Abstract：
:Animal models are an important tool to study the pathophysiology of Machado-Joseph Disease (MJD). So far, animal models using simple organisms (like the round worm Caenorhabditis elegans or the fruit fly drosophila) but also mammalian models (mouse and even a non-human primate model) have been generated to study MJD. While simple organisms made an important contribution to the identification of pathophysiological mechanisms in MJD and were further used for modifier and screening purposes, mammalian models recapitulate major disease features of MJD in humans and are therefore a highly valuable tool for e.g. the validation of mechanisms or for pre-clinical validation of treatment approaches. Here we give an overview about the strategies which were used to model MJD and about the different models generated so far. We further highlight advantages of specific model organisms and describe the new findings which were made employing these animal models of MJD.
journal_name
Adv Exp Med Bioljournal_title
Advances in experimental medicine and biologyauthors
Schmidt J,Schmidt Tdoi
10.1007/978-3-319-71779-1_15subject
Has Abstractpub_date
2018-01-01 00:00:00pages
289-308eissn
0065-2598issn
2214-8019journal_volume
1049pub_type
杂志文章,评审abstract：:Since the birth of proteomics science in the 1990, the number of applications and of sample preparation methods has grown exponentially, making a huge contribution to the knowledge in life science disciplines. Continuous improvements in the sample treatment strategies unlock and reveal the fine details of disease mech...
journal_title：Advances in experimental medicine and biology
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doi：10.1007/978-3-030-12298-0_4
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abstract：:Vaccines against viral disease have traditionally relied on attenuated virus strains or inactivation of infectious virus. Subunit vaccines based on viral proteins expressed in heterologous systems have been effective for some pathogens, but have often suffered from poor immunogenicity due to incorrect protein folding ...
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journal_title：Advances in experimental medicine and biology
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doi：10.1007/978-1-4939-2404-2_7
更新日期：2015-01-01 00:00:00
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journal_title：Advances in experimental medicine and biology
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journal_title：Advances in experimental medicine and biology
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doi：
更新日期：2002-01-01 00:00:00
abstract：:The Utah electrode array (UEA) and its many derivatives have become a gold standard for high-channel count bi-directional neural interfaces, in particular in human subject applications. The chapter provides a brief overview of leading electrode concepts and the context in which the UEA has to be understood. It goes on...
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
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journal_title：Advances in experimental medicine and biology
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doi：10.1007/978-981-32-9636-7_17
更新日期：2019-01-01 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,评审
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更新日期：2002-01-01 00:00:00
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journal_title：Advances in experimental medicine and biology
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doi：
更新日期：2010-01-01 00:00:00
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pub_type： 杂志文章
doi：10.1007/978-1-4614-4989-8_14
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,评审
doi：10.1007/978-3-319-42023-3_1
更新日期：2016-01-01 00:00:00
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journal_title：Advances in experimental medicine and biology
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doi：
更新日期：2010-01-01 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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更新日期：2019-01-01 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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更新日期：2012-01-01 00:00:00
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pub_type： 杂志文章,评审
doi：10.1007/978-1-4614-7783-9_2
更新日期：2014-01-01 00:00:00
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pub_type： 杂志文章,评审
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更新日期：2019-01-01 00:00:00
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journal_title：Advances in experimental medicine and biology
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更新日期：2017-01-01 00:00:00
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journal_title：Advances in experimental medicine and biology
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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
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journal_title：Advances in experimental medicine and biology
pub_type： 杂志文章,评审
doi：10.1007/978-1-4615-9026-2_25
更新日期：1975-01-01 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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更新日期：2019-01-01 00:00:00
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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
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更新日期：2019-01-01 00:00:00
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journal_title：Advances in experimental medicine and biology
pub_type： 杂志文章
doi：10.1007/0-306-46826-3_15
更新日期：2000-01-01 00:00:00
abstract：:Genetic variants of high-temperature requirement A serine peptidase 1 (HTRA1) and age-related maculopathy susceptibility 2 (ARMS2) are associated with age-related macular degeneration (AMD). One HTRA1 single nucleotide polymorphism (SNP) is situated in the promotor region (rs11200638) resulting in increased expression...
journal_title：Advances in experimental medicine and biology
pub_type： 杂志文章
doi：10.1007/978-3-030-27378-1_1
更新日期：2019-01-01 00:00:00
abstract：:The 3'-portion of the genome from a Taiwan isolate of porcine reproductive and respiratory syndrome (PRRS) virus, strain MD-001, was cloned and sequenced. The resultant 549 nucleotides contained an open reading frame with a coding capacity of 123 amino acids (predicted Mr 13,600). The predicted protein corresponds to ...
journal_title：Advances in experimental medicine and biology
pub_type： 杂志文章
doi：10.1007/978-1-4615-5331-1_103
更新日期：1998-01-01 00:00:00
