone bodies was investigated in two cases. METHODS Blood and urine samples were collected from two patients and healthy volunteers 12,

Adult-onset type II citrullinemia: Current insights and therapy.

Nur registrierte Benutzer können Artikel übersetzen

Einloggen Anmelden

Citrin deficiency is a recessively inherited metabolic disorder with age-dependent clinical manifestations. It causes neonatal intrahepatic cholestasis (NICCD) and adult-onset type II citrullinemia (CTLN2). Patients with NICCD present with intrahepatic cholestasis in the neonatal period and usually

Diabetes mellitus exacerbates citrin deficiency via glucose toxicity.

Nur registrierte Benutzer können Artikel übersetzen

Einloggen Anmelden

Citrin is an aspartate/glutamate carrier that composes the malate-aspartate reduced nicotinamide adenine dinucleotide (NADH) shuttle in the liver. Citrin deficiency causes neonatal intrahepatic cholestasis (NICCD), failure to thrive and dyslipidemia (FTTDCD) and adult-onset type II

Cholesterol Metabolism Is Enhanced in the Liver and Brain of Children With Citrin Deficiency.

Nur registrierte Benutzer können Artikel übersetzen

Einloggen Anmelden

UNASSIGNED Citrin-deficient infants present neonatal intrahepatic cholestasis caused by citrin deficiency (NICCD), which resolves at 12 months. Thereafter, they have normal liver function associated with hypercholesterolemia, and a preference for lipid-rich carbohydrate-restricted diets. However,

Clinical heterogeneity of neonatal intrahepatic cholestasis caused by citrin deficiency: case reports from 16 patients.

Nur registrierte Benutzer können Artikel übersetzen

Einloggen Anmelden

A deficiency of citrin, which is encoded by the SLC25A13 gene, causes both adult-onset type II citrullinemia (CTLN2) and neonatal intrahepatic cholestasis (NICCD). We analyzed 16 patients with NICCD to clarify the clinical features of the disease. Severe intrahepatic cholestasis with fatty liver was

[Treatment and Pathomechanism of Citrin Deficiency].

Nur registrierte Benutzer können Artikel übersetzen

Einloggen Anmelden

Citrin, encoded by SLC25A13, is a component of the malate-aspartate shuttle, which is the main NADH-transporting system in the liver. Citrin deficiency causes neonatal intrahepatic cholestasis (NICCD), which usually resolves within the first year of life. However, a small number of adults with

Effects of supplementation on food intake, body weight and hepatic metabolites in the citrin/mitochondrial glycerol-3-phosphate dehydrogenase double-knockout mouse model of human citrin deficiency.

Nur registrierte Benutzer können Artikel übersetzen

Einloggen Anmelden

The C57BL/6:Slc23a13(-/-);Gpd2(-/-) double-knockout (a.k.a., citrin/mitochondrial glycerol 3-phosphate dehydrogenase double knockout or Ctrn/mGPD-KO) mouse displays phenotypic attributes of both neonatal intrahepatic cholestasis (NICCD) and adult-onset type II citrullinemia (CTLN2), making it a

[Failure to thrive and dyslipidemia caused by citrin deficiency: a novel clinical phenotype].

Nur registrierte Benutzer können Artikel übersetzen

Einloggen Anmelden

Two clinical phenotypes for citrin deficiency (CD) have been reported. One is adult-onset citrullinemia type II (CTLN2) and another is neonatal intrahepatic cholestasis caused by citrin deficiency (NICCD). A child with CD and who had failure to thrive (FTT) and dyslipidemia as main clinical

Current treatment for citrin deficiency during NICCD and adaptation/compensation stages: Strategy to prevent CTLN2.

Nur registrierte Benutzer können Artikel übersetzen

Einloggen Anmelden

Identification of the genes responsible for adult-onset type II citrullinemia (CTLN2) and citrin protein function have enhanced our understanding of citrin deficiency. Citrin deficiency is characterized by 1) neonatal intrahepatic cholestasis caused by citrin deficiency (NICCD); 2)

Metabolic basis and treatment of citrin deficiency

Nur registrierte Benutzer können Artikel übersetzen

Einloggen Anmelden

Citrin deficiency is a hereditary disorder caused by SLC25A13 mutations and manifests as neonatal intrahepatic cholestasis (NICCD), failure to thrive and dyslipidemia (FTTDCD), and adult-onset type II citrullinemia (CTLN2). Citrin is a component of the malate-aspartate nicotinamide adenine

Treten Sie unserer
Facebook-Seite bei

Herbal & Natural Medicine

Die vollständigste Datenbank für Heilkräuter, die von der Wissenschaft unterstützt wird

Arbeitet in 55 Sprachen
Von der Wissenschaft unterstützte Kräuterkuren
Kräutererkennung durch Bild
Interaktive GPS-Karte - Kräuter vor Ort markieren (in Kürze)
Lesen Sie wissenschaftliche Veröffentlichungen zu Ihrer Suche
Suchen Sie nach Heilkräutern nach ihrer Wirkung
Organisieren Sie Ihre Interessen und bleiben Sie über Neuigkeiten, klinische Studien und Patente auf dem Laufenden

Geben Sie ein Symptom oder eine Krankheit ein und lesen Sie über Kräuter, die helfen könnten, geben Sie ein Kraut ein und sehen Sie Krankheiten und Symptome, gegen die es angewendet wird.
* Alle Informationen basieren auf veröffentlichten wissenschaftlichen Forschungsergebnissen