ChloroquineV1, Ncbi, Merge, bibRecord, 000157

Lysosomal acid lipase deficiency impairs regulation of ABCA1 gene and formation of high density lipoproteins in cholesteryl ester storage disease.

Identifieur interne : 000157 ( Ncbi/Merge ); précédent : 000156; suivant : 000158

Lysosomal acid lipase deficiency impairs regulation of ABCA1 gene and formation of high density lipoproteins in cholesteryl ester storage disease.

Auteurs : Kristin L. Bowden [Canada] ; Nicolas J. Bilbey ; Leanne M. Bilawchuk ; Emmanuel Boadu ; Rohini Sidhu ; Daniel S. Ory ; Hong Du ; Teddy Chan ; Gordon A. Francis

Source :

The Journal of biological chemistry [ 1083-351X ] ; 2011.

RBID : pubmed:21757691

Descripteurs français

English descriptors

KwdEn :
- ATP Binding Cassette Transporter 1, ATP-Binding Cassette Transporters (metabolism), Chloroquine (metabolism), Cholesterol (metabolism), Cholesterol Ester Storage Disease (genetics), Cholesterol Ester Storage Disease (metabolism), Culture Media, Conditioned (pharmacology), Fibroblasts (metabolism), Humans, Lipoproteins (metabolism), Lipoproteins, HDL (metabolism), Mutation, Phospholipids (metabolism), Recombinant Proteins (metabolism), Skin (metabolism), Sterols (chemistry), Wolman Disease (genetics), Wolman Disease (metabolism).
MESH :
- chemical , chemistry : Sterols.
- chemical , metabolism : ATP-Binding Cassette Transporters, Chloroquine, Cholesterol, Lipoproteins, Lipoproteins, HDL, Phospholipids, Recombinant Proteins.
- chemical , pharmacology : Culture Media, Conditioned.
- chemical : ATP Binding Cassette Transporter 1.
- genetics : Cholesterol Ester Storage Disease, Wolman Disease.
- metabolism : Cholesterol Ester Storage Disease, Fibroblasts, Skin, Wolman Disease.
- Humans, Mutation.

Abstract

ATP-binding cassette transporter A1 (ABCA1) mediates the rate-limiting step in high density lipoprotein (HDL) particle formation, and its expression is regulated primarily by oxysterol-dependent activation of liver X receptors. We previously reported that ABCA1 expression and HDL formation are impaired in the lysosomal cholesterol storage disorder Niemann-Pick disease type C1 and that plasma HDL-C is low in the majority of Niemann-Pick disease type C patients. Here, we show that ABCA1 regulation and activity are also impaired in cholesteryl ester storage disease (CESD), caused by mutations in the LIPA gene that result in less than 5% of normal lysosomal acid lipase (LAL) activity. Fibroblasts from patients with CESD showed impaired up-regulation of ABCA1 in response to low density lipoprotein (LDL) loading, reduced phospholipid and cholesterol efflux to apolipoprotein A-I, and reduced α-HDL particle formation. Treatment of normal fibroblasts with chloroquine to inhibit LAL activity reduced ABCA1 expression and activity, similar to that of CESD cells. Liver X receptor agonist treatment of CESD cells corrected ABCA1 expression but failed to correct LDL cholesteryl ester hydrolysis and cholesterol efflux to apoA-I. LDL-induced production of 27-hydroxycholesterol was reduced in CESD compared with normal fibroblasts. Treatment with conditioned medium containing LAL from normal fibroblasts or with recombinant human LAL rescued ABCA1 expression, apoA-I-mediated cholesterol efflux, HDL particle formation, and production of 27-hydroxycholesterol by CESD cells. These results provide further evidence that the rate of release of cholesterol from late endosomes/lysosomes is a critical regulator of ABCA1 expression and activity, and an explanation for the hypoalphalipoproteinemia seen in CESD patients.

DOI: 10.1074/jbc.M111.274381
PubMed: 21757691

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to stream PubMed, to step Corpus: 000377
to stream PubMed, to step Curation: 000377
to stream PubMed, to step Checkpoint: 000365

