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Amyloidosis, Inflammation, and Oxidative Stress in the Heart of an Alkaptonuric Patient
المؤلفون المشاركون
Ghezzi, Lorenzo
Paccagnini, Eugenio
Braconi, Daniela
Giorgetti, Giovanna
Viti, Cecilia
Laschi, Marcella
Geminiani, Michela
Soldani, Patrizia
Lupetti, Pietro
Orlandini, Maurizio
Benvenuti, Chiara
Perfetto, Federico
Spreafico, Adriano
Millucci, Lia
Santucci, Annalisa
Bernardini, Giulia
المصدر
العدد
المجلد 2014، العدد 2014 (31 ديسمبر/كانون الأول 2014)، ص ص. 1-12، 12ص.
الناشر
Hindawi Publishing Corporation
تاريخ النشر
2014-04-28
دولة النشر
مصر
عدد الصفحات
12
التخصصات الرئيسية
الملخص EN
Background.
Alkaptonuria, a rare autosomal recessive metabolic disorder caused by deficiency in homogentisate 1,2-dioxygenase activity, leads to accumulation of oxidised homogentisic acid in cartilage and collagenous structures present in all organs and tissues, especially joints and heart, causing a pigmentation called ochronosis.
A secondary amyloidosis is associated with AKU.
Here we report a study of an aortic valve from an AKU patient.
Results.
Congo Red birefringence, Th-T fluorescence, and biochemical assays demonstrated the presence of SAA-amyloid deposits in AKU stenotic aortic valve.
Light and electron microscopy assessed the colocalization of ochronotic pigment and SAA-amyloid, the presence of calcified areas in the valve.
Immunofluorescence detected lipid peroxidation of the tissue and lymphocyte/macrophage infiltration causing inflammation.
High SAA plasma levels and proinflammatory cytokines levels comparable to those from rheumatoid arthritis patients were found in AKU patient.
Conclusions.
SAA-amyloidosis was present in the aortic valve from an AKU patient and colocalized with ochronotic pigment as well as with tissue calcification, lipid oxidation, macrophages infiltration, cell death, and tissue degeneration.
A local HGD expression in human cardiac tissue has also been ascertained suggesting a consequent local production of ochronotic pigment in AKU heart.
نمط استشهاد جمعية علماء النفس الأمريكية (APA)
Millucci, Lia& Ghezzi, Lorenzo& Paccagnini, Eugenio& Giorgetti, Giovanna& Viti, Cecilia& Braconi, Daniela…[et al.]. 2014. Amyloidosis, Inflammation, and Oxidative Stress in the Heart of an Alkaptonuric Patient. Mediators of Inflammation،Vol. 2014, no. 2014, pp.1-12.
https://search.emarefa.net/detail/BIM-1043460
نمط استشهاد الجمعية الأمريكية للغات الحديثة (MLA)
Millucci, Lia…[et al.]. Amyloidosis, Inflammation, and Oxidative Stress in the Heart of an Alkaptonuric Patient. Mediators of Inflammation No. 2014 (2014), pp.1-12.
https://search.emarefa.net/detail/BIM-1043460
نمط استشهاد الجمعية الطبية الأمريكية (AMA)
Millucci, Lia& Ghezzi, Lorenzo& Paccagnini, Eugenio& Giorgetti, Giovanna& Viti, Cecilia& Braconi, Daniela…[et al.]. Amyloidosis, Inflammation, and Oxidative Stress in the Heart of an Alkaptonuric Patient. Mediators of Inflammation. 2014. Vol. 2014, no. 2014, pp.1-12.
https://search.emarefa.net/detail/BIM-1043460
نوع البيانات
مقالات
لغة النص
الإنجليزية
الملاحظات
Includes bibliographical references
رقم السجل
BIM-1043460
قاعدة معامل التأثير والاستشهادات المرجعية العربي "ارسيف Arcif"
أضخم قاعدة بيانات عربية للاستشهادات المرجعية للمجلات العلمية المحكمة الصادرة في العالم العربي
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