Int J Mol Sci
February 2024
Loss of function of members of the muscleblind-like (MBNL) family of RNA binding proteins has been shown to play a key role in the spliceopathy of RNA toxicity in myotonic dystrophy type 1 (DM1), the most common muscular dystrophy affecting adults and children. MBNL1 and MBNL2 are the most abundantly expressed members in skeletal muscle. A key aspect of DM1 is poor muscle regeneration and repair, leading to dystrophy.
View Article and Find Full Text PDFA novel flavone glycoside was isolated from the methanolic extract of Cynotis axillaris Schult. Various analysis and characterization techniques were used to determine its structure and properties. The compound exhibited a melting point range of 231-232 °C and had a molecular formula of C H O .
View Article and Find Full Text PDFA novel bioactive flavan glycoside was isolated by solvent extraction method with the help of Soxhlet apparatus from the methanolic extract of Sw. Flavan glycoside having molecular formula CHO, melting point 175-178C, molecular weight by ESI-MS m/z (M + H] 423, optical rotation was[α]-45.1(c 0.
View Article and Find Full Text PDFMyotonic dystrophy type 1 (DM1), the most common muscular dystrophy affecting adults and children, is a multi-systemic disorder affecting skeletal, cardiac, and smooth muscles as well as neurologic, endocrine and other systems. This review is on the cardiac pathology associated with DM1. The heart is one of the primary organs affected in DM1.
View Article and Find Full Text PDFRNA toxicity underlies the pathogenesis of disorders such as myotonic dystrophy type 1 (DM1). Muscular dystrophy is a key element of the pathology of DM1. The means by which RNA toxicity causes muscular dystrophy in DM1 is unclear.
View Article and Find Full Text PDFMyotonic dystrophy type 1 (DM1), the most common adult muscular dystrophy, is an autosomal dominant disorder caused by an expansion of a (CTG)n tract within the 3' untranslated region (3'UTR) of the dystrophia myotonica protein kinase (DMPK) gene. Mutant DMPK mRNAs are toxic, present in nuclear RNA foci and correlated with a plethora of RNA splicing defects. Cardinal features of DM1 are myotonia and cardiac conduction abnormalities.
View Article and Find Full Text PDFMyotonic dystrophy type 1 (DM1) is caused by an expanded (CTG)n tract in the 3'UTR of the DM protein kinase (DMPK) gene. The RNA transcripts produced from the expanded allele sequester or alter the function of RNA-binding proteins (MBNL1, CUGBP1, etc.).
View Article and Find Full Text PDFThe motion of two-filament artificial swimmers is modeled by assuming interfilament coupling via hydrodynamic viscous drag. The filaments are assumed to be in parallel and attached to a rigid spherical head. The boundary actuation is assumed to occur at the head-filament joint through an external oscillatory magnetic field and the filament motion is taken to be confined to the flexural plane.
View Article and Find Full Text PDFThe paper presents a simulation study of breath analysis based on theoretical models of microelectromechanical structure (MEMS) cantilever sensor array. The purpose of this study is to suggest a methodology for the development of MEMS electronic nose (e-nose) for monitoring disease-specific volatiles in exhaled breath. Oxidative stress and diabetes are taken as case studies for the assessment of e-nose designs.
View Article and Find Full Text PDFThe aim of the present study was to assess the effects of different organic and inorganic fertilizers on the functional diversity of soil microbial community under a vegetable production system. The Biolog Eco-plate technique and indices, such as average well-colour development (AWCD), McIntosh and Shannon diversity were employed to study the diversity of soil microorganisms. The AWCD, i.
View Article and Find Full Text PDFA new compound 1 was isolated from the methanolic extract of the stems of the Caesalpinia pulcherrima Linn. along with a reported compound (2) 3-O-β-D-glucopyranosyl-(1→4)-β-D-xylopyranosyl-(1→3)-α-L-rhamnopyranosyl-(1→2)-α-L-arabinopyranosyl hederagenin 28-O-β-D-glucopyranosyl-(1→6)-β-D-glucopyranosyl ester. The new compound 1 has m.
View Article and Find Full Text PDFMyotonic dystrophy type 1(DM1) is the prototype for diseases caused by RNA toxicity. RNAs from the mutant allele contain an expanded (CUG)n tract within the 3' untranslated region of the dystrophia myotonica protein kinase (DMPK) gene. The toxic RNAs affect the function of RNA binding proteins leading to sequestration of muscleblind-like (MBNL) proteins and increased levels of CELF1 (CUGBP, Elav-like family member 1).
