Lessons from models of SOD1-linked familial ALS.

Abstract:

:Ten years ago, the linkage between mutations in the gene coding for the antioxidant enzyme Cu,Zn superoxide dismutase (SOD1) and the neurodegenerative disease known as familial amyotrophic lateral sclerosis (FALS) was established. This finding has prompted a myriad of new studies in experimental models aimed at investigating the toxic function of the mutant enzymes. The cellular functions that are impaired in motoneurons as a consequence of molecular alterations induced by the expression of FALS SOD1 converge on pathways that might be activated in sporadic ALS by other toxic factors. Recent data demonstrate that, although motoneurons are lost in patients, other cell types are also affected and actively contribute to the pathogenesis of the disease.

journal_name

Trends Mol Med

authors

Bendotti C,Carrì MT

doi

10.1016/j.molmed.2004.06.009

keywords:

subject

Has Abstract

pub_date

2004-08-01 00:00:00

pages

393-400

issue

8

eissn

1471-4914

issn

1471-499X

pii

S1471-4914(04)00159-5

journal_volume

10

pub_type

杂志文章,评审
  • Metabolic regulation by SIRT3: implications for tumorigenesis.

    abstract::Cancer cells meet their needs for energy and biomass production by consuming high levels of nutrients and rewiring metabolism to support macromolecular biosynthesis. Mitochondrial enzymes play central roles in anabolic growth, and acetylation may provide a key layer of regulation over mitochondrial metabolic pathways....

    journal_title:Trends in molecular medicine

    pub_type: 杂志文章,评审

    doi:10.1016/j.molmed.2012.05.004

    authors: Finley LW,Haigis MC

    更新日期:2012-09-01 00:00:00

  • Kaposi's sarcoma as a model for cancer immunotherapy.

    abstract::Physicians have, for over a century, attempted to harness the potential therapeutic power of the immune system to treat patients with cancer. The discovery that cancer regression can be achieved by immune rejection of tumour antigens theoretically allows the eradication of neoplastic cells without toxicity to normal t...

    journal_title:Trends in molecular medicine

    pub_type: 杂志文章,评审

    doi:10.1016/j.molmed.2004.02.006

    authors: Stebbing J,Bower M,Srivastava P

    更新日期:2004-04-01 00:00:00

  • CAR-T cells and solid tumors: tuning T cells to challenge an inveterate foe.

    abstract::Recent reports on the impressive efficacy of adoptively transferred T cells to challenge cancer in early phase clinical trials have significantly raised the profile of T cell therapy. Concomitantly, general expectations are also raised by these reports, with the natural aspiration to deliver this therapy over a wide r...

    journal_title:Trends in molecular medicine

    pub_type: 杂志文章,评审

    doi:10.1016/j.molmed.2012.04.009

    authors: Gilham DE,Debets R,Pule M,Hawkins RE,Abken H

    更新日期:2012-07-01 00:00:00

  • Protein transport into the endoplasmic reticulum: mechanisms and pathologies.

    abstract::Transport into the endoplasmic reticulum (ER) is the crucial step in the biosynthesis of most secretory proteins and many membrane proteins. The products of the SIL1, SEC62 and SEC63 genes act in concert with the SEC61 complex and the molecular chaperones BiP and GRP170 to transport proteins into the ER. Interestingly...

    journal_title:Trends in molecular medicine

    pub_type: 杂志文章

    doi:10.1016/j.molmed.2006.10.004

    authors: Zimmermann R,Müller L,Wullich B

    更新日期:2006-12-01 00:00:00

  • p53 post-translational modification: deregulated in tumorigenesis.

    abstract::The p53 tumor suppressor protein has well-established roles in monitoring various types of stress signals by activating specific transcriptional targets that control cell cycle arrest and apoptosis, although some activities are also mediated in a transcription-independent manner. Here, we review the recent advances in...

    journal_title:Trends in molecular medicine

    pub_type: 杂志文章,评审

    doi:10.1016/j.molmed.2010.09.002

    authors: Dai C,Gu W

    更新日期:2010-11-01 00:00:00

  • Learning from Jekyll to control Hyde: Hedgehog signaling in development and cancer.

    abstract::The Hedgehog (Hh) cascade controls cell proliferation, differentiation and patterning of tissues during embryogenesis but is largely suppressed in the adult. The Hh pathway can become reactivated in cancer. Here, we assimilate data from recent studies to understand how and when the Hh pathway is turned on to aid the n...

    journal_title:Trends in molecular medicine

    pub_type: 杂志文章,评审

    doi:10.1016/j.molmed.2010.05.003

    authors: Barakat MT,Humke EW,Scott MP

    更新日期:2010-08-01 00:00:00

  • Immunotherapy: target the stroma to hit the tumor.

