TY - JOUR
T1 - Oxysterol changes along with cholesterol and vitamin D changes in adult phenylketonuric patients diagnosed by newborn mass-screening
AU - Nagasaka, Hironori
AU - Okano, Yoshiyuki
AU - Kimura, Akihiko
AU - Mizuochi, Tatsuki
AU - Sanayama, Yoshitami
AU - Takatani, Tomozumi
AU - Nakagawa, Saori
AU - Hasegawa, Eri
AU - Hirano, Ken ichi
AU - Mochizuki, Hiroshi
AU - Ohura, Toshihiro
AU - Ishige-Wada, Mika
AU - Usui, Hiromi
AU - Yorifuji, Tohru
AU - Tsukahara, Hirokazu
AU - Hirayama, Satoshi
AU - Ohtake, Akira
AU - Yamato, Susumu
AU - Miida, Takashi
N1 - Copyright:
Copyright 2013 Elsevier B.V., All rights reserved.
PY - 2013/2/1
Y1 - 2013/2/1
N2 - Background: Phenylketonuria (PKU) possibly leads to hypocholesterolemia and lowered vitamin D (VD) status. Metabolism of oxysterols linking with those of cholesterol and VD has never been examined in PKU. Methods: Blood oxysterols along with blood phenylalanine, lipids and VD were examined for 33 PKU adults aged 21-38. years and 20 age-matched healthy controls. Results: Total- and low-density cholesterols, and 25-hydroxy VD3 were decreased significantly in the PKU group (cholesterols, 10% decrease; 25-hydroxy VD3 35% decrease vs. the control group). 24S-hydroxycholesterol (24S-OHC) eliminating brain cholesterol, and 27-OHC and 7α-hydroxycholesterol (7α-OHC) representing peripheral and hepatic cholesterol elimination, respectively, were significantly decreased in PKU group: 24S-OHC, 25% decrease, p<.01; 27-OHC and 7α-OHC, 35-40% decrease, p<.001. 7β-Hydroxycholesterol (7β-OHC) reflecting oxidative stress was increased significantly in PKU group (p<.05). 7α-OHC and 27-OHC levels in PKU group always showed similar values, regardless of other parameters while the 24S-OHC and 7β-OHC levels decreased and increased, respectively, showing significant correlations with phenylalanine level (p<.005). 27-OHC level showed a significant positive correlation with the 25-hydroxy VD3 level in this group (p<.001). Conclusion: Blood oxysterol changes predominate over blood cholesterol changes and influence on VD status in adult PKU patients.
AB - Background: Phenylketonuria (PKU) possibly leads to hypocholesterolemia and lowered vitamin D (VD) status. Metabolism of oxysterols linking with those of cholesterol and VD has never been examined in PKU. Methods: Blood oxysterols along with blood phenylalanine, lipids and VD were examined for 33 PKU adults aged 21-38. years and 20 age-matched healthy controls. Results: Total- and low-density cholesterols, and 25-hydroxy VD3 were decreased significantly in the PKU group (cholesterols, 10% decrease; 25-hydroxy VD3 35% decrease vs. the control group). 24S-hydroxycholesterol (24S-OHC) eliminating brain cholesterol, and 27-OHC and 7α-hydroxycholesterol (7α-OHC) representing peripheral and hepatic cholesterol elimination, respectively, were significantly decreased in PKU group: 24S-OHC, 25% decrease, p<.01; 27-OHC and 7α-OHC, 35-40% decrease, p<.001. 7β-Hydroxycholesterol (7β-OHC) reflecting oxidative stress was increased significantly in PKU group (p<.05). 7α-OHC and 27-OHC levels in PKU group always showed similar values, regardless of other parameters while the 24S-OHC and 7β-OHC levels decreased and increased, respectively, showing significant correlations with phenylalanine level (p<.005). 27-OHC level showed a significant positive correlation with the 25-hydroxy VD3 level in this group (p<.001). Conclusion: Blood oxysterol changes predominate over blood cholesterol changes and influence on VD status in adult PKU patients.
KW - Cholesterol
KW - Oxysterols
KW - Phenylalanine
KW - Vitamin D
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U2 - 10.1016/j.cca.2012.10.011
DO - 10.1016/j.cca.2012.10.011
M3 - Article
C2 - 23168022
AN - SCOPUS:84871445684
VL - 416
SP - 54
EP - 59
JO - Clinica Chimica Acta
JF - Clinica Chimica Acta
SN - 0009-8981
ER -