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Monoclonal Antibodies Used for the Diagnosis of the Small Round Cell Tumors of Childhood
Published in John T. Kemshead, Pediatric Tumors: Immunological and Molecular Markers, 2020
J.T. Kemshead, J. Clayton, K. Patel
Intermediate filament proteins can be divided into five different groups, namely vimentin, desmin, cytokeratin, glial fibrillary acidic protein (GFAP) and neurofilaments. Within these groups subdivisions have been made. For example many different forms of cytokeratins have been identified. The precise function of intermediate filament proteins has to be elucidated, but it is thought that they play a structural role in the integrity of the cell. These proteins are composed of polymorphic subunits, and the expression of a particular intermediate filament type is restricted to a subset of tissues. In general the specific pattern of intermediate filament expression is maintained during transformation making the proteins useful as markers for malignant disease.
The Human Anti-DNA Idiotype (16/6 Idiotype)
Published in Thomas F. Kresina, Monoclonal Antibodies, Cytokines, and Arthritis, 2020
Howard Amital-Teplizki, Yehuda Shoenfeld
The cytoskeletal structure we studied belonged to the intermediate filament subclasses, which probably anchor the nucleus to the cytoplasm, yet their precise role has not been completely elucidated. Several types of intermediate filaments have been described according to their cellular origin: vimentin in mesenchymal cells, desmin in muscular cells, neurofilaments in neurons, and others (14). All types bear a certain resemblance when compared by electron microscopy, X-ray diffraction, and amino acid sequencing (15).
Structural Organization of the Liver
Published in Robert G. Meeks, Steadman D. Harrison, Richard J. Bull, Hepatotoxicology, 2020
Intermediate filaments are a family of morphologically similar protein fibers with a diameter of 8–10 nm, which is intermediate between that of actin fibers (6 nm) and myosin fibers (15 nm). They are present throughout the cytoplasm, but are more numerous in the regions of the cell subjected to mechanical stress. Although their function is not known, they are thought to serve as scaffolding within the cytoplasm and as “mechanical integrater of cellular space” by providing support for actin filament/microtubule systems (Lazarides, 1980). Individual polypeptide subunits comprising intermediate filaments are arranged in an α-helical coil, which provides the filaments with high tensile strength and flexibility (Steinert, 1978). Although intermediate filaments are similar structurally, they are heterogeneous in their subunit composition and antigenicity. They are of five major types: cytokeratin, vimentin, desmin, glial fibrillary acidic protein, and neurofilament triplet protein (Wang et al., 1985). Cytokeratins are characteristic of epithelial cells including hepatocytes (Moll et al., 1982). They undergo alterations in their antigenicity as the differentiation of epithelial cells proceeds, so that epithelial cells can be classified according to the expression of their cytokeratin polypeptides (Sun et al., 1985).
Data-independent acquisition mass spectrometry reveals comprehensive plasma protein profiles in the natural history of patients with hereditary transthyretin amyloidosis (ATTRv)
Published in Expert Review of Proteomics, 2023
Shan He, XinYue He, RuoKai Pan, LuRong Pan, Xiaoying Lv, YuTong Jin, Yue Fan, YuTong Wang, Zhuang Tian, ShuYang Zhang
GO enrichment analysis is shown in Figure 2, and the top 10 GO function entries were identified based on their significance represented by –log10 p values in the three GO types. Figure 2a shows 30 DEPs between the ATTRv-PN and control groups. GO enrichment analysis showed that cornification, keratinization, phagocytosis, and programmed cell death were the most significant biological processes. Intermediate filaments, intermediate filament cytoskeletons, keratin filaments, and polymeric cytoskeletal fibers were the most significant cellular components. The structural constituents of the skin epidermis, hormone activity, and receptor regulator activity were the most significant molecular functions. KEGG pathway enrichment analysis (Figure 2b) showed the estrogen signaling pathway and cell adhesion molecule (CAM) pathway as the two pathways with a higher number of differentially expressed proteins and more reliable enrichment significance in ATTRv-PN.
Naxos disease – a narrative review
Published in Expert Review of Cardiovascular Therapy, 2020
Marianna Leopoulou, Gustav Mattsson, Jo Ann LeQuang, Joseph V Pergolizzi, Giustino Varrassi, Marita Wallhagen, Peter Magnusson
Carvajal syndrome was described by Carvajal-Huerta and may be considered a variant of Naxos syndrome [34]. It seems to be most prevalent in Ecuador and India. Compared to Naxos disease, it manifests at a younger age, even during childhood, and the involvement of the left ventricle, including dilatation, is common [9,12]. Notably, the affected skin in the Carvajal-Huerta series of patients presents with epidermolytic keratoderma [34]. The underlying genetic cause refers to a mutation that truncates the intermediate filament-binding site of desmoplakin. It is associated with early morbidity [6]. In addition, homozygous mutations in genes that encode the proteins plakoglobin and desmocollin-2 have been associated with Carvajal syndrome [13]. Interestingly, histological findings in a case Carvajal syndrome lacked the fatty component in myocardial replacement process seen in Naxos disease [28].
Synaptic remodeling, lessons from C. elegans
Published in Journal of Neurogenetics, 2020
Andrea Cuentas-Condori, David M. Miller, 3rd
Most MTs in DD neurons adopt the ‘plus-end out’ orientation both before and after remodeling (Figure 6; Kurup et al., 2015). ‘Plus-end’ refers to the MT end to which more α and β tubulin dimers are added during MT growth and removed during MT shrinkage (Baas & Lin, 2011). The resultant ‘dynamic instability’ of MTs is characteristically elevated during cell biological events (e.g. cell division) in which the MT cytoskeleton is actively reorganized (Gardner, Zanic, & Howard, 2013). Several lines of evidence indicate that DD remodeling depends on MT dynamics. First, genetic mutations that stabilize MTs block DD remodeling and this effect can be partially relieved by treatment with the MT depolymerizing drug, nocodazole (Kurup et al., 2015). Second, factors that regulate the transition from MT growth to shrinkage (i.e. ‘catastrophe’) such as the conserved kinase, DLK-1, and MT associated proteins, Kinesin-3/KLP-7 and Spastin/SPAS-1, promote DD remodeling (Kurup et al., 2015). Third, the MT stabilizing role of intermediate filaments antagonizes remodeling (Figure 6(D,F); Kurup, Li, Goncharov, & Jin, 2018). A role for DLK-1 in synaptic remodeling is notable because DLK-1 also promotes axon regeneration in a cell biological mechanism that drives MT growth (Ghosh-Roy, Goncharov, Jin, & Chisholm, 2012). Additional unknown factors are likely required for activating MT dynamics in DD neurons, however, because a genetic ablation of DLK-1 activity results in only a slight delay in synaptic remodeling (Kurup et al., 2015).