    abstract::For decades it has been assumed that T cells reject tumors essentially by direct killing. However, solid tumors are composed of malignant cells and a variety of different nonmalignant cells, referred to as tumor stroma. Stromal cells, such as endothelial cells, fibroblasts and inflammatory cells, often support tumor g...

    journal_title:Trends in molecular medicine

    pub_type: 杂志文章,评审

    doi:10.1016/j.molmed.2005.03.002

    authors: Kammertoens T,Schüler T,Blankenstein T

    更新日期:2005-05-01 00:00:00

  • Ocular gene therapy: current progress and future prospects.

    abstract::As gene therapy begins to produce its first clinical successes, interest in ocular gene transfer has grown owing to the favorable safety and efficacy characteristics of the eye as a target organ for drug delivery. Important advances also include the availability of viral and non-viral vectors that are able to efficien...

    journal_title:Trends in molecular medicine

    pub_type: 杂志文章,评审

    doi:10.1016/j.molmed.2008.11.003

    authors: Colella P,Cotugno G,Auricchio A

    更新日期:2009-01-01 00:00:00

  • Neuronal Regulation of eIF2α Function in Health and Neurological Disorders.

    abstract::A key site of translation control is the phosphorylation of the eukaryotic translation initiation factor 2α (eIF2α), which reduces the rate of GDP to GTP exchange by eIF2B, leading to altered translation. The extent of eIF2α phosphorylation within neurons can alter synaptic plasticity. Phosphorylation of eIF2α is trig...

    journal_title:Trends in molecular medicine

    pub_type: 杂志文章,评审

    doi:10.1016/j.molmed.2018.04.001

    authors: Moon SL,Sonenberg N,Parker R

    更新日期:2018-06-01 00:00:00

  • What are microarrays teaching us about sleep?

    abstract::Many fundamental questions about sleep remain unanswered. The presence of sleep across phyla suggests that it must serve a basic cellular and/or molecular function. Microarray studies, performed in several model systems, have identified classes of genes that are sleep-state regulated. This has led to the following con...

    journal_title:Trends in molecular medicine

    pub_type: 杂志文章,评审

    doi:10.1016/j.molmed.2008.12.002

    authors: Mackiewicz M,Zimmerman JE,Shockley KR,Churchill GA,Pack AI

    更新日期:2009-02-01 00:00:00

  • Apolipoprotein E and cholesterol metabolism in the pathogenesis and treatment of Alzheimer's disease.

    abstract::There is much evidence suggesting that there is a strong relationship between the deterioration of brain lipid homeostasis, vascular changes and the pathogenesis of Alzheimer's disease (AD). These associations include: (1). recognition that a key cholesterol transporter, apolipoprotein E type 4, acts a major genetic r...

    journal_title:Trends in molecular medicine

    pub_type: 杂志文章,评审

    doi:10.1016/s1471-4914(03)00007-8

    authors: Poirier J

    更新日期:2003-03-01 00:00:00

  • Transgenerational epigenetic inheritance requires a much deeper analysis.

    abstract::In his article, Szyf [1] has addressed the key challenges in and the controversies surrounding nongenetic inheritance. However, crucial findings that are important to counter two major criticisms held against transgenerational inheritance, especially in mammals--namely epigenetic memory survival across generations, an...

    journal_title:Trends in molecular medicine

    pub_type: 评论,信件

    doi:10.1016/j.molmed.2015.02.010

    authors: Sharma A

    更新日期:2015-05-01 00:00:00

  • mTOR: A pathogenic signaling pathway in developmental brain malformations.

    abstract::The mTOR signaling network functions as a pivotal regulatory cascade during the development of the cerebral cortex. Aberrant hyperactivation of mTOR as a consequence of loss-of-function gene mutations encoding mTOR inhibitor proteins such as TSC1, TSC2, PTEN and STRADα has been recently linked to developmental cortica...

    journal_title:Trends in molecular medicine

    pub_type: 杂志文章,评审

    doi:10.1016/j.molmed.2011.07.008

    authors: Crino PB

    更新日期:2011-12-01 00:00:00

  • Human cancer immunotherapy with antibodies to the PD-1 and PD-L1 pathway.

    abstract::The programmed death 1 (PD-1) receptor and its ligands programmed death ligand 1 (PD-L1) and PD-L2, members of the CD28 and B7 families, play critical roles in T cell coinhibition and exhaustion. Overexpression of PD-L1 and PD-1 on tumor cells and tumor-infiltrating lymphocytes, respectively, correlates with poor dise...

    journal_title:Trends in molecular medicine

    pub_type: 杂志文章,评审

    doi:10.1016/j.molmed.2014.10.009

    authors: Ohaegbulam KC,Assal A,Lazar-Molnar E,Yao Y,Zang X

    更新日期:2015-01-01 00:00:00

  • Towards a molecular understanding of human diseases using Dictyostelium discoideum.

