Fragile X syndrome, the Fragile X related proteins, and animal models.

Abstract:

:The Fragile X syndrome (FraX), which is characterized among other physical and neurologic impairments by mental retardation, is caused by the absence of the product of the FMR1 gene. The Fragile X Mental Retardation Protein (FMRP) is a member of a novel family of RNA-binding proteins. The latter includes two other proteins highly homologous with FMRP: the fragile X related proteins 1 and 2 (FXRP1 and FXRP2). Characterization of FXRPs, including their interaction with FMRP, will provide critical information about the mechanisms of action of FMRP and the role of this group of proteins in FMRP-deficient conditions such as FraX. Genetic manipulations of FMRP and the FXRPs should also provide valuable tools for investigating pathophysiology and gene therapies in FraX. The present review summarizes the strategies used for identifying the FXRPs, their chromosomal localization, molecular structure, and tissue distribution. It also reviews interactions between different members of this family of RNA-binding proteins. Animal models, both knockout and transgenic, of FMRP and the FXRPs are discussed. Phenotypic features of the FMR1 knockout mouse, the FMR1 transgenic rescue mouse, and other novel strategies for manipulating and delivering FMRP and FXRPs to the brain and other tissues are described.

journal_name

Microsc Res Tech

authors

Hoogeveen AT,Willemsen R,Oostra BA

doi

10.1002/jemt.10064

keywords:

subject

Has Abstract

pub_date

2002-05-01 00:00:00

pages

148-55

issue

3

eissn

1059-910X

issn

1097-0029

journal_volume

57

pub_type

杂志文章,评审
  • Light-triggered Janus nanomotor for targeting and photothermal lysis of pathogenic bacteria.

    abstract::The rapid photothermal lysis of Escherichia coli O157:H7 treated with light-triggered Janus nanomotors was visualized by Hilbert differential contrast transmission electron microscopy (HDC-TEM). The extraordinary advantage of this high-resolution microscopic technique was that it revealed the detailed ultrastructure a...

    journal_title:Microscopy research and technique

    pub_type: 杂志文章

    doi:10.1002/jemt.23657

    authors: Loukanov A

    更新日期:2020-11-27 00:00:00

  • Development of the vomeronasal receptor epithelium and the accessory olfactory bulb in sheep.

    abstract::The morphological development of the vomeronasal organ (VNO) and accessory olfactory bulb (AOB) of the sheep from anlage to birth were studied by classical and histochemical methods using embryos and fetuses obtained from an abattoir with ages estimated from crown-to-rump length. Both VNO and AOB developed in a biolog...

    journal_title:Microscopy research and technique

    pub_type: 杂志文章

    doi:10.1002/jemt.10362

    authors: Salazar I,Lombardero M,Alemañ N,Sánchez Quinteiro P

    更新日期:2003-08-01 00:00:00

  • In situ hybridization in HIV research.

    abstract::In situ hybridization (ISH) for HIV is an arduous, demanding means of detecting viral genetic material in cells and tissues. Good ISH requires broad technical skills and devotion to controls for every step of the process as well as a critical eye when interpreting results. ISH may be used to detect HIV in three ways: ...

    journal_title:Microscopy research and technique

    pub_type: 杂志文章,评审

    doi:10.1002/jemt.1070250111

    authors: Fox CH,Cottler-Fox M

    更新日期:1993-05-01 00:00:00

  • Stress and strain distribution in demineralized enamel: A micro-CT based finite element study.

    abstract::Physiological oral mechanical forces may play a role on the progression of enamel carious lesions to cavitation. Thus, the aim of this study was to describe, by 3D finite element analysis, stress, and strain patterns in sound and carious enamel after a simulated occlusal load. Micro-CT based models were created and me...

    journal_title:Microscopy research and technique

    pub_type: 杂志文章

    doi:10.1002/jemt.22549

    authors: Neves AA,Coutinho E,Alves HD,de Assis JT

    更新日期:2015-10-01 00:00:00

  • The effect of various asthma medications on surface roughness of pediatric dental restorative materials: An atomic force microscopy and scanning electron microscopy study.

