Abstract:
:We have generated a transgenic rat model of Charcot-Marie-Tooth disease type 1A (CMT1A) providing formal proof that this neuropathy can be caused by increased expression of peripheral myelin protein-22 (PMP22). Heterozygous PMP22-transgenic rats develop muscle weakness and gait abnormalities as well as reduced nerve conduction velocities and EMG abnormalities, which closely resemble recordings in patients with CMT1A. Dys- and demyelination, Schwann cell hypertrophy, and "onion bulb" formation are also similar to findings in humans. When bred to homozygosity, transgenic rats completely fail to elaborate myelin, but all myelin-forming Schwann cells segregate with axons in the normal one-to-one ratio. Although arrested at this "promyelin" stage, differentiation proceeds in homozygous rats at the molecular level, as demonstrated by high-level expression of myelin structural genes. Intracellular trafficking of the wild-type protein is not visibly impaired, even when strongly overexpressed, suggesting that PMP22 blocks myelin assembly in a late Golgi/cell membrane compartment of the affected Schwann cell.
journal_name
Ann N Y Acad Scijournal_title
Annals of the New York Academy of Sciencesauthors
Niemann S,Sereda MW,Rossner M,Stewart H,Suter U,Meinck HM,Griffiths IR,Nave KAsubject
Has Abstractpub_date
1999-09-14 00:00:00pages
254-61eissn
0077-8923issn
1749-6632journal_volume
883pub_type
杂志文章,评审abstract::We have previously shown that increased dosage of the mouse protein zero gene (Mpz) causes a dysmyelinating neuropathy in transgenic (Tg80) mice. To ask whether the dysmyelination is dose dependent, we inbred one of the Tg80 lines and compared the resulting phenotype in homozygous and heterozygous mice. Whereas hetero...
journal_title:Annals of the New York Academy of Sciences
pub_type: 杂志文章
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更新日期:1999-10-01 00:00:00
abstract::Membrane bioreactors can be operated with intermittent permeation and continuous aeration. Aeration close to the surface of a submerged membrane helps to maintain a membrane surface that is free from fouling. The conditions under which this occurs depend on the interaction between flux and aeration rate. Increased flu...
journal_title:Annals of the New York Academy of Sciences
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abstract::It is imperative that operating personnel and regulatory agencies have secure, advance, and accurate knowledge of the true surface properties of materials to be contacted with flowing blood. The actual devices must be characterized in their sterile, immediate pre-exposure states. Although very sensitive electronic ins...
journal_title:Annals of the New York Academy of Sciences
pub_type: 杂志文章,评审
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更新日期:1987-01-01 00:00:00
abstract::Pulmonary arterial hypertension (PAH) is an important cause of death in systemic sclerosis (SSc), despite the improvement of therapies. An early diagnosis and the use of drugs interfering with the main pathogenic pathways of PAH is pivotal for the improvement of prognosis in primary PAH and PAH secondary to autoimmune...
journal_title:Annals of the New York Academy of Sciences
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journal_title:Annals of the New York Academy of Sciences
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abstract::Few studies have evaluated the impact of nutritional supplementation among pregnant adolescents. We examined the effects of the Rang Din Nutrition Study (RDNS) interventions on children born to mothers <20 years of age. The RDNS was a cluster-randomized effectiveness trial with four arms: (1) women and children both r...
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更新日期:2011-05-01 00:00:00
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journal_title:Annals of the New York Academy of Sciences
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更新日期:2010-08-01 00:00:00
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journal_title:Annals of the New York Academy of Sciences
pub_type: 杂志文章,评审
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更新日期:2002-09-01 00:00:00
abstract::We investigated the role of thrombin in the pathogenesis in atherosclerosis and restenosis. First we examined the effect of thrombin on cultured human vascular smooth muscle cells (VSMC). We showed that thrombin acts as a mitogen on VSMC through thrombin receptor. The expression of thrombin receptor was increased in t...
journal_title:Annals of the New York Academy of Sciences
pub_type: 杂志文章
doi:10.1111/j.1749-6632.1997.tb52024.x
更新日期:1997-04-15 00:00:00
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journal_title:Annals of the New York Academy of Sciences
pub_type: 杂志文章
doi:
更新日期:1998-05-30 00:00:00
abstract::Currently, several rickettsioses are officially being reported in the Russian Federation. These are epidemic typhus and Brill-Zinsser disease, both caused by Rickettsia prowazekii which has a historic prevalence in Russia. Nowadays only single sporadic cases of R. prowazekii infection are reported. The last significan...