    abstract::The social amoeba Dictyostelium discoideum is increasingly being used as a simple model for the investigation of problems that are relevant to human health. This article focuses on several recent examples of Dictyostelium-based biomedical research, including the analysis of immune-cell disease and chemotaxis, centroso...

    journal_title:Trends in molecular medicine

    pub_type: 杂志文章,评审

    doi:10.1016/j.molmed.2006.07.003

    authors: Williams RS,Boeckeler K,Gräf R,Müller-Taubenberger A,Li Z,Isberg RR,Wessels D,Soll DR,Alexander H,Alexander S

    更新日期:2006-09-01 00:00:00

  • ANGPTL4 in Metabolic and Cardiovascular Disease.

    abstract::Alterations in circulating lipids and ectopic lipid deposition impact on the risk of developing cardiovascular and metabolic diseases. Lipoprotein lipase (LPL) hydrolyzes fatty acids (FAs) from triglyceride (TAG)-rich lipoproteins including very low density lipoproteins (VLDLs) and chylomicrons, and regulates their di...

    journal_title:Trends in molecular medicine

    pub_type: 杂志文章,评审

    doi:10.1016/j.molmed.2019.05.010

    authors: Aryal B,Price NL,Suarez Y,Fernández-Hernando C

    更新日期:2019-08-01 00:00:00

  • MicroRNA Regulators of Anxiety and Metabolic Disorders.

    abstract::Anxiety-related and metabolic disorders are under intense research focus. Anxiety-induced microRNAs (miRNAs) are emerging as regulators that are not only capable of suppressing inflammation but can also induce metabolic syndrome-related processes. We summarize here evidence linking miRNA pathways which share regulator...

    journal_title:Trends in molecular medicine

    pub_type: 杂志文章,评审

    doi:10.1016/j.molmed.2016.07.001

    authors: Meydan C,Shenhar-Tsarfaty S,Soreq H

    更新日期:2016-09-01 00:00:00

  • Differential Markers of Bacterial and Viral Infections in Children for Point-of-Care Testing.

    abstract::Children suffering from infectious diseases, both bacterial and viral, are often treated with empirical antibiotics. Keeping in mind both the menace of microorganisms and antibiotic toxicity, it is imperative to develop point-of-care testing (POCT) to discriminate bacterial from viral infections, and to define indicat...

    journal_title:Trends in molecular medicine

    pub_type: 杂志文章,评审

    doi:10.1016/j.molmed.2020.09.004

    authors: Tsao YT,Tsai YH,Liao WT,Shen CJ,Shen CF,Cheng CM

    更新日期:2020-12-01 00:00:00

  • Balancing histone methylation activities in psychiatric disorders.

    abstract::Alterations in histone lysine methylation and other epigenetic regulators of gene expression contribute to changes in brain transcriptomes in mood and psychosis spectrum disorders, including depression and schizophrenia. Genetic association studies and animal models implicate multiple lysine methyltransferases and dem...

    journal_title:Trends in molecular medicine

    pub_type: 杂志文章,评审

    doi:10.1016/j.molmed.2011.02.003

    authors: Peter CJ,Akbarian S

    更新日期:2011-07-01 00:00:00

  • An Immunological Perspective on Neonatal Sepsis.

    abstract::Despite concerted international efforts, mortality from neonatal infections remains unacceptably high in some areas of the world, particularly for premature infants. Recent developments in flow cytometry and next-generation sequencing technologies have led to major discoveries over the past few years, providing a more...

    journal_title:Trends in molecular medicine

    pub_type: 杂志文章,评审

    doi:10.1016/j.molmed.2016.02.001

    authors: Kan B,Razzaghian HR,Lavoie PM

    更新日期:2016-04-01 00:00:00

  • Apoptosis-regulating proteins as targets for drug discovery.

    abstract::Defects in the regulation of apoptosis (programmed cell death) contribute to many diseases, including pathologies associated with cell loss (e.g. stroke, heart failure, neurodegeneration and AIDS), and disorders characterized by a failure to eliminate harmful cells (e.g. cancer, autoimmunity). Apoptosis is caused by a...

    journal_title:Trends in molecular medicine

    pub_type: 杂志文章,评审

    doi:10.1016/s1471-4914(01)02026-3

    authors: Reed JC

    更新日期:2001-07-01 00:00:00

  • MLL: a histone methyltransferase disrupted in leukemia.

    abstract::Rearrangements of the MLL gene, which is located at chromosome 11q23, are associated with aggressive acute leukemias in both children and adults. MLL regulates Hox gene expression through direct promoter binding and histone modification. MLL rearrangements occurring in leukemia include MLL fusion genes, partial tandem...