    abstract::The aim of the study is to examine and compare the effects of various inhaled asthma medications (IAMs) on the surface roughness of dental restorative materials (DRMs). In total, 192 samples were prepared including 32 samples for the each material group from six different DRMs [Nanohybrid composite (Filtek Z550), Nano...

    journal_title:Microscopy research and technique

    pub_type: 杂志文章

    doi:10.1002/jemt.23584

    authors: Candan M,Ünal M

    更新日期:2021-02-01 00:00:00

  • Image signal-to-noise ratio estimation using Shape-Preserving Piecewise Cubic Hermite Autoregressive Moving Average model.

    abstract::We propose to cascade the Shape-Preserving Piecewise Cubic Hermite model with the Autoregressive Moving Average (ARMA) interpolator; we call this technique the Shape-Preserving Piecewise Cubic Hermite Autoregressive Moving Average (SP2CHARMA) model. In a few test cases involving different images, this model is found t...

    journal_title:Microscopy research and technique

    pub_type: 杂志文章

    doi:10.1002/jemt.20610

    authors: Sim KS,Wee MY,Lim WK

    更新日期:2008-10-01 00:00:00

  • Acquisition of volume regulatory response of sperm upon maturation in the epididymis and the role of the cytoplasmic droplet.

    abstract::In certain cases of infertility in domestic species, and in the homozygous c-ros tyrosine kinase knockout mouse, males are infertile as a result of unopposed sperm swelling. This induces flagellar angulation, preventing their normal migration in the female tract. Angulation always occurs at the site of the cytoplasmic...

    journal_title:Microscopy research and technique

    pub_type: 杂志文章,评审

    doi:10.1002/jemt.10314

    authors: Cooper TG,Yeung CH

    更新日期:2003-05-01 00:00:00

  • Ultrastructural and functional connectivity of intracellularly stained neurones in the vertebrate retina: correlative analyses.

    abstract::A variety of intracellular recording and staining techniques has been used to establish structure-function and, in some cases, structure-function-neurochemical correlations in fish, turtle, and cat retinae. Cone photoreceptor-horizontal cell connectivity has been studied extensively in the cyprinid fish retina by intr...

    journal_title:Microscopy research and technique

    pub_type: 杂志文章

    doi:10.1002/jemt.1070240106

    authors: Djamgoz MB,Kolb H

    更新日期:1993-01-01 00:00:00

  • A novel method for transmission electron microscopy study of cytoplasmic fragments from preimplantation human embryos.

    abstract::Transmission electron microscopy (TEM) is the main tool for exploring the intracellular damage and organelle distribution. The cause of producing embryo cytoplsamic fragmentation is not completely understood. Since the fragments have detrimental effects on embryo development, the ultrastructural analysis of fragments ...

    journal_title:Microscopy research and technique

    pub_type: 杂志文章

    doi:10.1002/jemt.22657

    authors: Halvaei I,Khalili MA,Nottola SA

    更新日期:2016-06-01 00:00:00

  • Imaging molecular structure and physiological function of gap junctions and hemijunctions by multimodal atomic force microscopy.

    abstract::Gap junctions are specialized plasma membrane structures that join neighboring cells via specialized intercellular ion channels (hemichannels) and provide a direct pathway for cell-cell communication. They presumably mediate regulation of growth, transmission of developmental signals, coordination of muscle contractio...

    journal_title:Microscopy research and technique

    pub_type: 杂志文章,评审

    doi:10.1002/1097-0029(20010201)52:3<273::AID-JEMT1013>

    authors: Lal R,Lin H

    更新日期:2001-02-01 00:00:00

  • Intramandibular glands in different castes of leaf-cutting ant, Atta laevigata (Fr. Smith, 1858) (Formicidae: Attini).

    abstract::Intramandibular glands have been poorly studied in polymorphic ants, where the differences between castes were unsufficiently scrutinized. Leaf-cutting ants possess one of the most complex systems of communication and labor division, which is polymorphic well as age polyethism, and makes them an ideal model for the st...

