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

Year 2021, , 565 - 571, 30.12.2021

Aslı İnci , Asburce Olgac , Betül Genç Derin Gürsel Biberoğlu , İlyas Okur , Fatih Süheyl Ezgü , Leyla Tümer

https://doi.org/10.17343/sdutfd.928607

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

Supporting Institution

Yoktur

Project Number

Yoktur

Thanks

Tüm hastalarımıza ve sağlık çalışanlarına teşekkür ederiz.

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

Project Number

Yoktur

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