Abstract:
:In today's world of genomics and large computational analyses, rapid progress has been made in identifying genes associated with human retinal diseases. Nevertheless, before significant advances toward effective therapeutic intervention is made, a clearer understanding of the molecular and cellular role of these gene products in normal and diseased photoreceptor cell biology is required. Given the complexity of the vertebrate retina, these advancements are unlikely to be revealed in isolated human cell lines, but instead, will require the use of numerous model systems. Here, we describe several parallels between vertebrate and invertebrate photoreceptor cell biology that are beginning to emerge and advocate the use of Drosophila melanogaster as a powerful genetic model system for uncovering molecular mechanisms of human retinal pathologies, in particular photoreceptor neurodegeneration.
journal_name
Prog Mol Biol Transl Scijournal_title
Progress in molecular biology and translational scienceauthors
Cook T,Zelhof A,Mishra M,Nie Jdoi
10.1016/B978-0-12-384878-9.00008-Xsubject
Has Abstractpub_date
2011-01-01 00:00:00pages
331-68eissn
1877-1173issn
1878-0814pii
B978-0-12-384878-9.00008-Xjournal_volume
100pub_type
杂志文章,评审abstract::Among the most sensitive, specific and practical of methods for detecting prions are the real-time quaking-induced conversion (RT-QuIC) assays. These assays exploit the fundamental self-propagating activity of prions to amplify the presence of prion seeds by as much as a trillion-fold. The reactions can detect most of...
journal_title:Progress in molecular biology and translational science
pub_type: 杂志文章,评审
doi:10.1016/bs.pmbts.2017.06.003
更新日期:2017-01-01 00:00:00
abstract::The muscular dystrophies (MDs) represent a diverse collection of inherited human disorders, which affect to varying degrees skeletal, cardiac, and sometimes smooth muscle (Emery, 2002). To date, more than 50 different genes have been implicated as causing one or more types of MD (Bansal et al., 2003). In many cases, i...
journal_title:Progress in molecular biology and translational science
pub_type: 杂志文章,评审
doi:10.1016/B978-0-12-394596-9.00004-4
更新日期:2012-01-01 00:00:00
abstract::In eukaryotes, methylation of nucleosomal histones and other nuclear proteins is a central aspect of chromatin structure and dynamics. The past 15 years have seen an enormous advance in our understanding of the biochemistry of these modifications, and of their role in establishing the epigenetic code. We provide a syn...
journal_title:Progress in molecular biology and translational science
pub_type: 杂志文章
doi:10.1016/B978-0-12-387685-0.00004-4
更新日期:2011-01-01 00:00:00
abstract::The control of immune responses against self and nonharmful environmental antigens is of critical importance to the immune homeostasis. Regulatory T (Treg) cells are the key players of such immune regulation and their deficiency and dysfunction are associated with various immune disorders, such as autoimmunity and all...
journal_title:Progress in molecular biology and translational science
pub_type: 杂志文章,评审
doi:10.1016/bs.pmbts.2015.07.011
更新日期:2015-01-01 00:00:00
abstract::The development of chronic lung disease occurs as a consequence of multiple cellular events that involve an initial insult which often leads to the development of chronic inflammation, and the dysregulation of cellular proliferation and cell death mechanisms. Multiple cell types in the lung are key to the respiratory ...
journal_title:Progress in molecular biology and translational science
pub_type: 杂志文章
doi:10.1016/bs.pmbts.2020.02.001
更新日期:2020-01-01 00:00:00
abstract::Pathologic aggregates of tau protein are observed in several neurodegenerative diseases and are used to diagnose and stage disease postmortem. Recent advances in positron emission tomography radioligands allow for the detection of aggregated tau proteins in living persons. This chapter describes the development and ch...
journal_title:Progress in molecular biology and translational science
pub_type: 杂志文章,评审
doi:10.1016/bs.pmbts.2019.07.007
更新日期:2019-01-01 00:00:00
abstract::Guanine nucleotide-binding proteins of the inhibitory (Gi/o) class play critical physiological roles and the receptors that activate them are important therapeutic targets (e.g., mu opioid, serotonin 5HT1a, etc.). Gi/o proteins are negatively regulated by regulator of G protein signaling (RGS) proteins. The redundant ...
journal_title:Progress in molecular biology and translational science
pub_type: 杂志文章,评审
doi:10.1016/bs.pmbts.2015.04.010
更新日期:2015-01-01 00:00:00
abstract::Accumulating evidence showed that the luteinizing hormone/chorionic gonadotropin receptor (LHCGR) is an essential regulator of sexual development and reproduction from zebrafish to human. Activating and inactivating mutations of LHCGR gene have been identified from patients of different phenotypes. Familial male-limit...
journal_title:Progress in molecular biology and translational science
pub_type: 杂志文章,评审
doi:10.1016/bs.pmbts.2018.09.007
更新日期:2019-01-01 00:00:00
abstract::Transmissible spongiform encephalopathies or prion diseases describe a number of different human disorders that differ in their clinical phenotypes, which are nonetheless united by their transmissible nature and common pathology. Clinical variation in the absence of a conventional infectious agent is believed to be en...
