Abstract:
:Amyotrophic lateral sclerosis (ALS) is one of the major forms of motor neuron disease (MND), a group of degenerative disorders causing progressive motor neuron death leading to eventual paralysis and death. The pathogenesis of MND is poorly understood and may include genetic and/or environmental factors, with a common end-stage outcome. The majority of cases are sporadic, with a small percentage of familial cases identified. Mutations in the copper/zinc superoxide dismutase (SOD1) enzyme are frequent in familial ALS, and have allowed for the development of transgenic SOD1 rodent models of ALS. There has been evidence for immune system involvement in the disease, and activated components of the classical complement pathway have been observed in the serum, cerebrospinal fluid and neuronal tissue of diseased individuals. Furthermore, motor neurons and spinal cord tissue from SOD1 transgenic mice show an upregulation in C1q mRNA transcript and protein, in some cases prior to disease onset. Our laboratory has preliminary data indicating a specific pathogenic role for the activation fragment of complement C5 (C5a) in this disease. Using selective C5a receptor antagonists, we dosed SOD1 transgenic rats and observed an extension in survival and reduced motor symptoms compared to untreated rats. Collectively, these clinical and experimental findings suggest that targeting complement using specific inhibitors may represent a novel therapeutic approach to treating MND. Further experimental and clinical studies are required to validate this hypothesis. This review will summarize the clinical and experimental evidence to date implicating complement in the pathogenesis of MND.
journal_name
Adv Exp Med Bioljournal_title
Advances in experimental medicine and biologyauthors
Woodruff TM,Costantini KJ,Taylor SM,Noakes PGsubject
Has Abstractpub_date
2008-01-01 00:00:00pages
143-58eissn
0065-2598issn
2214-8019journal_volume
632pub_type
杂志文章,评审abstract::Reduced Expression in Immortalized Cell (REIC) was cloned by subtractive hybridization method as a gene whose expression is reduced in many human immortalized and neoplastic tumor cells. The REIC, when over-expressed by an adenovirus (Ad-REIC), exhibited a dramatic therapeutic effect on a wide variety of human cancers...
journal_title:Advances in experimental medicine and biology
pub_type: 杂志文章,评审
doi:10.1007/978-1-4614-0254-1_17
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abstract::Cinematographic recordings of sarcomere shortening were performed on glycerinated horseshoe crab muscle myofibrils during Ca-activated contraction. When the preparations at slack length (sarcomere length, 7-9 microns) were locally activated with iontophoretically applied Ca ions, the A-band length did not change appre...
journal_title:Advances in experimental medicine and biology
pub_type: 杂志文章
doi:10.1007/978-1-4684-4703-3_10
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pub_type: 杂志文章,评审
doi:10.1007/978-94-017-7417-8_7
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journal_title:Advances in experimental medicine and biology
pub_type: 杂志文章
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journal_title:Advances in experimental medicine and biology
pub_type: 杂志文章
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journal_title:Advances in experimental medicine and biology
pub_type: 杂志文章
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journal_title:Advances in experimental medicine and biology
pub_type: 杂志文章,评审
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journal_title:Advances in experimental medicine and biology
pub_type: 杂志文章
doi:10.1007/978-981-13-1370-7_5
更新日期:2018-01-01 00:00:00
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journal_title:Advances in experimental medicine and biology
pub_type: 杂志文章
doi:10.1007/978-94-007-6627-3_50
更新日期:2013-01-01 00:00:00
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journal_title:Advances in experimental medicine and biology
pub_type: 杂志文章
doi:10.1007/978-1-4615-4143-1_31
更新日期:1999-01-01 00:00:00
abstract::Drug addiction is a chronic relapsing disorder and a burden to society and individuals. The toxicity induced by drugs related with addiction may trigger dysfunction and death of cells of the central nervous system. The study of alterations of proteins and biomarkers in buccal cells would be beneficial in understanding...
