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Oligodendrocyte Slc48a1 (Hrg1) encodes a functional heme transporter required for myelin integrity.
Glia
February 2025
Wellcome-MRC Cambridge Stem Cell Institute, University of Cambridge, Cambridge, UK.
Article Synopsis
- * Hrg1 is specifically expressed in mature oligodendrocytes and is co-localized with myelin sheaths, suggesting its significant role in myelination.
- * Hrg1 null mutant mice displayed myelin defects and lower levels of myelin iron, indicating that Hrg1 is essential for maintaining myelin integrity and proper cell differentiation.
Myelinated axons are the primary target of hemin-mediated oxidative damage in a model of the central nervous system.
Exp Neurol
August 2022
Institute of Infection, Immunity and Inflammation, University of Glasgow, G12 8TA Glasgow, United Kingdom. Electronic address:
Iron released from oligodendrocytes during demyelination or derived from haemoglobin breakdown products is believed to amplify oxidative tissue injury in multiple sclerosis (MS). However, the pathophysiological significance of iron-containing haemoglobin breakdown products themselves is rarely considered in the context of MS and their cellular specificity and mode of action remain unclear. Using myelinating cell cultures, we now report the cytotoxic potential of hemin (ferriprotoporphyrin IX chloride), a major degradation product of haemoglobin, is 25-fold greater than equimolar concentrations of free iron in myelinating cultures; a model that reproduces the complex multicellular environment of the CNS.
View Article and Find Full Text PDFHomozygous hydroxymethylbilane synthase knock-in mice provide pathogenic insights into the severe neurological impairments present in human homozygous dominant acute intermittent porphyria.
Hum Mol Genet
June 2019
Department of Genetics and Genomic Sciences, Icahn School of Medicine at Mount Sinai, New York, NY, USA.
Acute intermittent porphyria (AIP) is an inborn error of heme biosynthesis due to the deficiency of hydroxymethylbilane synthase (HMBS) activity. Human AIP heterozygotes have episodic acute neurovisceral attacks that typically start after puberty, whereas patients with homozygous dominant AIP (HD-AIP) have early-onset chronic neurological impairment, including ataxia and psychomotor retardation. To investigate the dramatically different manifestations, knock-in mice with human HD-AIP missense mutations c.
View Article and Find Full Text PDFIdentification of progesterone receptor membrane component-1 as an interaction partner and possible regulator of fatty acid 2-hydroxylase.
Biochem J
March 2018
Institute of Biochemistry and Molecular Biology, University of Bonn, Nussallee 11, 53115 Bonn, Germany
The fatty acid 2-hydroxylase (FA2H) is essential for synthesis of 2-hydroxylated fatty acids in myelinating and other cells, and deficiency of this enzyme causes a complicated form of hereditary spastic paraplegia also known as fatty acid hydroxylase-associated neurodegeneration. Despite its important role in sphingolipid metabolism, regulation of FA2H and its interaction with other proteins involved in the same or other metabolic pathways is poorly understood. To identify potential interaction partners of the enzyme, quantitative mass spectrometry using stable isotope labeling of cells was combined with formaldehyde cross-linking and proximity biotinylation, respectively.
View Article and Find Full Text PDFPotential role of ferric hemoglobin in MS pathogenesis: Effects of oxidative stress and extracellular methemoglobin or its degradation products on myelin components.
Free Radic Biol Med
November 2017
Department of Molecular and Cellular Biology, University of Guelph, 50 Stone Road East, Guelph, Ontario, Canada N1G 2W1. Electronic address:
There is a well-documented relationship between cerebral vasculature and multiple sclerosis (MS) lesions: abnormal accumulations of iron have been found in the walls of the dilated veins in cerebral MS plaques. The source of this iron is unknown, but could be related to the recognized phenomenon of capillary and venous hemorrhages leading to blood extravasation. In turn, hemorrhaging leading to hemolysis results in extracellular release of hemoglobin, a reactive molecule that could induce local oxidative stress, inflammation, and tissue damage.
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