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Nuclear bodies in neurodegenerative disease.

Neurodegenerative diseases are characterized by a relentlessly progressive loss of the functional and structural integrity of the central ne … ses. Co-ordinated nuclear function is predicated on the activity of distinct nuclear subdomains, or nuclear bodies, each responsible for a specific function. If nuclear dysfunction represents an important etiopathological feature in neurodegenerative disease, then this should be reflected by functional and/or morphological alterations in this nuclear compartment …

Measuring the Proximity of Nuclear lncRNAs to Nuclear Bodies.

Long noncoding RNAs (lncRNAs) are classified as RNAs longer than 200 nucleotides, which do not code for proteins. The precise functions of lncRNAs in the cell are not always well understood, but it is believed that they often localize to their site of action. Some lncRNAs can associate with nuclear bodies under different conditions, and so it is of interest to measure lncRNA localization in relation to different nuclear bodies under different conditions. In this protocol, we describe a method by which to measure the relative intensity of a specific RNA in the vicinity of nuclear bodies known a…

A monoclonal antibody recognizing nuclear matrix-associated nuclear bodies.

We have isolated a monoclonal antibody, 5E10, that labels discrete spots in the interphase nucleus. By immunoblotting mAb 5E10 recognized pr … e antigen is localized in 0.3 microns diameter spherical, electron-dense structures, reminiscent of nuclear bodies. Double-labeling experiments showed that these spots do not colocalize with U1 snRNPs and centromeres. The spots did colocalize with nuclear speckles recognized by a primary biliary cirrhosis autoimmune serum, which is thought to recognize nuclear b …

Local translation in nuclear condensate amyloid bodies.

Biomolecular condensates concentrate molecules to facilitate basic biochemical processes, including transcription and DNA replication. While liquid-like condensates have been ascribed various functions, solid-like condensates are generally thought of as amorphous sites of protein storage. Here, we show that solid-like amyloid bodies coordinate local nuclear protein synthesis (LNPS) during stress. On stimulus, translationally active ribosomes accumulate along fiber-like assemblies that characterize amyloid bodies. Mass spectrometry analysis identified regulatory ribosomal proteins and translati

Structural and functional heterogeneity of nuclear bodies.

The nuclear body is a cellular structure that appears to be involved in the pathogenesis of acute promyelocytic leukemia and viral infection … infected with the adenovirus expressing Sp140 and incubated with IFN-gamma, the number of PML-Sp100 nuclear bodies per cell increased but immunoreactive Sp140 was not evenly distributed among the nuclear bodies. Sp140 associated with a subset of IFN-gamma-induced PML-Sp100 nuclear bodies. To examine the potential effect of Sp140 on gene transcription, a plasmid …

Nuclear bodies in the caecum of the rabbit.

The structure and cytochemistry of nuclear bodies in the caecum of the rabbit are described and a developmental pathway from morphologically simple to complex forms is proposed. Simple nuclear bodies are proteinaceous in nature while complex bodies possess a proteinaceous capsule with some associated ribonucleoprotein. The interna of complex bodies possess various individual components consisting either of deoxyribonucleoprotein, ribonucleoprotein, or unassociated proteins. The significance of these nuclear bodies in caecal biology is discussed.

Nuclear bodies in Creutzfeldt-Jakob and Alzheimer's diseases.

Nuclear bodies types I, II, III, and IV, as described by Bouteille, Kalifat, and Delarue, have been found in one case of Creutzfeldt-Jakob disease and one case of Alzheimer's disease. The relationship of nuclear bodies to viral disease in the central nervous system is not clear.

Discovery of myeloid zinc finger (MZF) 1 nuclear bodies.

Myeloid zinc finger 1 (MZF1) is a multifaceted transcription factor that can act either as a transcriptional activator or a gene repressor. We examined its production of nuclear bodies (NBs) and subcellular localization. Proteomic and protein-protein interaction analysis were used to identify its cofactors and interactions. These revealed the presence of MZF1-NBs (intranuclear oligomers containing MZF1). MZF-NBs are similar to some other nuclear bodies, notably promyelocytic leukemia (PML) -NBs in terms of size and morphology. However the two structures appear to be different. MZF-NBs and PML-…

Nuclear p62 condensates stabilize the promyelocytic leukemia nuclear bodies by sequestering their ubiquitin ligase RNF4.

Liquid-liquid phase separation has emerged as a crucial mechanism driving the formation of membraneless biomolecular condensates, which play important roles in numerous cellular processes. These condensates, found both in the nucleus and cytoplasm, are formed through multivalent, low-affinity interactions between various molecules. P62-containing condensates serve, among other functions, as proteolytic hubs for the ubiquitin-proteasome system. In this study, we investigated the dynamic interplay between nuclear p62 condensates and promyelocytic nuclear bodies (PML-NBs). We show that p62 conden…

Hepatitis C-associated liver carcinogenesis: role of PML nuclear bodies.