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journal_title:Annals of the New York Academy of Sciences
pub_type: 杂志文章,评审
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abstract::Our paper reviews recent progress in the study and understanding of observed trends in extreme rainfall events in the Metropolitan Area of São Paulo (MASP). These are discussed in relation to hydrometeorological hazards that trigger natural disasters, such as flash floods, landslides, and droughts, that affect the pop...
journal_title:Annals of the New York Academy of Sciences
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abstract::Sm-p80-based vaccine efficacy for Schistosoma mansoni was evaluated in a baboon model of infection and disease. The study was designed to replicate a human vaccine implementation scenario for endemic regions in which vaccine would be administered following drug treatment of infected individuals. In our study, the Sm-p...
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journal_title:Annals of the New York Academy of Sciences
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abstract::To live beyond the octogenarian years, population and molecular genetic studies of centenarian sibships indicate that genetic factors play an increasingly important role as the limit of life span is approached. These factors are likely to influence basic mechanisms of aging that in turn broadly influence susceptibilit...
journal_title:Annals of the New York Academy of Sciences
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journal_title:Annals of the New York Academy of Sciences
pub_type: 杂志文章
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更新日期:2000-04-01 00:00:00
abstract::The free radical theory of aging proposes that oxidative stress is one of the determinants of an organism's life span. In Caenorhabditis elegans, genetic or environmental changes have been shown to modulate life span. Here we discuss whether changes in the generation and destruction of free radicals are implicated in ...
journal_title:Annals of the New York Academy of Sciences
pub_type: 杂志文章,评审
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abstract::After successful marrow transplantation (BMT) iron overload remains an important cause of morbidity in Thalassemia. After BMT, patients have normal erythropoiesis capable of producing a hyperplastic response to phlebotomy so that this procedure can be contemplated as a method of mobilizing iron from overloaded tissues...
journal_title:Annals of the New York Academy of Sciences
pub_type: 杂志文章,多中心研究
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更新日期:1998-06-30 00:00:00
abstract::The results reviewed here demonstrate that vestibulospinal and vestibulo-ocular interneurons in the chicken embryo are organized into coherent clusters, each of which occupies a unique spatial domain and has a characteristic axon trajectory. Moreover, the vestibulo-ocular clusters have specific termination patterns in...
journal_title:Annals of the New York Academy of Sciences
pub_type: 杂志文章,评审
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abstract::Human immunodeficiency virus type 1 (HIV-1) causes neuronal degeneration and, at a late stage, creates HIV-associated dementia (HAD) and other neurological abnormalities. Therefore, the need for neuroprotective agents is great. However, therapeutic agents that reduce HIV neurotoxicity are difficult to characterize and...
journal_title:Annals of the New York Academy of Sciences
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更新日期:2007-12-01 00:00:00
abstract::The discovery of mtDNA types common to Asians and Amerinds (types A, B, C, and D) forced investigators to search for those nations of Asia which, though not considered the ancestors of the Amerinds, have retained a close genetic resemblance with them. We collected samples and studied the gene pools of the Turkic-speak...
journal_title:Annals of the New York Academy of Sciences
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abstract::The gene encoding thermostable alanine recemase from Bacillus stearothermophilus was cloned and expressed in E. coli. The enzyme was purified to homogeneity from cell extracts of E. coli carrying a plasmid designated pICR4. The alanine racemase gene sequenced was found to contain an open reading frame of 1158 nucleoti...
journal_title:Annals of the New York Academy of Sciences
pub_type: 杂志文章,评审
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更新日期:1990-01-01 00:00:00