Altered Cell Membranes in Creutzfeldt-Jakob Disease

Bass, Norman H.; Hess, Helen H.; Pope, Alfred

doi:10.1001/archneur.1974.00490390056005

Cited by 16 publications

(2 citation statements)

References 44 publications

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“…In contrast, the expression of the gene coding for galactocerebrosidase (GALC) is reduced in CJD MM1 and VV2, thus indicating possible impairment in glycosphingolipid metabolism. To our knowledge, most studies of brain lipids in CJD were carried out many years ago and the main focus was on ganglioside alterations [39][40][41][42]. Since the present studies are focused on the cerebral cortex, further investigation is needed to get information about brain lipid alterations in the white matter in prion diseases.…”

Section: Discussionmentioning

confidence: 95%

Altered gene transcription linked to astrocytes and oligodendrocytes in frontal cortex in Creutzfeldt-Jakob disease

Benito

Gonzalez

Ferrer

2018

Prion

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Targeted expression of genes coding for proteins specific to astrocytes, oligodendrocytes and myelin was performed in frontal cortex area 8 of Creutzfeldt-Jakob disease methionine/methionine and valine/valine (CJD MM1 and VV2, respectively) compared with controls. GFAP (glial fibrillary acidic protein) mRNA was up-regulated whereas SLC1A2 (solute carrier family 1 member 2, coding for glutamate transporter 1: GLT1), AQ4 (aquaporin 4), MPC1 (mitochondrial pyruvate carrier 1) and UCP5 (mitochondrial uncoupled protein 5) mRNAs were significantly down-regulated in CJD MM1 and CJD VV2, and GJA1 (connexin 43) in CJD VV2. OLIG1 and OLIG2 (oligodendocyte transcription factor 1 and 2, respectively), SOX10 (SRY-Box10) and oligodendroglial precursor cell (OPC) marker NG2 (neuronal/glial antigen) 2 were preserved, but GALC (coding for galactosylceramidase), SLC2A1 (solute carrier family 2 member 1: glucose transporter member 1: GLUT1) and MCT1 (monocarboxylic acid transporter 1) mRNA expression levels were significantly reduced in CJD MM1 and CJD VV2. Expression levels of most genes linked to myelin were not altered in the cerebral cortex in CJD. Immunohistochemistry to selected proteins disclosed individual variations but GFAP, Olig-2, AQ4 and GLUT1 correlated with mRNA levels, whereas GLT1 was subjected to individual variations. However, MPC1, UCP5 and MCT1 decrease was more closely related to the respective reduced neuronal immunostaining. These observations support the idea that molecular deficits linked to energy metabolism and solute transport in astrocytes and oligodendrocytes, in addition to neurons, are relevant in the pathogenesis of cortical lesions in CJD.

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Section: Discussionmentioning

confidence: 95%

Altered gene transcription linked to astrocytes and oligodendrocytes in frontal cortex in Creutzfeldt-Jakob disease

Benito

Gonzalez

Ferrer

2018

Prion

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“…Over the four past decades substantial progress have been made in elucidating the mechanisms by which nerve plasma membranes and cytomembranes are damaged. Nerve cell membrane alterations were earlier described by Bass [1] in Creutzfeldt-Jacob disease. Significant enrichment in cholesterol ester was found by [2] In nerve cell membranes in adrenoleukodystrophy.…”

Section: Introductionmentioning

confidence: 76%

Ultrastructural Pathology of Plasma and Endoplasmic Reticulum Membranes of Nerve and Glial Cells: A Review

Castejón¹

2018

BJSTR

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The alteration of nerve cell plasma membranes is reviewed in some neuropathological conditions. In moderate brain oedema a continuous plasma membrane is observed but the cytoplasmic membranes, such as smooth and rough endoplasmic reticulum membranes appear damaged. In severe oedema, fragmentation of plasma membrane, enlargement and focal necrosis of rough endoplasmic cisterns and nuclear envelope, detachment of membrane-bound ribosomes, and reduction of polysome are found. Shallow and deep invaginations of plasma membrane, and the formation of endocytic and clathrin-coated vesicles are seen. In astrocyte cells, areas of focal necrosis and fragmented limiting plasma membrane, overdistended rough endoplasmic reticulum cisterns with extense degranulated membrane domains, and vacuoles of smooth endoplasmic reticulum with necrotic limiting membrane are observed. Oligodendroglia cells show notably edematous changes featured by lacunar enlargement of rough endoplasmic reticulum and nuclear envelope, detachment of membrane bound ribosomes, and discontinuous plasma membrane. Plastic changes and damage of synaptic membranes are found. Synaptic vesicle exocytosis and endocytosis at the non-specialized regions of presynaptic ending limiting membrane are frequently observed at activated synapses. In severe brain edema, synaptic disassembly occurs featured by wide separation of pre and postsynaptic membranes, and loss of peri synaptic astrocytic glial escheatment. Disruption, fusion and disassembly of interastrocytary gap junctions have also been observed. The endothelial cell luminal membrane of brain capillaries undergoes profound activity changes that characterize increased cerebrovascular permeability, such as increased formation of micro-and macropinocytotic vesicles, clathrin coated vesicles, and emission of pseudopods to form endothelial vacuoles. The alterations of nerve cell plasma membranes and cytomembranes are related with the anoxic-ischemic conditions of brain parenchyma. The role of free radical and lipid peroxidation, disturbed energy metabolism, altered metabolic cascades, glutamate excitotoxicity, haemoglobin toxicity, protein aggregation, and presence of extracellular oedema fluid are discussed in relation with the derangement of nerve cells membranes.

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The Presenile Dementias

Poser¹

1975

JAMA

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Altered Cell Membranes in Creutzfeldt-Jakob Disease

Cited by 16 publications

References 44 publications

Altered gene transcription linked to astrocytes and oligodendrocytes in frontal cortex in Creutzfeldt-Jakob disease

Altered gene transcription linked to astrocytes and oligodendrocytes in frontal cortex in Creutzfeldt-Jakob disease

Ultrastructural Pathology of Plasma and Endoplasmic Reticulum Membranes of Nerve and Glial Cells: A Review

The Presenile Dementias

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