PROPIONYLCARNITINE AND FREE CARNITINE ARE NEW BIOMARKERS IN THE FOLLOW-UP PERIOD OF MUCOPOLYSACCARIDOSIS TO SCREEN OXIDATIVE STRESS

Abstract

Objective There is no applicable and easy procedure to screen oxidative stress in mucopolysaccaridosis (MPS) patients. The aim herein was to show the antioxidative properties of free carnitine (FC) and propionylcarnitine (PC) with respect to oxidative markers in MPS and use a simple and easy method in patient follow-up. Material and Methods FC and PC were studied as an antioxidative marker using tandem mass spectroscopy and malondialdehyde (MDA) was studied as an oxidative marker in 27 MPS patients and 24 healthy volunteers. Results While the PC and FC levels were significantly decreased, the MDA levels were higher in the MPS patients than in the healthy volunteers.When compared between the enzyme-treated MPS patients and untreated MPS patients, there were no significant differences between the groups. MDA was found to inversely correlated with PC (r =–0.402, P=0.003).It was noteworthy that PC had a significant correlation with MDA. Conclusion The findings revealed that the affected patients had higher MDA levels and lower PC and FC levels, indicating an imbalance through the oxidative side. An applicable method of FC and PC measurement could be used to screen patients, considering them as new antioxidative markers in the patient follow-up period for the response of enzyme replacement therapy and/ or antioxidant drugs.

Keywords

• free carnitine
• mucopolysaccaridosis
• propionylcarnitine
• antioxidation
• MDA

MUKOPOLİSAKKARİDOZ HASTALARININ TAKİP SÜRECİNDE OKSİDATİF STRESİ TARAMADA YENİ BİYOBELİRTEÇLER, PROPİONİLKARNİTİN VE SERBEST KARNİTİN

Abstract

Amaç Mukopolisakkaridoz (MPS) hastalarında oksidatif stresi taramak için uygulanabilir ve kolay bir prosedür mevcut değildir. Bu çalışmada amaç, MPS'deki oksidatif belirteçlere göre serbest karnitin (SK) ve propiyonilkarnitinin (PK) antioksidatif özelliklerini göstermek ve hasta takibinde basit ve kolay bir yöntem olarak kullanmaktır. Gereç ve Yöntem Çalışmaya, 27 MPS hastasında ve 24 sağlıklı gönüllü dahil edilerek bu hastalarda SK ve PK, antioksidatif belirteç olarak tandem kütle spektroskopisi kullanılarak çalışıldı ve malondialdehit (MDA), ise oksidatif belirteç olarak kullanıldı. Bulgular MPS hastalarında, PK ve SK seviyeleri önemli ölçüde azalırken, MDA seviyeleri sağlıklı gönüllülere göre daha yüksek bulundu. Enzim yerine koyma tedavisi alan MPS hastaları ile tedavi edilmeyen MPS hastaları karşılaştırıldığında, gruplar arasında anlamlı bir fark saptanmadı. Çalışma, MDA'nın PK ile anlamlı oranda ters orantılı olduğu bulundu (r = –0.402, P = 0.003). PK'nin MDA ile anlamlı bir korelasyona sahip olması dikkat çekiciydi. Sonuç MPS hastalarının daha yüksek MDA düzeylerine ve daha düşük PK ve SK düzeylerine sahip olduğunu göstermiştir, buna göre MPS hastalarının oksidatif yönde bir dengesizliğe sahip olduğunu ortaya koymuştur. PK ve SK, MPS hastalarının takibinde uygulanabilir bir yöntem olabilir ve hastaların enzim yerine koyma ve/veya antioksidan ilaçların tedavilerinin cevabının gözlemek için yeni biyobelirteçler olarak kullanılabilir.

Keywords

• mukopolisakkaridoz
• propionilkarnitin
• serbest karnitin
• antioksidasyon
• MDA

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