journal_title:Progress in molecular biology and translational science
pub_type: 杂志文章
doi:10.1016/bs.pmbts.2020.08.006
更新日期:2020-01-01 00:00:00
abstract::Amyloids are nanoscopic ordered self-assemblies of misfolded proteins that are formed via aggregation of partially unfolded or intrinsically disordered proteins (IDPs) and are commonly linked to devastating human diseases. An enlarging body of recent research has demonstrated that certain amyloids can be beneficial an...
journal_title:Progress in molecular biology and translational science
pub_type: 杂志文章,评审
doi:10.1016/bs.pmbts.2019.05.005
更新日期:2019-01-01 00:00:00
abstract::Matrix metalloproteinases (MMPs) have been implicated in the pathogenesis of aortic aneurysm because the histology of thoracic aortic aneurysm (TAA) and abdominal aortic aneurysm (AAA) is characterized by the loss of smooth muscle cells in the aortic media and the destruction of extracellular matrix (ECM). Furthermore...
journal_title:Progress in molecular biology and translational science
pub_type: 杂志文章,评审
doi:10.1016/bs.pmbts.2017.02.002
更新日期:2017-01-01 00:00:00
abstract::Viruses have evolved to exploit the vast complexity of cellular processes for their success within the host cell. The entry mechanisms of enveloped viruses (viruses with a surrounding outer lipid bilayer membrane) are usually classified as being either endocytotic or fusogenic. Different mechanisms have been proposed ...
journal_title:Progress in molecular biology and translational science
pub_type: 杂志文章,评审
doi:10.1016/bs.pmbts.2014.10.002
更新日期:2015-01-01 00:00:00
abstract::Alpha-fetoprotein (AFP) is a glycoprotein and belongs to the gene family of serum albumins. The serum AFP levels were found to be elevated in the sera of liver cancer patients in 1964 and were subsequently developed and used as a liver cancer biomarker. However, elevated serum AFP levels have been observed in patients...
journal_title:Progress in molecular biology and translational science
pub_type: 杂志文章
doi:10.1016/bs.pmbts.2019.01.001
更新日期:2019-01-01 00:00:00
abstract::The innate immune system senses microbial infections using pattern-recognition receptors and signals to activate adaptive immunity. Type I transmembrane protein Toll-like receptors (TLRs) play important roles in antimicrobial immune responses. Upon the recognition of pathogen-associated molecular patterns, TLRs homo- ...
journal_title:Progress in molecular biology and translational science
pub_type: 杂志文章,评审
doi:10.1016/B978-0-12-386931-9.00018-0
更新日期:2013-01-01 00:00:00
abstract::Longitudinal growth results from multifactorial and complex processes that take place in the context of different genetic traits and environmental influences. Thus, in view of the difficulties in comprehension of the physiological mechanisms involved in the achievement of normal height, our ability to make a definitiv...
journal_title:Progress in molecular biology and translational science
pub_type: 杂志文章,评审
doi:10.1016/bs.pmbts.2015.10.009
更新日期:2016-01-01 00:00:00
abstract::Protein misfolding and aggregation of amyloid proteins is the fundamental cause of more than 20 diseases. Molecular mechanisms of the self-assembly and the formation of the toxic aggregates are still elusive. Computer simulations have been intensively used to study the aggregation of amyloid peptides of various amino ...
journal_title:Progress in molecular biology and translational science
pub_type: 杂志文章
doi:10.1016/bs.pmbts.2019.12.002
更新日期:2020-01-01 00:00:00
abstract::Being responsible for more than 90% of cellular functions, protein molecules are workhorses in all the life forms. In order to cater for such a high demand, proteins have evolved to adopt diverse structures that allow them to perform myriad of functions. Beginning with the genetically directed amino acid sequence, the...
journal_title:Progress in molecular biology and translational science
pub_type: 杂志文章
doi:10.1016/bs.pmbts.2020.06.001
更新日期:2020-01-01 00:00:00
abstract::The failure of neuropathic pain to abate even years after trauma suggests that adverse changes to synaptic function must exist in a chronic pathological state in nociceptive pathways. The chronicity of neuropathic pain therefore underscores the importance of understanding the contribution of dendritic spines--micron-s...
journal_title:Progress in molecular biology and translational science
pub_type: 杂志文章,评审
doi:10.1016/bs.pmbts.2014.12.001
更新日期:2015-01-01 00:00:00
abstract::Alzheimer's disease (AD) is the leading cause of dementia and sixth cause of death in elderly adults. AD poses a huge economic burden on society and constitutes an unprecedented challenge for caregivers and families affected. Aging of the population is projected to drastically aggravate the situation in the near futur...
journal_title:Progress in molecular biology and translational science
pub_type: 杂志文章
doi:10.1016/bs.pmbts.2020.10.002
更新日期:2021-01-01 00:00:00
abstract::Cell membrane-associated junctional complexes mediate cell-cell adhesion, intercellular interactions, and other fundamental processes required for proper embryo morphogenesis. Cadherins are calcium-dependent transmembrane proteins at the core of adherens junctions and are expressed in distinct spatiotemporal patterns ...
