Abnormal Sphingolipid World in Inflammation Specific for Lysosomal Storage Diseases and Skin Disorders

被引：33

作者：

Moskot, Marta ^{[1
,2
]}

Bochenska, Katarzyna ^{[2
]}

Jakobkiewicz-Banecka, Joanna ^{[2
]}

Banecki, Bogdan ^{[3
]}

Gabig-Ciminska, Magdalena ^{[1
,2
]}

机构：

[1] Polish Acad Sci, Inst Biochem & Biophys, Lab Mol Biol, Kadki 24, PL-80822 Gdansk, Poland

[2] Univ Gdansk, Dept Med Biol & Genet, Wita Stwosza 59, PL-80308 Gdansk, Poland

[3] UG MUG, Intercollegiate Fac Biotechnol, Dept Mol & Cellular Biol, Abrahama 58, PL-80307 Gdansk, Poland

来源：

INTERNATIONAL JOURNAL OF MOLECULAR SCIENCES | 2018年 / 19卷 / 01期

关键词：

sphingolipid metabolism alterations; inflammatory diseases; lysosomal storage diseases; skin disorders; treatments; workable therapies; STRATUM-CORNEUM LIPIDS; MITOCHONDRIAL PERMEABILITY TRANSITION; CENTRAL-NERVOUS-SYSTEM; GAUCHER-DISEASE; KRABBE DISEASE; SANDHOFF-DISEASE; FATTY-ACID; EPIDERMAL SPHINGOLIPIDS; SULFATIDE DEFICIENCY; SIGNAL-TRANSDUCTION;

D O I：

10.3390/ijms19010247

中图分类号：

Q5 [生物化学]; Q7 [分子生物学];

学科分类号：

071010 ; 081704 ;

摘要：

Research in recent years has shown that sphingolipids are essential signalling molecules for the proper biological and structural functioning of cells. Long-term studies on the metabolism of sphingolipids have provided evidence for their role in the pathogenesis of a number of diseases. As many inflammatory diseases, such as lysosomal storage disorders and some dermatologic diseases, including psoriasis, atopic dermatitis and ichthyoses, are associated with the altered composition and metabolism of sphingolipids, more studies precisely determining the responsibilities of these compounds for disease states are required to develop novel pharmacological treatment opportunities. It is worth emphasizing that knowledge from the study of inflammatory metabolic diseases and especially the possibility of their treatment may lead to insight into related metabolic pathways, including those involved in the formation of the epidermal barrier and providing new approaches towards workable therapies.

引用

页数：28

共 209 条

[21] The role of sphingolipid metabolism in cutaneous permeability barrier formation [J].