0 52

Cited 2 times in

Integrative lipidomic and transcriptomic analysis of X-linked adrenoleukodystrophy reveals distinct lipidome signatures between adrenomyeloneuropathy and childhood cerebral adrenoleukodystrophy

Authors
 Dong-Kyu Lee  ;  Nguyen Phuoc Long  ;  Juwon Jung  ;  Tae Joon Kim  ;  Euiyeon Na  ;  Yun Pyo Kang  ;  Sung Won Kwon  ;  Jiho Jang 
Citation
 Biochemical and Biophysical Research Communications, Vol.508(2) : 536-569, 2019 
Journal Title
 Biochemical and Biophysical Research Communications 
ISSN
 0006-291X 
Issue Date
2019
Keywords
Adrenomyeloneuropathy ; Childhood cerebral adrenoleukodystrophy ; Lipidomics ; Transcriptomics ; X-linked adrenoleukodystrophy
Abstract
Precise pathophysiology with respect to the phenotypic variations and severity of X-ALD, specifically between adrenomyeloneuropathy (AMN) and childhood cerebral adrenoleukodystrophy (CCALD), has not been fully discovered. Herein, a systematic analysis using multi-layered lipidomics and transcriptomics was conducted to elucidate distinctive metabolic biosignatures among healthy control, AMN, and CCALD. Significant alterations regarding the accumulation of very long chain fatty acids were found in various lipid species such as phospholipids, glycerolipids, and sphingolipids. Remarkably, TG and CER that are physiologically essential were markedly down-regulated in CCALD than AMN. Transcriptomic analysis further supported the robustness of our findings by providing valuable information on the gene expressions of the regulatory factors. For instance, regulators of sphingolipid catabolism (SMPD1, CERK, and SPHK1) and TG anabolism (GPAM, GPAT2, and MBOAT2) were more up-regulated in AMN than in CCALD. These observations, among others, were in line with the recognized alterations of the associated lipidomes. In conclusion, the homeostatic imbalance of the complex lipid networks may be pathogenically important in X-ALD and the particular dysregulations of TG and CER may further influence the severity of CCALD among X-ALD patients.
Full Text
https://www.sciencedirect.com/science/article/pii/S0006291X18325555
DOI
10.1016/j.bbrc.2018.11.123
Appears in Collections:
1. College of Medicine (의과대학) > Dept. of Physiology (생리학교실) > 1. Journal Papers
Yonsei Authors
장지호(Jang, Ji Ho) ORCID logo https://orcid.org/0000-0001-5551-3514
Export
RIS (EndNote)
XLS (Excel)
XML
URI
https://ir.ymlib.yonsei.ac.kr/handle/22282913/167346
사서에게 알리기
  feedback

qrcode

Items in DSpace are protected by copyright, with all rights reserved, unless otherwise indicated.

Browse