    journal_title:Microscopy research and technique

    pub_type: 杂志文章

    doi:10.1002/jemt.22516

    authors: Martins LC,Della Lucia TM,Gonçalves WG,Delabie JH,Zanuncio JC,Serrão JE

    更新日期:2015-07-01 00:00:00

  • Peripheral and central structures involved in insect gustation.

    abstract::Studies in insect gustation have a long history in general physiology, particularly with work on fly labellar and tarsal sensilla and in the general field of insect-plant interactions, where work on immature Lepidoptera and chrysomelid beetles has been prominent. Much more emphasis has been placed on the physiological...

    journal_title:Microscopy research and technique

    pub_type: 杂志文章,评审

    doi:10.1002/(SICI)1097-0029(19991215)47:6<401::AID-JEM

    authors: Mitchell BK,Itagaki H,Rivet MP

    更新日期:1999-12-15 00:00:00

  • Molecular pathology and pathogenesis of inclusion-body myositis.

    abstract::We summarize the molecular phenotype, diagnostic criteria, and the newest advances related to seeking the pathogenic mechanism(s) of sporadic inclusion-body myositis (s-IBM), a muscle disease usually of persons over age 50. On the basis of our research, several processes seem to be important in relation to the still-s...

    journal_title:Microscopy research and technique

    pub_type: 杂志文章,评审

    doi:10.1002/jemt.20186

    authors: Askanas V,Engel WK

    更新日期:2005-07-01 00:00:00

  • Mirror lenses in light microscopy--theoretical considerations and practical implications.

    abstract::Various types of mirror lenses were developed some decades ago designed for several special tasks in microscopy. When compared with high-end glass lenses, mirror lenses lead to an extraordinary image quality because of their supraapochromatic color fidelity and their high planarity; the working distances are significa...

    journal_title:Microscopy research and technique

    pub_type: 杂志文章

    doi:10.1002/jemt.20809

    authors: Piper J

    更新日期:2010-07-01 00:00:00

  • Immunocytochemical investigations of sodium channels along nodal and internodal portions of demyelinated axons.

    abstract::Voltage-gated sodium channels are largely localized to the nodes of Ranvier in myelinated axons, providing the physiological basis for saltatory conduction. Studies using antisodium channel antibodies have shown that along demyelinated axons sodium channels form new distributions. The nature of this changed distributi...

    journal_title:Microscopy research and technique

    pub_type: 杂志文章,评审

    doi:10.1002/(SICI)1097-0029(19960801)34:5<445::AID-JEM

    authors: England JD,Levinson SR,Shrager P

    更新日期:1996-08-01 00:00:00

  • Unique dentition of lungfish.

    abstract::Lungfish are a group of ancient fish, represented almost continuously in the fossil record from their first appearance in the Devonian to the present time. They have numerous unique characters in the dentition, found only among lungfish, as well as some that are shared with other groups of fish and with higher vertebr...

    journal_title:Microscopy research and technique

    pub_type: 杂志文章

    doi:10.1002/jemt.10221

    authors: Kemp A

    更新日期:2002-12-01 00:00:00

  • Ultrastructural alterations in liver of mice exposed chronically and transgenerationally to aqueous extract of betel nut: Implications in betel nut-induced carcinogenesis.

    abstract::The aqueous extract of betel nut (AEBN) induces the formation of preneoplastic nodules in the liver of Swiss Albino mice and leads to increased predisposition to cancer when administered transgenerationally. The aim of this investigation was to elucidate the alterations in ultrastructure of subcellular organelles in t...

    journal_title:Microscopy research and technique

    pub_type: 杂志文章

    doi:10.1002/jemt.20791

    authors: Choudhury Y,Sharan RN

    更新日期:2010-05-01 00:00:00

  • The effect of final irrigation with MTAD, QMix, and EDTA on smear layer removal and mineral content of root canal dentin.

    abstract::To compare the smear layer removal ability and mineral content of root canal dentine after initial irrigation with NaOCl and final irrigation with MTAD, QMix, and 17% EDTA. Forty extracted human maxillary incisors before root canal preparation and irrigation with NaOCl were randomly divided into four groups (n = 10) a...

