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1.
J Neuromuscul Dis ; 11(2): 285-297, 2024.
Artigo em Inglês | MEDLINE | ID: mdl-38363615

RESUMO

Background: Dilated cardiomyopathy (DCM) is a major complication of, and leading cause of mortality in Duchenne muscular dystrophy (DMD). Its severity, age at onset, and rate of progression display wide variability, whose molecular bases have been scarcely elucidated. Potential DCM-modifying factors include glucocorticoid (GC) and cardiological treatments, DMD mutation type and location, and variants in other genes. Methods and Results: We retrospectively collected 3138 echocardiographic measurements of left ventricular ejection fraction (EF), shortening fraction (SF), and end-diastolic volume (EDV) from 819 DMD participants, 541 from an Italian multicentric cohort and 278 from the Cooperative International Neuromuscular Group Duchenne Natural History Study (CINRG-DNHS). Using generalized estimating equation (GEE) models, we estimated the yearly rate of decrease of EF (-0.80%) and SF (-0.41%), while EDV increase was not significantly associated with age. Utilizing a multivariate generalized estimating equation (GEE) model we observed that mutations preserving the expression of the C-terminal Dp71 isoform of dystrophin were correlated with decreased EDV (-11.01 mL/m2, p = 0.03) while for dp116 were correlated with decreased EF (-4.14%, p = <0.001). The rs10880 genotype in the LTBP4 gene, previously shown to prolong ambulation, was also associated with increased EF and decreased EDV (+3.29%, p = 0.002, and -10.62 mL/m2, p = 0.008) with a recessive model. Conclusions: We quantitatively describe the progression of systolic dysfunction progression in DMD, confirm the effect of distal dystrophin isoform expression on the dystrophin-deficient heart, and identify a strong effect of LTBP4 genotype of DCM in DMD.


Assuntos
Cardiomiopatias , Distrofia Muscular de Duchenne , Humanos , Distrofina/genética , Distrofina/metabolismo , Haplótipos , Estudos Retrospectivos , Volume Sistólico , Função Ventricular Esquerda , Distrofia Muscular de Duchenne/genética , Distrofia Muscular de Duchenne/complicações , Cardiomiopatias/etiologia , Cardiomiopatias/genética , Isoformas de Proteínas/genética , Proteínas de Ligação a TGF-beta Latente/genética
2.
Front Pharmacol ; 14: 1306135, 2023.
Artigo em Inglês | MEDLINE | ID: mdl-38074123

RESUMO

Introduction: Solanum aethiopicum L., commonly known as scarlet eggplant (Solanaceae family) is one of the most traditionally cultivated vegetables in Basilicata, a southern region of Italy. Although multiple uses have been given to this vegetable, data about its anti-obesogenic activity are still limited. Methods: This study focuses on testing two different extracts obtained either from the peel or from the whole fruit of the Lucanian Solanum aethiopicum. Their ability to inhibit certain enzymatic activities was tested in vitro and then, the one that showed the better outcomes was tested on an experimental model of High-Fat Diet (HFD) induced obesity. Results: Spectrophotometric assays demonstrated that the peel extract possessed the highest ability to inhibit the selected enzymatic activities and so, its phytochemical profile was obtained through LC-MS chromatography. The oral administration of this extract (25 mg/kg) to HFD-fed mice reduced body weight gain and improved glucose and lipid metabolism. Similarly, the extract ameliorated the obesity-induced inflammatory status by reducing the expression of pro-inflammatory cytokines in both adipose and hepatic tissues. Interestingly, these effects were associated with the improvement of vascular dysfunction. Discussion: Lucanian Solanum aethiopicum extract may represent a new strategic approach for managing obesity and its associated diseases.

3.
Plants (Basel) ; 12(15)2023 Aug 03.
Artigo em Inglês | MEDLINE | ID: mdl-37571013

RESUMO

Obesity is widely associated with intestine barrier impairment, nonalcoholic fatty liver disease (NAFLD) outbreaks, oxidative stress, and inflammation. In a previous investigation, the Solanum aethiopicum L. growing in Basilicata Region has demonstrated to have antioxidant activity; hence this investigation was aimed to evaluate for the first time the antilipidemic and anti-inflammatory activity of the Lucanian S. aethiopicum L. peel extract in vitro on OA-treated HepG2 and Caco-2 cell lines. It was shown that the extract could reduce lipogenesis by down-regulating SREBP-1c and HMGCR expression and fatty acid ß-oxidation by up-regulating PPARα, CPT1A, and UCP2 expression. In addition, the S. aethiopicum L. peel extract might also improve oxidative stress by reducing endoplasmic reticulum stress and regulating the Nrf2 and Nf-κB molecular pathways. Altogether, these results demonstrated for the first time the possible application of the Lucanian S. aethiopicum peel extract for preventing obesity and managing NAFLD.

4.
Pharmaceutics ; 14(6)2022 May 30.
Artigo em Inglês | MEDLINE | ID: mdl-35745745

RESUMO

Food extract's biological effect and its improvement using nanotechnologies is one of the challenges of the last and the future decades; for this reason, the antioxidant effect of scarlet eggplant extract liposomal incorporation was investigated. Scarlet eggplant (Solanum aethiopicum L.) is a member of the Solanaceae family, and it is one of the most consumed vegetables in tropical Africa and south of Italy. This study investigated the antioxidant activity and the phytochemical composition of S. aethiopicum grown in the Basilicata Region for the first time. The whole fruit, peel, and pulp were subjected to ethanolic exhaustive maceration extraction, and all extracts were investigated. The HPLC-DAD analysis revealed the presence of ten phenolic compounds, including hydroxycinnamic acids, flavanones, flavanols, and four carotenoids (one xanthophyll and three carotenes). The peel extract was the most promising, active, and the richest in specialized metabolites; hence, it was tested on HepG2 cell lines and incorporated into liposomes. The nanoincorporation enhanced the peel extract's antioxidant activity, resulting in a reduction of the concentration used. Furthermore, the extract improved the expression of endogenous antioxidants, such as ABCG2, CAT, and NQO1, presumably through the Nrf2 pathway.

5.
Foods ; 6(4)2017 Apr 10.
Artigo em Inglês | MEDLINE | ID: mdl-28394267

RESUMO

Lycium barbarum (Goji) fruits are mainly cultivated in northwestern China and are well known for their beneficial and healthy effects. In this work, the biological and functional properties of Calabrian Goji extract, obtained from Goji berries cultivated in the Sibari Plain (in the Italian region of Calabria), were demonstrated. In order to evaluate the use of this extract as a food supplement for cognitive and mental disorders, the quantification of Carotenoids as Zeaxanthin equivalents was made. The antioxidant activity was investigated by evaluating the scavenging properties against 2,2'-diphenyl-1-picrylhydrazyl (DPPH) and 2,2'-Azinobis-(3-ethylbenzothiazoline-6-sulfonic acid) (ABTS) radicals and by performing the ORAC (Oxygen Radical Absorbance Capacity) assay. The inhibition of lipid peroxidation was quantified by bleaching test and the ability to inhibit acetylcholinesterase enzyme and to scavenge nitric oxide radical was also evaluated. All the results were compared to those obtained from a Chinese Goji extract used as a reference. Based on the reported data, Calabrian Goji might be used as a food supplement with a possible application in cognitive disorders, mental impairments and other neurodegenerative diseases, due to its biological properties and the high levels of Carotenoids.

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