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Diseases of the Nervous System
Published in George Feuer, Felix A. de la Iglesia, Molecular Biochemistry of Human Disease, 2020
George Feuer, Felix A. de la Iglesia
The time sequence of changes occurring in the proteolipid-protein pattern of degenerating tracts of white matter is different from that of peripheral nerves. The initial decrease of protein synthesis is prolonged up to 140 to 150 d and illustrates the general tendency that nerve cells of the central system show a delayed response to injury. The principal difference between the degenerating peripheral nerve and central white matter is the marked retardation in the removal of various substances in the latter. This may be a reflection of a less intense response of the glia cell in comparison which Schwann cells.
Multiple Sclerosis
Published in Irun R. Cohen, Perspectives on Autoimmunity, 2020
The simplest test which has been applied to various myelin components concerns their ability to induce inflammatory lesions of EAE in CNS (but not PNS) white matter, when injected with adjuvants in experimental animals. MBP is highly effective in such experiments9,110,111 and can produce both acute and chronic relapsing forms of EAE.114 Proteolipid or its apoprotein (PLP), also known as lipophilin, is also an effective encephalitogen.115-118a However, there is a lingering suspicion that active preparations of this protein may be contaminated with traces of MBP. This suspicion is enhanced by the presence of delayed skin reactivity to MBP in guinea pigs sensitized with lipophilin.119 Such cross-reactivity nonetheless, and indeed the encephalitogenic activity itself, may well be explained by sequence homologies as well as homologies of tertiary and quarternary structure between MBP120 and PLP.75,76 In addition to MBP and PLP, an unknown protein in the chloroform-methanol insoluble fraction of bovine myelin has been reported to produce local granulomatous infiltrates in rabbits.121
Axonal Injury and Disease Progression in Multiple Sclerosis
Published in Richard K. Burt, Alberto M. Marmont, Stem Cell Therapy for Autoimmune Disease, 2019
Extensive axonal loss and progression of disability, even in the absence of overt inflammatory activity, suggests that mechanisms other than inflammatory demyelination contribute to axonal degeneration during SP-MS. A number of genes coding for myelin related proteins contribute to long-term viability of axons.26,27 For example, late onset axonal pathology such as atrophy or swelling, cytoskeleton alterations, organelle accumulation and degeneration was observed in mice lacking myelin associated glycoprotein (MAG)12 and proteolipid protein (PLP).13 In PLP-null mice, the axonal pathology was accompanied with progressive clinical disability including impaired gait, tremor and spasticity. Hence, chronically demyelinated axons may undergo degeneration due to lack of trophic support from myelin or myelin forming cells. Recently, it was proposed that abnormal expression of sodium channel subtypes—maladaptive responses to demyelination—may render axons vulnerable to degeneration, conforming with the interesting possibility that MS may involve an acquired channelopathy.28
Exome sequencing of a Pakistani family with spastic paraplegia identified an 18 bp deletion in the cytochrome B5 domain of FA2H
Published in Neurological Research, 2021
Safdar Abbas, Beatrice Brugger, Muhammad Zubair, Sana Gul, Jasmin Blatterer, Julian Wenninger, Khurram Rehman, Benjamin Tatrai, Muzammil Ahmad Khan, Christian Windpassinger
HSP follows different modes of inheritance such as autosomal dominant, autosomal recessive and X-linked, indicating a detailed phenotypical characterization as a critical step prior to molecular analysis [3, 9]. To date, more than 70 chromosomal loci have been linked to HSP [1, 10–12], in which autosomal dominant HSP accounts for about 38% of cases and autosomal recessive accounts for 53% of cases [13]. Physiologically, the HSP proteins have various functions such as (i) axon transport (e.g., KIF1A and KIF5A), (ii) morphologic role in endoplasmic reticulum (e.g., Atlastin, Spastin and reticulon 2), (iii) physiologic role in mitochondria (e.g., chaperonin 60/heat-shock protein 60, paraplegin and mitochondrial ATP6), (iv) synthesis of myelin (e.g., Proteolipid protein and Connexin 47), (v) protein folding and ER-stress response (e.g., NIPA1, K1AA0196 (Strumpellin), and BSCL2 (Seipin), (vi) corticospinal tract and other neuronal development (e.g., cell adhesion molecule and thyroid transporter MCT8), (vii) fatty acid and phospholipid metabolism (e.g., DDHD1, FA2H, NTE, and CYP2U1); and (viii) endosome membrane trafficking and vesicle formation (e.g., AP4B1, KIAA0415, AP4M1, and AP4E) [see 14,for details]. The clinical heterogeneity of HSP greatly reflects the contribution of diverse cellular pathways in disease pathogenesis [5, 8, 15, 16].
Physical exercise counteracts the increase in velocity of propagation of cortical spreading depression imposed by early over-nutrition in rats
Published in Nutritional Neuroscience, 2020
Heloísa Mirelle Costa Monteiro, Débora Carneiro de Mendonça, Mariana Séfora Bezerra Sousa, Angela Amancio-dos-Santos
Regarding nutrition-related effects on brain excitability, HF diet intake accelerated CSD velocity (group HF/S versus group C/S). In fact, the lactation period, which in rats corresponds to the three first weeks of life, is considered very critical and sensitive to adverse environmental conditions.30 HF diet exposure exclusively during lactation has been associated with significant effects in the cerebral environment of the offspring.31 For example, Soares et al.29 have suggested that conjugated linoleic acid treatment carried out during lactation may impair myelin synthesis in the progeny, and this would causally involve CSD acceleration. In our study, the HF diet used contained higher amounts of palmitic, stearic, and oleic acids than the C diet. All these are constituents of the proteolipid encountered in myelin in the central nervous system of the rat brain.32,33 It is possible that fat imbalance during suckling may influence myelination negatively and so promote long-lasting brain electrophysiological effects. Myelin content is reported to be inversely associated to CSD propagation velocity.34
Progress towards the development of Klebsiella vaccines
Published in Expert Review of Vaccines, 2019
Myeongjin Choi, Sharon M Tennant, Raphael Simon, Alan S Cross
A crude outer membrane protein (OMP) vaccine from an unencapsulated KP strain provided complete protection from bacterial challenge [75]. Since OmpA has been considered an antigen which is involved in host defense mechanism with cytokine production and neutrophil recruitment, it was considered a good vaccine candidate [76]. KP OmpA was first cloned in 1998 [77]. Recombinant OmpK17 and OmpK36 [78] as well as OmpA [79] were used as vaccine candidates against lung infection and sepsis caused by KP in murine models with little [78] to modest [79] protective efficacy. OmpA also has been considered a good carrier protein for polysaccharide haptens that induces CD4 + T cell lymphoproliferative and Th1/Th2 responses as well as primes for cytotoxic T lymphocytes (CTL) epitopes in mice [80]. KP recombinant rP40 OMP has been reported to be an effective carrier protein for other important bacterial polysaccharide vaccine antigens such as a peptide of respiratory syncytial virus [81] or a polysaccharide derived from Haemophilus [80]. KP-derived extracellular vesicles, spherical, nanometer-sized proteolipids enriched in OMPs and LPS, induced innate immune responses and both humoral and cellular immunity leading to protection against homologous KP challenge [82].