Successful escape from immune response characterises chronic hepatitis C virus (HCV) infection, which results in persistence of infection in … nt cellular processes like differentiation and apoptosis. In cancer biology, PML and its associated nuclear bodies (NBs) have initially attracted intense interest due to its role in the pathogenesis of acute promyelocytic leukemia (APL). More recently, loss of PML has been implicated in human cancers of various histologic origins. Moreover, number and intensity …

Regulation of neuronal differentiation by proteins associated with nuclear bodies.

Nuclear bodies are large sub-nuclear structures composed of RNA and protein molecules. The Survival of Motor Neuron (SMN) protein localizes to Cajal bodies (CBs) and nuclear gems. Diminished cellular concentration of SMN is associated with the neurodegenerative disease Spinal Muscular Atrophy (SMA). How nuclear body architecture and its structural components influence neuronal differentiation remains elusive. In this study, we analyzed the effects of SMN and two of its interaction partners in cellular models of neuronal differentiation. The nuclear 23 kDa isoform of Fibroblast Growth Factor - …

Targeting SMN to Cajal bodies and nuclear gems during neuritogenesis.

Neurite outgrowth is a central feature of neuronal differentiation. PC12 cells are a good model system for studying the peripheral nervous system and the outgrowth of neurites. In addition to the dramatic changes observed in the cytoplasm, neuronal differentiation is also accompanied by striking changes in nuclear morphology. The large and sustained increase in nuclear transcription during neuronal differentiation requires synthesis of a large number of factors involved in pre-mRNA processing. We show that the number and composition of the nuclear subdomains called Cajal bodies and gems change

Fibroblast growth factor-2 regulates the stability of nuclear bodies.

Nuclear bodies are distinct subnuclear structures. The survival of motoneuron (SMN) gene is mutated or deleted in patients with the neurodegenerative disease spinal muscular atrophy (SMA). The gene product SMN is a marker protein for one class of nuclear bodies denoted as nuclear gems. SMN has also been found in Cajal bodies, which co-localize with gems in many cell types. Interestingly, SMA patients display a reduced number of gems. Little is known about the regulation of nuclear body formation and stabilization. We have previously shown that a nuclear isoform of the fibroblast growth factor-…

Absence of gemin5 from SMN complexes in nuclear Cajal bodies.

BACKGROUND: Spinal muscular atrophy is caused by reduced levels of the survival of motor neurons (SMN) protein. SMN is found in large complexes with Sm proteins and at least eight other proteins, including seven "gemins". These complexes are involved in the assembly of snRNPs in the cytoplasm and their transport into the nucleus, but the precise roles of the individual protein components are largely unknown. RESULTS: We have investigated the subcellular distribution of gemins using novel antibodies against gemins 3-7, and existing mAbs against SMN, gemin2, unrip, fibrillarin and profilin II.

Nuclear domain 10 (ND10) associated proteins are also present in nuclear bodies and redistribute to hundreds of nuclear sites after stress.

The promyelocytic leukemia protein fused to the retinoic acid receptor alpha in t(15;17) acute promyelocytic leukemia, the primary biliary cirrhosis autoantigen, Sp100, as well as the incompletely characterized protein NDP55, are co-localized in specific immunohistochemically defined nuclear domains (ND10), which are potential equivalents of ultrastructurally defined nuclear bodies. We investigated whether the distribution of these proteins depends on environmental conditions and whether ND10 correlate with nuclear bodies. Certain nuclear bodies and ND10 react in a similar way and share antige…

Интерплей между герпесвирусными инфекциями и защитой приёма ядерными телами PML.

В ходе недавних исследований мы и другие идентифицировали клеточные белки PML, hDaxx и Sp100, которые образуют субядерное устройство, известное как ядерный домен 10 (ND10) или ядерные тела PML (PML-NBs), в качестве факторов ограничения содержания, которые противодействуют герпесвирусным инфекциям и препятствуют распространению вирусов на различных этапах. Однако антивирусная функция ND10 антагонизируется вирусными регуляторными белками (например, PIP0 вируса герпеса simpex; IE1 цитомегаловируса человека), которые вызывают либо модификацию, либо нарушение ND10. Этот обзор резюмирует текущие зна

Совпадение в аннотации оригинала
In recent studies we and others have identified the cellular proteins PML, hDaxx, and Sp100, which form a subnuclear structure known as nuclear domain 10 (ND10) or PML nuclear bodies (PML-NBs), as host restriction factors that counteract herpesviral infections by inhibiting viral replication at different stages. The antiviral function of ND10, however, is an…

Double-edged role of PML nuclear bodies during human adenovirus infection.

PML nuclear bodies are matrix-bound nuclear structures with a variety of functions in human cells. These nuclear domains are interferon regulated and play an essential role during virus infections involving accumulation of SUMO-dependent host and viral factors. PML-NBs are targeted and subsequently manipulated by adenoviral regulatory proteins, illustrating their crucial role during productive infection and virus-mediated oncogenic transformation. PML-NBs have a longstanding antiviral reputation; however, the genomes of Human Adenoviruses and initial sites of viral transcription/replication ar…