Palmitoylation and depalmitoylation defects
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  • 作者:Thorsten Hornemann (1) (2)

    1. Institute for Clinical Chemistry
    ; University Hospital Zurich ; Raemistrasse 100 ; CH-8091 ; Zurich ; Switzerland
    2. Institute of Physiology and Zurich Center for Integrative Human Physiology (ZIHP)
    ; University of Zurich ; CH- 8057 ; Zurich ; Switzerland
  • 刊名:Journal of Inherited Metabolic Disease
  • 出版年:2015
  • 出版时间:January 2015
  • 年:2015
  • 卷:38
  • 期:1
  • 页码:179-186
  • 全文大小:411 KB
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  • 刊物类别:Medicine
  • 刊物主题:Medicine & Public Health
    Internal Medicine
    Metabolic Diseases
    Human Genetics
    Biochemistry
    Pediatrics
  • 出版者:Springer Netherlands
  • ISSN:1573-2665
文摘
Palmitoylation describes the enzymatic attachment of a 16-carbon atom fatty acid to a target protein. Such lipidation events occur in all eukaryotes and can be of reversible (S-palmitoylation) or irreversible (N-palmitoylation) nature. In particular S-palmitoylation is dynamically regulated by two opposing types of enzymes which add (palmitoyl acyltransferases - PAT) or remove (acyl protein thioesterases) palmitate from proteins. Protein palmitoylation is an important process that dynamically regulates the assembly and compartmentalization of many neuronal proteins at specific subcellular sites. Enzymes that regulate protein palmitoylation are critical for several biological processes. To date, eight palmitoylation related genes have been reported to be associated with human disease. This review intends to give an overview on the pathological changes which are associated with defects in the palmitoylation/depalmitoylation process.

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