View Article and Find Full Text PDFMyotonic dystrophy type 1 (DM1), the most common form of muscular dystrophy in adults, is caused by toxic RNAs produced from the mutant DM protein kinase (DMPK) gene. DM1 is characterized by progressive muscle wasting and weakness. Therapeutic strategies have mainly focused on targeting the toxic RNA.
View Article and Find Full Text PDFMyotonic dystrophy type 1 (DM1), the most prevalent muscular dystrophy in adults, is characterized by progressive muscle wasting and multi-systemic complications. DM1 is the prototype for disorders caused by RNA toxicity. Currently, no therapies exist.
View Article and Find Full Text PDFRNA toxicity is implicated in a number of disorders; especially those associated with expanded repeat sequences, such as myotonic dystrophy (DM1). Previously, we have shown increased NKX2-5 expression in RNA toxicity associated with DM1. Here, we investigate the relationship between NKX2-5 expression and muscle pathology due to RNA toxicity.
View Article and Find Full Text PDFEur J Obstet Gynecol Reprod Biol
October 2014
Stroke, defined as a focal or global disturbance of cerebral function lasting over 24h resulting from disruption of its blood supply, is a devastating event for a pregnant woman. This can result in long-term disability or death, and impact on her family and unborn child. In addition to pre-existing patient risk factors, the hypercoagulable state and pre-eclampsia need to be taken into account.
View Article and Find Full Text PDFMyotonic dystrophy type 1 (DM1), the most common form of adult-onset muscular dystrophy, is caused by an expanded (CTG)n repeat in the 3' untranslated region of the DM protein kinase (DMPK) gene. The toxic RNA transcripts produced from the mutant allele alter the function of RNA-binding proteins leading to the functional depletion of muscleblind-like (MBNL) proteins and an increase in steady state levels of CUG-BP1 (CUGBP-ETR-3 like factor 1, CELF1). The role of increased CELF1 in DM1 pathogenesis is well studied using genetically engineered mouse models.
View Article and Find Full Text PDFThe study empirically evaluates the abatement of As(III) uptake in rice seedlings (7d), in presence of Se and phosphate (P) under hydroponic condition. Positive correlation between As(III) translocation to the shoots of As(III) and P treated seedlings, shows P dependent As(III) translocation in rice. Whereas, presence of both P (5 and 10μgml(-1)) and (0.
View Article and Find Full Text PDFIn myotonic dystrophy type 1 (DM1), dystrophia myotonica protein kinase messenger ribonucleic acids (RNAs; mRNAs) with expanded CUG repeats (CUG(exp)) aggregate in the nucleus and become toxic to cells by sequestering and/or misregulating RNA-binding proteins, resulting in aberrant alternative splicing. In this paper, we find that the RNA-binding protein Staufen1 is markedly and specifically increased in skeletal muscle from DM1 mouse models and patients. We show that Staufen1 interacts with mutant CUG(exp) mRNAs and promotes their nuclear export and translation.
View Article and Find Full Text PDFThe paper presents an equivalent circuit model of the polymer coated surface acoustic wave (SAW) resonators by combining coupling-of-mode (COM) description of SAW resonators and perturbation calculation of SAW propagation under polymer loading. An expression for the motional load produced by polymer coating is deduced in terms of COM parameters and polymer characteristics. In addition, expressions for the shifts in resonance frequency and attenuation due to polymer loading are obtained.
View Article and Find Full Text PDFHerbal medicines play an important role in modern human life and have significant effects on treating diseases; however, the quality and safety of these herbal products has now become a serious issue due to increasing pollution in air, water, soil, etc. The present study proposes Fourier transform infrared spectroscopy (FTIR) along with the statistical method principal component analysis (PCA) to identify and discriminate herbal medicines for quality control. Herbal plants have been characterized using FTIR spectroscopy.
View Article and Find Full Text PDFThis article is concerned with the development of some stochastic models better suited to describe the observed distributions of the menstruating interval relating to last closed-birth interval of two types of females. In this context, two models suited for couples using or not using contraception have been proposed. In the first case, the model assumes a constant conception rate (analogous to the constant hazard rate in the life-testing problem) over time whereas the second case assumes a time-dependent form of the conception rate.
View Article and Find Full Text PDFSimplified relations for the changes in SAW velocity and attenuation due to thin polymer coatings and vapor sorption are presented by making analytic approximations to the complex theoretical model developed earlier by Martin et al. [Anal. Chem.
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