دورية أكاديمية
أعرض في EDS

Rare and intractable fibrodysplasia ossificans progressiva shows different PBMC phenotype possibly modulated by ascorbic acid and propranolol treatment.

التفاصيل البيبلوغرافية
العنوان: Rare and intractable fibrodysplasia ossificans progressiva shows different PBMC phenotype possibly modulated by ascorbic acid and propranolol treatment.
المؤلفون: Nascimento DR; UFMS/ Faculty of Medicine, Campo Grande, MS, Brazil., Balaniuc SLB; UFMS/ Faculty of Medicine, Campo Grande, MS, Brazil., Palhares DB; UFMS/ Faculty of Medicine, Campo Grande, MS, Brazil., Underwood A; Walsh University, Division of Mathematics and Sciences, North Canton, OH, USA., Palhares MG; UFMS/ Faculty of Medicine, Campo Grande, MS, Brazil., Alves F; UFMG/ Department of Physiology and Biophysics, Belo Horizonte, MG, Brazil.; Centro Universitário Metodista Izabela Hendrix- IMIH, Belo Horizonte, MG, Brazil., Vieira FO; UFMG/ Department of Physiology and Biophysics, Belo Horizonte, MG, Brazil.; Centro Universitário Metodista Izabela Hendrix- IMIH, Belo Horizonte, MG, Brazil., Souza-Fagundes EM; UFMG/ Department of Physiology and Biophysics, Belo Horizonte, MG, Brazil., Giuliani LR; UFMS/ Faculty of Medicine, Campo Grande, MS, Brazil., Xavier PCN; UFMS/ Faculty of Medicine, Campo Grande, MS, Brazil., Puerto HLD; UFMG/ Department of Physiology and Biophysics, Belo Horizonte, MG, Brazil., Santos RAS; UFMG/ Department of Physiology and Biophysics, Belo Horizonte, MG, Brazil., Milsted A; Walsh University, Division of Mathematics and Sciences, North Canton, OH, USA., Brum JM; Procter & Gamble Health Care & Global Clinical Sciences, Mason, OH, USA., Silva IS; UFMS/ Faculty of Medicine, Campo Grande, MS, Brazil., Martins AS; UFMS/ Faculty of Medicine, Campo Grande, MS, Brazil.; UFMG/ Department of Physiology and Biophysics, Belo Horizonte, MG, Brazil.
المصدر: Intractable & rare diseases research [Intractable Rare Dis Res] 2021 Aug; Vol. 10 (3), pp. 179-189.
نوع المنشور: Journal Article
اللغة: English
بيانات الدورية: Publisher: International Advancement Center for Medicine & Health Research Co. (IACMHR Co.) Country of Publication: Japan NLM ID: 101586847 Publication Model: Print Cited Medium: Print ISSN: 2186-3644 (Print) Linking ISSN: 21863644 NLM ISO Abbreviation: Intractable Rare Dis Res Subsets: PubMed not MEDLINE
أسماء مطبوعة: Original Publication: Tokyo : International Advancement Center for Medicine & Health Research Co. (IACMHR Co.)
مستخلص: Fibrodysplasia Ossificans Progressiva (FOP) is a rare congenital intractable disease associated with a mutation in ACVR1 gene, characterized by skeleton malformations. Ascorbic acid (AA) and propranolol (PP) in combination is reported to minimize flare-ups in patients. FOP leukocyte phenotype may possibly be modulated by AA and PP treatment. In this study, expression of 22 potential target genes was analyzed by RT-PCR in peripheral blood mononuclear cells culture (PBMC) from FOP patients and controls to determine effectiveness of the combination therapy. PBMC were treated with AA, PP and AA+PP combination. Basal expression of 12 of the 22 genes in FOP PBMC was statistically different from controls. ACVR1 , ADCY2 , ADCY9 and COL3 were downregulated while COL1 was upregulated. ADRB1 , ADRB2 , RUNX2 , TNF-α and ACTB , were all overexpressed in FOP PBMC. In control, AA upregulated COL1, SVCT1, ACTB, AGTR2 and downregulated ADCY2 . In FOP cells, AA upregulated ACVR1, BMP4, COL1, COL3, TNF-α , ADCY2, ADCY9, AGTR2 and MAS , while downregulated ADBR2, RUNX2, ADCY1, SVCT1 and ACTB . PP increased ADBR1 and decreased RUNX2, TNF-α, AGTR1, ACTB and CHRNA7 genes in treated control PBMC compared to untreated. PP upregulated ADBR1, ADBR2 and MAS , and downregulated TNF-α and ACTB in treated FOP PBMC versus untreated. AA+PP augmented ADRB1 and ADRB2 expressions in control PBMC. In FOP PBMC, AA+PP augmented ACVR1, COL1, COL3, ADBR1, AGTR2 and MAS expression and downregulated ADBR2, RUNX2, ACTB and MRGD . These data show distinct gene expression modulation in leukocytes from FOP patients when treated with AA and or PP.
Competing Interests: The authors have no conflicts of interest to disclose.
(2021, International Research and Cooperation Association for Bio & Socio - Sciences Advancement.)