    journal_title:Microscopy research and technique

    pub_type: 杂志文章

    doi:10.1002/jemt.23239

    authors: Nogo-Živanović D,Kanjevac T,Bjelović L,Ristić V,Tanasković I

    更新日期:2019-06-01 00:00:00

  • Sexual dimorphism in antennal morphology and sensilla ultrastructure of Dendrolimus tabulaeformis Tsai et Liu (Lepidoptera: Lasiocampidae).

    abstract::Insects rely heavily on olfaction to locate habitat, mates, and oviposition sites, while odorant molecules and the antennal olfactory sensory cells of insects are two indispensable components of olfactory response. Our previous work identified the sex pheromones and volatile compounds derived from host plant of Dendro...

    journal_title:Microscopy research and technique

    pub_type: 杂志文章

    doi:10.1002/jemt.22134

    authors: Zhang S,Zhang Z,Kong X,Wang H

    更新日期:2013-01-01 00:00:00

  • Hindbrain floor plate of the rat: ultrastructural changes occurring during development.

    abstract::Most of the molecular and experimental studies on the floor plate (FP) have been performed on the FP region extending along the spinal cord. However, little is known about the hindbrain FP. The FP undergoes regional and temporal changes throughout development, but information with respect to the ultrastructural correl...

    journal_title:Microscopy research and technique

    pub_type: 杂志文章

    doi:10.1002/1097-0029(20010301)52:5<615::AID-JEMT1045>

    authors: del Brio MA,Riera P,Peruzzo B,Rodríguez EM

    更新日期:2001-03-01 00:00:00

  • Assessment of mold and yeast in some bakery products of Lahore, Pakistan based on LM and SEM.

    abstract::The present investigation was designed to throw light on the microbial status of bakery products available in bakeries and supermarkets of Lahore. Different bakery samples such as biscuits, pizza, patties were collected from different localities such as Anar Kali, Chauburji, Faisal Town, Iqbal Town, Model Town, Muslim...

    journal_title:Microscopy research and technique

    pub_type: 杂志文章

    doi:10.1002/jemt.23103

    authors: Saeed I,Shaheen S,Hussain K,Khan MA,Jaffer M,Mahmood T,Khalid S,Sarwar S,Tahir A,Khan F

    更新日期:2019-02-01 00:00:00

  • Plasmodium species aware based quantification of malaria parasitemia in light microscopy thin blood smear.

    abstract::Malaria is a serious worldwide disease, caused by a bite of a female Anopheles mosquito. The parasite transferred into complex life round in which it is grown and reproduces into the human body. The detection and recognition of Plasmodium species are possible and efficient through a process called staining (Giemsa). T...

    journal_title:Microscopy research and technique

    pub_type: 杂志文章

    doi:10.1002/jemt.23269

    authors: Abbas N,Saba T,Rehman A,Mehmood Z,Javaid N,Tahir M,Khan NU,Ahmed KT,Shah R

    更新日期:2019-07-01 00:00:00

  • Evaluating the lucrative role of probiotics in the aquaculture using microscopic and biochemical techniques.

    abstract::The present study focused to elucidate the promising role of probiotics in the fish culture, by employing microscopic and biochemical parameters to rationalize the possible outcomes. After preliminary feeding trial histopathological analysis revealed clear and visible changes in the intestinal and liver cells of CTRL ...

    journal_title:Microscopy research and technique

    pub_type: 杂志文章

    doi:10.1002/jemt.23416

    authors: Noor Z,Noor M,Khan I,Khan SA

    更新日期:2020-03-01 00:00:00

  • Research on the drag reduction mechanism of antlion (Myrmeleon sagax) larvae nonsmooth structural surface.

    abstract::Antlion (Myrmeleon sagax) larvae live in sandy soil and possess the ability to enter soil quickly. In this article, the hierarchical structure of the nonsmooth surface of antlion larvae was obtained using a scanning electron microscope (SEM). Based on the results, a bionic nonsmooth structure model was established to ...

