In:
The EMBO Journal, EMBO, Vol. 35, No. 24 ( 2016-12-15), p. 2699-2716
Abstract:
image Seipin, mutated in severe congenital lipodystrophy ( BSCL 2), is an ER –lipid droplet ( LD ) contact protein that regulates the extent of ER – LD contacts and facilitates incorporation of lipid and protein cargo into maturing LD s. Seipin deficiency increases the heterogeneity of ER – LD contacts, resulting in completely missing, rudimentary, or very extensive contacts. Seipin is required during LD formation for the targeting of ER ‐derived fatty acid‐activating enzyme ACSL 3 to LD s. An ER ‐to‐ LD targeting model peptide and a fluorescent fatty acid analog initially reach newly formed LD s, but their subsequent incorporation into LD s is impaired in the absence of seipin. In seipin deficiency, the fatty acid flux to neutral lipids becomes compromised when LD formation has been initiated.
Type of Medium:
Online Resource
ISSN:
0261-4189
,
1460-2075
DOI:
10.15252/embj.201695170
Language:
English
Publisher:
EMBO
Publication Date:
2016
detail.hit.zdb_id:
1467419-1
detail.hit.zdb_id:
586044-1
SSG:
12
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