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pubmed:21757691

Le document en format XML

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<term>Cholesterol (metabolism)</term>
<term>Cholesterol Ester Storage Disease (genetics)</term>
<term>Cholesterol Ester Storage Disease (metabolism)</term>
<term>Culture Media, Conditioned (pharmacology)</term>
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<term>Humans</term>
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<term>Lipoproteins, HDL (metabolism)</term>
<term>Mutation</term>
<term>Phospholipids (metabolism)</term>
<term>Recombinant Proteins (metabolism)</term>
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<term>Cholestérol (métabolisme)</term>
<term>Fibroblastes (métabolisme)</term>
<term>Humains</term>
<term>Lipoprotéines (métabolisme)</term>
<term>Lipoprotéines HDL (métabolisme)</term>
<term>Maladie de Wolman (génétique)</term>
<term>Maladie de Wolman (métabolisme)</term>
<term>Maladie de stockage des esters de cholestérol (génétique)</term>
<term>Maladie de stockage des esters de cholestérol (métabolisme)</term>
<term>Milieux de culture conditionnés (pharmacologie)</term>
<term>Mutation</term>
<term>Peau (métabolisme)</term>
<term>Phospholipides (métabolisme)</term>
<term>Protéines recombinantes (métabolisme)</term>
<term>Stérols ()</term>
<term>Transporteur-1 à cassette liant l'ATP</term>
<term>Transporteurs ABC (métabolisme)</term>
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<front><div type="abstract" xml:lang="en">ATP-binding cassette transporter A1 (ABCA1) mediates the rate-limiting step in high density lipoprotein (HDL) particle formation, and its expression is regulated primarily by oxysterol-dependent activation of liver X receptors. We previously reported that ABCA1 expression and HDL formation are impaired in the lysosomal cholesterol storage disorder Niemann-Pick disease type C1 and that plasma HDL-C is low in the majority of Niemann-Pick disease type C patients. Here, we show that ABCA1 regulation and activity are also impaired in cholesteryl ester storage disease (CESD), caused by mutations in the LIPA gene that result in less than 5% of normal lysosomal acid lipase (LAL) activity. Fibroblasts from patients with CESD showed impaired up-regulation of ABCA1 in response to low density lipoprotein (LDL) loading, reduced phospholipid and cholesterol efflux to apolipoprotein A-I, and reduced α-HDL particle formation. Treatment of normal fibroblasts with chloroquine to inhibit LAL activity reduced ABCA1 expression and activity, similar to that of CESD cells. Liver X receptor agonist treatment of CESD cells corrected ABCA1 expression but failed to correct LDL cholesteryl ester hydrolysis and cholesterol efflux to apoA-I. LDL-induced production of 27-hydroxycholesterol was reduced in CESD compared with normal fibroblasts. Treatment with conditioned medium containing LAL from normal fibroblasts or with recombinant human LAL rescued ABCA1 expression, apoA-I-mediated cholesterol efflux, HDL particle formation, and production of 27-hydroxycholesterol by CESD cells. These results provide further evidence that the rate of release of cholesterol from late endosomes/lysosomes is a critical regulator of ABCA1 expression and activity, and an explanation for the hypoalphalipoproteinemia seen in CESD patients.</div>
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<Abstract><AbstractText>ATP-binding cassette transporter A1 (ABCA1) mediates the rate-limiting step in high density lipoprotein (HDL) particle formation, and its expression is regulated primarily by oxysterol-dependent activation of liver X receptors. We previously reported that ABCA1 expression and HDL formation are impaired in the lysosomal cholesterol storage disorder Niemann-Pick disease type C1 and that plasma HDL-C is low in the majority of Niemann-Pick disease type C patients. Here, we show that ABCA1 regulation and activity are also impaired in cholesteryl ester storage disease (CESD), caused by mutations in the LIPA gene that result in less than 5% of normal lysosomal acid lipase (LAL) activity. Fibroblasts from patients with CESD showed impaired up-regulation of ABCA1 in response to low density lipoprotein (LDL) loading, reduced phospholipid and cholesterol efflux to apolipoprotein A-I, and reduced α-HDL particle formation. Treatment of normal fibroblasts with chloroquine to inhibit LAL activity reduced ABCA1 expression and activity, similar to that of CESD cells. Liver X receptor agonist treatment of CESD cells corrected ABCA1 expression but failed to correct LDL cholesteryl ester hydrolysis and cholesterol efflux to apoA-I. LDL-induced production of 27-hydroxycholesterol was reduced in CESD compared with normal fibroblasts. Treatment with conditioned medium containing LAL from normal fibroblasts or with recombinant human LAL rescued ABCA1 expression, apoA-I-mediated cholesterol efflux, HDL particle formation, and production of 27-hydroxycholesterol by CESD cells. These results provide further evidence that the rate of release of cholesterol from late endosomes/lysosomes is a critical regulator of ABCA1 expression and activity, and an explanation for the hypoalphalipoproteinemia seen in CESD patients.</AbstractText>
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Lysosomal acid lipase deficiency impairs regulation of ABCA1 gene and formation of high density lipoproteins in cholesteryl ester storage disease.

Lysosomal acid lipase deficiency impairs regulation of ABCA1 gene and formation of high density lipoproteins in cholesteryl ester storage disease.

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