    journal_title:Microscopy research and technique

    pub_type: 杂志文章

    doi:10.1002/jemt.23419

    authors: Tong J,Wu B,Song Z,Gao Z,Sun J,Ma Y,Zhuang J

    更新日期:2020-04-01 00:00:00

  • CaBPs and other immunohistochemical markers of the human vomeronasal system: a comparison with other mammals.

    abstract::After more than two centuries of almost sporadic inquiry as to the existence and function of the human vomeronasal system (VNS), the last decade has seen a resurgent interest in it. The principal question vexing many laboratories is whether adult humans retain the VNS that clearly develops during fetal growth. Additio...

    journal_title:Microscopy research and technique

    pub_type: 杂志文章,评审

    doi:10.1002/(SICI)1097-0029(19980615)41:6<530::AID-JEM

    authors: Johnson EW

    更新日期:1998-06-15 00:00:00

  • Micro-scale chromophore-assisted laser inactivation of nerve growth cone proteins.

    abstract::Directed growth cone movement is crucial for the correct wiring of the nervous system. This movement is governed by the concerted actions of cell surface receptors, signaling proteins, cytoskeleton-associated molecules, and molecular motors. In order to investigate the molecular basis of growth cone motility, we appli...

    journal_title:Microscopy research and technique

    pub_type: 杂志文章,评审

    doi:10.1002/(SICI)1097-0029(20000115)48:2<97::AID-JEMT

    authors: Buchstaller A,Jay DG

    更新日期:2000-01-15 00:00:00

  • Light adaptation affects synaptic vesicle density but not the distribution of GABAA receptors in goldfish photoreceptor terminals.

    abstract::GABA is a likely feedback transmitter from H1 horizontal cells to cone photoreceptors in fish retinas. Spinules arise from H1 cell dendrites in light-adapted retinas, are correlated with responses attributed to feedback, and have been proposed to be the GABA release sites. We used mAb 62-3G1, an antibody against the b...

    journal_title:Microscopy research and technique

    pub_type: 杂志文章

    doi:10.1002/(SICI)1097-0029(19970101)36:1<43::AID-JEMT

    authors: Yazulla S,Studholme KM

    更新日期:1997-01-01 00:00:00

  • Red pigments and Boraginaceae leaves in mortuary ritual of late Neolithic China: A case study of Shengedaliang site.

    abstract::Investigation of mortuary ritual is an important method to reconstruct many aspects of past societies. Due to the lack of relevant analytical work, little evidence related to organic materials in a burial can be found in China. Here we report materials collected from a burial during the excavation of the Shengedaliang...

    journal_title:Microscopy research and technique

    pub_type: 历史文章,杂志文章

    doi:10.1002/jemt.22791

    authors: Wu Y,Guo X,Wang W,Chen X,Zhao Z,Xia X,Yang Y

    更新日期:2017-02-01 00:00:00

  • Effect of age on the structure of Meissner corpuscles in murine digital pads.

    abstract::A light and electron microscopic study was performed to determine age changes in Meissner corpuscles. In forepaw digital pads of mice aged to their maximum life expectancy, corpuscles were found to increase in size and complexity until middle age, and then to become smaller, disorganized and lobulated with more advanc...

    journal_title:Microscopy research and technique

    pub_type: 杂志文章

    doi:10.1002/(SICI)1097-0029(19960701)34:4<376::AID-JEM

    authors: Nava PB,Mathewson RC

    更新日期:1996-07-01 00:00:00

  • The comparison between force volume and peakforce quantitative nanomechanical mode of atomic force microscope in detecting cell's mechanical properties.

    abstract::Atomic force microscope (AFM) has been widely used in the biological field owing to its high sensitivity (subnanonewton), high spatial resolution (nanometer), and adaptability to physiological environments. Nowadays, force volume (FV) and peakforce quantitative nanomechanical (QNM) are two distinct modes of AFM used i...

    journal_title:Microscopy research and technique

    pub_type: 杂志文章

    doi:10.1002/jemt.23351

    authors: Yang Y,Xiao X,Peng Y,Yang C,Wu S,Liu Y,Yue T,Pu H,Liu N,Jiang H

    更新日期:2019-11-01 00:00:00