journal_title:Progress in molecular biology and translational science
pub_type: 杂志文章,评审
doi:10.1016/B978-0-12-394311-8.00013-3
更新日期:2013-01-01 00:00:00
abstract::Glycated serum protein (GSP, measured as serum fructosamine concentration in μmol/L) is a product of glycation reaction between glucose and serum proteins in the blood circulation. GSP is used along with blood glucose, glycated hemoglobin (HbA1c), and glycated albumin as indicators of glycemic control for diabetic pat...
journal_title:Progress in molecular biology and translational science
pub_type: 杂志文章
doi:10.1016/bs.pmbts.2019.01.007
更新日期:2019-01-01 00:00:00
abstract::Bacteriophage T4 is the archetype of virulent phage. It has evolved very efficient strategies to subvert host functions to its benefit and to impose the expression of its genome. T4 utilizes a combination of host and phage-encoded RNases and factors to degrade its mRNAs in a stage-dependent manner. The host endonuclea...
journal_title:Progress in molecular biology and translational science
pub_type: 杂志文章,评审
doi:10.1016/S0079-6603(08)00802-7
更新日期:2009-01-01 00:00:00
abstract::Since the discovery of specialized T cells with regulatory function, harnessing the power of these cells to ameliorate autoimmunity has been a major goal. Here we collate the evidence that regulatory T cells (Treg) can inhibit Type 1 diabetes in animal models and humans. We discuss the anatomical sites and molecular m...
journal_title:Progress in molecular biology and translational science
pub_type: 杂志文章,评审
doi:10.1016/bs.pmbts.2015.08.004
更新日期:2015-01-01 00:00:00
abstract::The increased prevalence of hyperlipidemia, hypertriglyceridemia, hypercholesterolemia, and fatty liver disease has provided increasingly negative connotations toward lipids. However, it is important to remember that lipids are essential components supporting life. Lipids are a class of molecules defined by their inhe...
journal_title:Progress in molecular biology and translational science
pub_type: 杂志文章,评审
doi:10.1016/bs.pmbts.2015.06.017
更新日期:2015-01-01 00:00:00
abstract::Ribosomal protein S6 kinase (S6K) has been implicated in the phosphorylation of multiple substrates and is subject to activation by a wide variety of signals that converge at mammalian target of rapamycin (mTOR). In the course of the search for its physiological role, it was proposed that S6K activation and ribosomal ...
journal_title:Progress in molecular biology and translational science
pub_type: 杂志文章,评审
doi:10.1016/S1877-1173(09)90003-5
更新日期:2009-01-01 00:00:00
abstract::Neuroinflammation is thought to play a key role in the development and progression of neurodegenerative diseases such as Alzheimer's disease. Positron emission tomography (PET) is an in vivo imaging technique capable of studying the biochemical processes which provide the molecular basis of disease. PET imaging of neu...
journal_title:Progress in molecular biology and translational science
pub_type: 杂志文章,评审
doi:10.1016/bs.pmbts.2019.04.005
更新日期:2019-01-01 00:00:00
abstract::The cellular recycling process of macroautophagy, which is the mechanism by which cellular material is delivered to lysosomes via double membraned vesicles called autophagosomes, is intimately connected to programmed cell death pathways, especially apoptosis. In this article, I discuss some underlying mechanisms and t...
journal_title:Progress in molecular biology and translational science
pub_type: 杂志文章
doi:10.1016/bs.pmbts.2020.04.023
更新日期:2020-01-01 00:00:00
abstract::The self-assembling protein shells of viruses have provided convenient scaffolds for the construction of many new materials with well-defined nanoscale architectures. In some cases, the native amino acid functional groups have served as nucleation sites for the deposition of metals and semiconductors, leading to organ...
journal_title:Progress in molecular biology and translational science
pub_type: 杂志文章,评审
doi:10.1016/B978-0-12-415906-8.00002-9
更新日期:2011-01-01 00:00:00
abstract::Cadherin-based cell-cell adhesions are dynamic structures that mediate tissue organization and morphogenesis. They link cells together, mediate cell-cell recognition, and influence cell shape, motility, proliferation, and differentiation. At the cellular level, operation of classical cadherin adhesion systems is coord...
journal_title:Progress in molecular biology and translational science
pub_type: 杂志文章,评审
doi:10.1016/B978-0-12-394311-8.00003-0
更新日期:2013-01-01 00:00:00
abstract::Insulin resistance plays a key role in the development of type 2 diabetes mellitus and is also associated with several other diseases, such as obesity, hypertension, and cardiovascular diseases. Type 2 diabetes and obesity have become epidemic worldwide in the past few decades, and epidemiological and metabolic eviden...
journal_title:Progress in molecular biology and translational science
pub_type: 杂志文章,评审
doi:10.1016/B978-0-12-800101-1.00008-9
更新日期:2014-01-01 00:00:00