References: Bone. 2018 Apr;109:232-240. (PMID: 29097342)
Shock. 2003 Feb;19(2):108-12. (PMID: 12578116)
Genes Nutr. 2014 May;9(3):390. (PMID: 24604612)
J Clin Invest. 2017 Sep 1;127(9):3339-3352. (PMID: 28758906)
Nat Genet. 2006 May;38(5):525-7. (PMID: 16642017)
PLoS One. 2013;8(3):e60021. (PMID: 23555867)
J Bone Miner Res. 2008 Mar;23(3):305-13. (PMID: 17967130)
Cytometry B Clin Cytom. 2018 Jul;94(4):613-622. (PMID: 28985649)
Methods. 2001 Dec;25(4):402-8. (PMID: 11846609)
J Biol Chem. 2010 Feb 12;285(7):4319-27. (PMID: 19946124)
Int J Clin Exp Pathol. 2013 Dec 15;7(1):48-55. (PMID: 24427325)
Expert Opin Orphan Drugs. 2017;5(4):291-294. (PMID: 29177121)
J Intern Med. 2009 Jun;265(6):663-79. (PMID: 19493060)
Exp Cell Res. 1997 Sep 15;235(2):362-9. (PMID: 9299160)
Biochim Biophys Acta. 2002 Jan 15;1569(1-3):1-9. (PMID: 11853951)
Tex Heart Inst J. 2007;34(3):268-74. (PMID: 17948074)
J Invest Dermatol. 2001 Jun;116(6):853-9. (PMID: 11407971)
Physiol Rev. 2018 Jan 1;98(1):505-553. (PMID: 29351514)
Cent Eur J Immunol. 2015;40(3):373-9. (PMID: 26648784)
Am J Pathol. 2007 Feb;170(2):620-32. (PMID: 17255330)
Proc Natl Acad Sci U S A. 2005 Sep 20;102(38):13604-9. (PMID: 16157892)
Gene. 2019 Jul 30;707:151-171. (PMID: 31075413)
Crit Rev Eukaryot Gene Expr. 2010;20(4):313-24. (PMID: 21395504)
Br J Clin Pharmacol. 2019 Jun;85(6):1180-1187. (PMID: 30501012)
Calcif Tissue Int. 1997 Apr;60(4):394. (PMID: 9075640)
J Biol Chem. 1996 Jun 7;271(23):13900-7. (PMID: 8662814)
J Bone Miner Res. 2012 Jun;27(6):1252-62. (PMID: 22407956)
Nat Med. 2004 Apr;10(4):396-401. (PMID: 15034573)
J Clin Invest. 2007 Jun;117(6):1616-26. (PMID: 17549257)
J Recept Signal Transduct Res. 2002 Feb-Nov;22(1-4):79-110. (PMID: 12503609)
Muscle Nerve. 2019 Apr;59(4):501-508. (PMID: 30623463)
Calcif Tissue Int. 2014 Jun;94(6):621-31. (PMID: 24626604)
Microsc Res Tech. 2002 Jul 15;58(2):77-84. (PMID: 12203706)
J Interferon Cytokine Res. 2005 Jul;25(7):384-94. (PMID: 16022583)
Sci Rep. 2017 Mar 28;7:45597. (PMID: 28349965)
Bone. 2016 Mar;84:169-180. (PMID: 26769004)
Cell Signal. 2020 Dec;76:109813. (PMID: 33080316)
Development. 2016 Nov 1;143(21):3933-3943. (PMID: 27621060)
Intractable Rare Dis Res. 2019 Feb;8(1):24-28. (PMID: 30881854)
Oral Dis. 2016 Sep;22(6):463-93. (PMID: 26808119)
JCI Insight. 2018 Nov 15;3(22):. (PMID: 30429363)
Curr Genomics. 2010 Mar;11(1):52-7. (PMID: 20808524)
Dev Cell. 2004 Mar;6(3):423-35. (PMID: 15030764)
Angiogenesis. 2003;6(4):259-69. (PMID: 15166494)
J Trace Elem Med Biol. 2017 Jan;39:186-192. (PMID: 27908413)
Immunol Rev. 2012 Jul;248(1):188-204. (PMID: 22725962)
Pediatr Endocrinol Rev. 2013 Jun;10 Suppl 2:437-48. (PMID: 23858627)
Biochim Biophys Acta. 2007 Jan;1772(1):32-9. (PMID: 17008068)
Front Pharmacol. 2015 Aug 13;6:171. (PMID: 26321956)
Eur J Clin Pharmacol. 1995;48(3-4):295-7. (PMID: 7589058)
Dev Biol. 2015 Apr 15;400(2):202-9. (PMID: 25722188)
فهرسة مساهمة: Keywords: FOP; FOPCON; gene expression modulation; peripheral blood mononuclear cells
تواريخ الأحداث: Date Created: 20210901 Latest Revision: 20210903
رمز التحديث: 20231215
مُعرف محوري في PubMed: PMC8397826
DOI: 10.5582/irdr.2021.01012
PMID: 34466340
قاعدة البيانات: MEDLINE
الوصف
تدمد:2186-3644
DOI:10.5582/irdr.2021.01012