    journal_title:Trends in molecular medicine

    pub_type: 杂志文章,评审

    doi:10.1016/j.molmed.2004.08.005

    authors: Hess JL

    更新日期:2004-10-01 00:00:00

  • Mitogen-activated protein kinases in male reproductive function.

    abstract::Recent studies have shown that male reproductive function is modulated via the mitogen-activated protein kinase (MAPK) cascade. The MAPK cascade is involved in numerous male reproductive processes, including spermatogenesis, sperm maturation and activation, capacitation and acrosome reaction, before fertilization of t...

    journal_title:Trends in molecular medicine

    pub_type: 杂志文章,评审

    doi:10.1016/j.molmed.2009.02.002

    authors: Li MW,Mruk DD,Cheng CY

    更新日期:2009-04-01 00:00:00

  • EphrinB2-EphB4-RASA1 Signaling in Human Cerebrovascular Development and Disease.

    abstract::Recent whole exome sequencing studies in humans have provided novel insight into the importance of the ephrinB2-EphB4-RASA1 signaling axis in cerebrovascular development, corroborating and extending previous work in model systems. Here, we aim to review the human cerebrovascular phenotypes associated with ephrinB2-Eph...

    journal_title:Trends in molecular medicine

    pub_type: 杂志文章,评审

    doi:10.1016/j.molmed.2019.01.009

    authors: Zeng X,Hunt A,Jin SC,Duran D,Gaillard J,Kahle KT

    更新日期:2019-04-01 00:00:00

  • Microchimerism: incidental byproduct of pregnancy or active participant in human health?

    abstract::It is now well recognized that cells traffic in both directions between fetus and mother during pregnancy. Moreover, fetal cells have been found to persist for years, probably for a lifetime, in the circulation of healthy women. Harboring of cells from another individual at low levels is called microchimerism. Women h...

    journal_title:Trends in molecular medicine

    pub_type: 杂志文章

    doi:10.1016/s1471-4914(01)02269-9

    authors: Nelson JL

    更新日期:2002-03-01 00:00:00

  • Oncogenic transformation of human cells: shortcomings of rodent model systems.

    abstract::Long-standing difficulties in the in vitro transformation of human cells have been overcome. Using telomerase, several successful oncogene-mediated transformations of human cells have been reported and the following cellular requirements for human cell transformation have been proposed: the maintenance of telomere seq...

    journal_title:Trends in molecular medicine

    pub_type: 杂志文章,评审

    doi:10.1016/j.molmed.2004.09.001

    authors: Akagi T

    更新日期:2004-11-01 00:00:00

  • Why have clinical trials in sepsis failed?

    abstract::The systemic inflammatory response is biologically complex, redundant, and activated by both infectious and noninfectious triggers. Its manipulation can cause both benefit and harm. More than 100 randomized clinical trials have tested the hypothesis that modulating the septic response to infection can improve survival...

    journal_title:Trends in molecular medicine

    pub_type: 杂志文章

    doi:10.1016/j.molmed.2014.01.007

    authors: Marshall JC

    更新日期:2014-04-01 00:00:00

  • Non-viral and hybrid vectors in human gene therapy: an update.

    abstract::Non-viral DNA vectors have several advantages over viral vectors. For example, virus production is expensive and there are safety concerns regarding viral manipulations. In addition, the size of the delivered plasmid is limited by the size of the viral capsid, whereas this is not a problem with non-viral vectors. The ...

    journal_title:Trends in molecular medicine

    pub_type: 杂志文章,评审

    doi:10.1016/s1471-4914(03)00005-4

    authors: Schmidt-Wolf GD,Schmidt-Wolf IG

    更新日期:2003-02-01 00:00:00

  • Immune Checkpoint Molecules, Personalized Immunotherapy, and Autoimmune Diabetes.

    abstract::Although significant progress has been made in understanding autoimmunity, no immunotherapy to effectively halt immune-mediated destruction of β cells in type 1 diabetes (T1D) is currently available. For successful immunotherapy it will be necessary to identify novel drug targets as well as robust immunologic biomarke...

    journal_title:Trends in molecular medicine

    pub_type: 杂志文章,评审

    doi:10.1016/j.molmed.2018.08.005

    authors: Orabona C,Mondanelli G,Puccetti P,Grohmann U

    更新日期:2018-11-01 00:00:00

  • Sialic acids in human health and disease.

    abstract::The surfaces of all vertebrate cells are decorated with a dense and complex array of sugar chains, which are mostly attached to proteins and lipids. Most soluble secreted proteins are also similarly decorated with such glycans. Sialic acids are a diverse family of sugar units with a nine-carbon backbone that are typic...

    journal_title:Trends in molecular medicine

    pub_type: 杂志文章,评审

    doi:10.1016/j.molmed.2008.06.002

    authors: Varki A

    更新日期:2008-08-01 00:00:00