journal_title:Advances in experimental medicine and biology
pub_type: 杂志文章,评审
doi:10.1007/978-3-030-32633-3_20
更新日期:2020-01-01 00:00:00
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journal_title:Advances in experimental medicine and biology
pub_type: 杂志文章
doi:10.1007/978-3-319-10984-8_12
更新日期:2015-01-01 00:00:00
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journal_title:Advances in experimental medicine and biology
pub_type: 杂志文章,评审
doi:10.1007/5584_2019_350
更新日期:2020-01-01 00:00:00
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journal_title:Advances in experimental medicine and biology
pub_type: 杂志文章,评审
doi:10.1007/978-0-387-78261-4_6
更新日期:2008-01-01 00:00:00
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journal_title:Advances in experimental medicine and biology
pub_type: 杂志文章,评审
doi:
更新日期:1996-01-01 00:00:00
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journal_title:Advances in experimental medicine and biology
pub_type: 杂志文章
doi:10.1007/978-1-4684-4394-3_74
更新日期:1982-01-01 00:00:00
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journal_title:Advances in experimental medicine and biology
pub_type: 杂志文章,评审
doi:
更新日期:2001-01-01 00:00:00
abstract::To assess and manage the impact of man-made sounds on fish, we need information on how behavior is affected. Here, wild unrestrained pelagic fish schools were observed under quiet conditions using sonar. Fish were exposed to synthetic piling sounds at different levels using custom-built sound projectors, and behaviora...
journal_title:Advances in experimental medicine and biology
pub_type: 杂志文章
doi:10.1007/978-1-4939-2981-8_113
更新日期:2016-01-01 00:00:00
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journal_title:Advances in experimental medicine and biology
pub_type: 杂志文章
doi:10.1007/978-94-017-9523-4_17
更新日期:2015-01-01 00:00:00
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journal_title:Advances in experimental medicine and biology
pub_type: 杂志文章,评审
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journal_title:Advances in experimental medicine and biology
pub_type: 杂志文章
doi:10.1007/978-1-4899-5199-1_15
更新日期:1979-01-01 00:00:00
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journal_title:Advances in experimental medicine and biology
pub_type: 杂志文章
doi:
更新日期:1997-01-01 00:00:00
abstract::Myocytes are characterized by the presence of highly specialized cytoskeletal structures that are part of regularly spaced functional units distributed over long distances. In this chapter we discuss previously published evidence as well as novel data showing that the proper positioning and architecture of Z-disks and...
journal_title:Advances in experimental medicine and biology
pub_type: 杂志文章
doi:10.1007/978-0-387-84847-1_12
更新日期:2008-01-01 00:00:00
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journal_title:Advances in experimental medicine and biology
pub_type: 杂志文章,评审
doi:10.1007/978-3-319-15774-0_9
更新日期:2015-01-01 00:00:00
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journal_title:Advances in experimental medicine and biology
pub_type: 杂志文章
doi:10.1007/978-3-319-63245-2_4
更新日期:2017-01-01 00:00:00
abstract::In the field of pathology it is clear that molecular genomics and digital imaging represent two promising future directions, and both are as relevant to the tumor microenvironment as they are to the tumor itself (Beck AH et al. Sci Transl Med 3(108):108ra113-08ra113, 2011). Digital imaging, or whole slide imaging (WSI...
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pub_type: 杂志文章,评审
doi:10.1007/978-3-319-42023-3_1
更新日期:2016-01-01 00:00:00
abstract::The clinical challenge on surface engineering of medical devices to prevent microorganisms adhesion and biofilm formation, has become an essential aspect for medical implants. Antibacterial properties of Graphene Oxide (GO) have been demonstrated across a broad spectrum of bacteria, and the different mechanisms of act...
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pub_type: 杂志文章,评审
doi:10.1007/5584_2019_434
更新日期:2020-01-01 00:00:00
abstract::Non-coding RNA (ncRNA) is a class of RNAs that are not act as translational protein templates. They are involved in the regulation of gene transcription, RNA maturation and protein translation, participating in a variety of physiological and physiological processes. NcRNAs have important functions, and are recently on...
journal_title:Advances in experimental medicine and biology
pub_type: 杂志文章,评审
doi:10.1007/978-981-15-1671-9_8
更新日期:2020-01-01 00:00:00
abstract::Membrane potential imaging using voltage-sensitive dyes can be combined with other optical techniques for a variety of applications. Combining voltage imaging with Ca2+ imaging allows correlating membrane potential changes with intracellular Ca2+ signals or with Ca2+ currents. Combining voltage imaging with uncaging t...
journal_title:Advances in experimental medicine and biology
pub_type: 杂志文章,评审
doi:10.1007/978-3-319-17641-3_4
更新日期:2015-01-01 00:00:00
abstract::The multifactorial etiology of hypospadias is becoming more clearly defined with ongoing investigation. Endogenous endocrine abnormalities identified so far include testosterone biosynthesis defects, 5alpha-reductase type 2 mutations, and androgen receptor mutations (the rarest cause, even in cases of severe hypospadi...
journal_title:Advances in experimental medicine and biology
pub_type: 杂志文章,评审
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更新日期:2004-01-01 00:00:00