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YANG Moujie^1abc,2WANG Junnan^1abc,2,WU Huixian^1abc,2, JIN Junfei^1abc,2 , PAN Zhixiong^1abc,2

( 1.a. Guangxi Key Laboratory of Molecular Medicine in Liver and Repair, b. Guangxi Key Laboratory ofBasic Research in $phingolipid Metabolism Related Diseases , c. Laboratory of Hepatobiliary and PancreaticSurgery , Affiliated Hospital of Guilin Medical University, Guilin 541001, China; 2. China-USA Lipids inHealth and Disease Research Center, Guilin Medical University, Guilin 541001, China)

Abstract Sphingolipids are important components of cell membrane lipids and play an important role in manyAbstractdiseases. When the sphingolipid synthesis pathway is disturbed , sphingolipid metabolism is reprogrammed, resultingin the transformation of synthetic products into 1-deoxysphingolipid( DoxSL,). This article introduees thebiosynthesis, metabolism and related funetions of DoxSL, with emphasis on the effects of DoxSL,on cytotoxicity,neurite effect and membrane hydrophobicity. In addition, abnormal DoxSL, level has been associated with a variety ofhuman diseases, with lower serine level leading to pathological inereases in DoxSL., and elevated DoxSl, has beenassociated with diabetes, non-alcoholie faty liver disease ( NAFLD) , and hereditary Sensory Autonomic Neuropathytype 1 ( HSAN1 ). DoxSL, can be used as a biomarker to predict diabetes, hepatocyte steatosis involved in NAFLD.and the increase of DoxSL, caused by mutation of serine palmitoyl transferase subunit is an important reason for theformation of HSAN1. Monitoring and regulating DoxSl, content may provide new ideas for the diagnosis and treatmentof clinically related diseases. 'This review will brielly diseuss the characteristics and functions of DoxSL and its role inthe occurrence and development of related diseases.Keywords: 1-deoxysphingolipid; biosynthesis; diabetes; non-aleoholic fatty liver disease; hereditary sensoryautonomic neuropathy type l

DOI:10.19296/j.cnki.1008-2409.2024-02-003

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