Abstract
| Original language | English |
|---|---|
| Pages (from-to) | 1843-1859 |
| Number of pages | 17 |
| Journal | Clinical Science |
| Volume | 130 |
| Issue number | 21 |
| Early online date | 22 Sep 2016 |
| DOIs | |
| Publication status | Published - Nov 2016 |
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Keywords
- cholesterol
- homoeostasis
- cellular function
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Intracellular cholesterol transport proteins: roles in health and disease. / Soffientini, Ugo; Graham, Annette.
In: Clinical Science, Vol. 130, No. 21, 11.2016, p. 1843-1859.Research output: Contribution to journal › Article
TY - JOUR
T1 - Intracellular cholesterol transport proteins: roles in health and disease
AU - Soffientini, Ugo
AU - Graham, Annette
N1 - Acceptance in SAN AAM: 12m embargo (see http://www.portlandpresspublishing.com/content/open-access-policy) Exception requested, author confirmed no exception (email in SAN 24-5-17) FUNDING This work was supported by the British Heart Foundation [grant numbers PG/07/039/22873 and PG04/063/17186]; the British Skin Foundation [grant number 807S]; the Diabetes UK [grant number 11/0004333]; and the Heart Research UK [grant number 2515/07/09].
PY - 2016/11
Y1 - 2016/11
N2 - Effective cholesterol homoeostasis is essential in maintaining cellular function, and this is achieved by a network of lipid-responsive nuclear transcription factors, and enzymes, receptors and transporters subject to post-transcriptional and post-translational regulation, whereas loss of these elegant, tightly regulated homoeostatic responses is integral to disease pathologies. Recent data suggest that sterol-binding sensors, exchangers and transporters contribute to regulation of cellular cholesterol homoeostasis and that genetic overexpression or deletion, or mutations, in a number of these proteins are linked with diseases, including atherosclerosis, dyslipidaemia, diabetes, congenital lipoid adrenal hyperplasia, cancer, autosomal dominant hearing loss and male infertility. This review focuses on current evidence exploring the function of members of the ‘START’ (steroidogenic acute regulatory protein-related lipid transfer) and ‘ORP’ (oxysterol-binding protein-related proteins) families of sterol-binding proteins in sterol homoeostasis in eukaryotic cells, and the evidence that they represent valid therapeutic targets to alleviate human disease.
AB - Effective cholesterol homoeostasis is essential in maintaining cellular function, and this is achieved by a network of lipid-responsive nuclear transcription factors, and enzymes, receptors and transporters subject to post-transcriptional and post-translational regulation, whereas loss of these elegant, tightly regulated homoeostatic responses is integral to disease pathologies. Recent data suggest that sterol-binding sensors, exchangers and transporters contribute to regulation of cellular cholesterol homoeostasis and that genetic overexpression or deletion, or mutations, in a number of these proteins are linked with diseases, including atherosclerosis, dyslipidaemia, diabetes, congenital lipoid adrenal hyperplasia, cancer, autosomal dominant hearing loss and male infertility. This review focuses on current evidence exploring the function of members of the ‘START’ (steroidogenic acute regulatory protein-related lipid transfer) and ‘ORP’ (oxysterol-binding protein-related proteins) families of sterol-binding proteins in sterol homoeostasis in eukaryotic cells, and the evidence that they represent valid therapeutic targets to alleviate human disease.
KW - cholesterol
KW - homoeostasis
KW - cellular function
U2 - 10.1042/CS20160339
DO - 10.1042/CS20160339
M3 - Article
VL - 130
SP - 1843
EP - 1859
JO - Clinical Science
JF - Clinical Science
SN - 0143-5221
IS - 21
ER -