Publications by authors named "Jihane Laafi"
Article Synopsis
- Acute intermittent porphyria (AIP) is a genetic disorder caused by a deficiency of the enzyme hydroxymethylbilane synthase (HMBS), affecting heme production and primarily impacting the nervous system.
- Research on Hmbs(-/-) mice revealed alterations in mitochondrial oxidative phosphorylation (OXPHOS), showing an initial adaptive response to HMBS deficiency, followed by significant changes due to phenobarbital treatment.
- Treatment with heme arginate (HA) mitigated the reduction in ATP production in skeletal muscle, indicating that both neurotoxicity from certain compounds and mitochondrial dysfunction are vital in understanding AIP's effects.
Heme biosynthesis begins in the mitochondrion with the formation of delta-aminolevulinic acid (ALA). In acute intermittent porphyria, hereditary tyrosinemia type I and lead poisoning patients, ALA is accumulated in plasma and in organs, especially the liver. These diseases are also associated with neuromuscular dysfunction and increased incidence of hepatocellular carcinoma.
View Article and Find Full Text PDFArticle Synopsis
- Acute intermittent porphyria (AIP) is a genetic liver disorder caused by a deficiency in the HMBS enzyme, leading to dangerous attacks due to high production of 5-aminolaevulinic acid (ALA).
- A study using Hmbs knockout mice treated with phenobarbital showed significant impairments in mitochondrial respiratory chain and TCA cycle activities.
- Treatment with heme arginate after phenobarbital helped restore some metabolic function, highlighting potential therapeutic strategies to mitigate the effects of AIP on mitochondrial energy metabolism.
Background: We compared three hyaluronic acid (HA) assays and analyzed the impact of their variations on FibroMeter scores.
Methods: In a test group of 165 patients, HA levels were assessed with the commonly used ELISA assay from Corgenix, a new ELISA assay from Teco and an immunoturbidimetry assay from Wako, this latter tested across three different instruments. Five different FibroMeter scores were calculated.
Background/aims: To compare blood tests of liver fibrosis specific for NAFLD: the FibroMeter NAFLD and the NAFLD fibrosis score (NFSA) with a non-specific test, APRI.
Methods: Two hundred and thirty-five NAFLD patients with liver Metavir staging and blood markers from two independent centres were randomly assigned to a test (n=121) or a validation population (n=114).
Results: The highest accuracy--91%--for significant fibrosis was obtained with the FibroMeter whose (i) AUROC (0.