Altered cholesterol homeostasis in cultured and in vivo models of cystic fibrosis.

Altered cholesterol homeostasis in cultured and in vivo models of cystic fibrosis. Research Abstract Details 

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Altered cholesterol homeostasis in cultured and in vivo models of cystic fibrosis. Abstract Text:

Nicole M White,Dechen Jiang,James D Burgess,Ilya R Bederman,Stephen F Previs,Thomas J Kelley,

Determining how the regulation of cellular processes is impacted in cystic fibrosis (CF) is fundamental to understanding disease pathology and to identifying new therapeutic targets. In this study, unesterified cholesterol accumulation is observed in lung and trachea sections obtained from CF patients compared with non-CF tissues, suggesting an inherent flaw in cholesterol processing. An alternate staining method utilizing a fluorescent cholesterol probe also indicates improper lysosomal storage of cholesterol in CF cells. Excess cholesterol is also manifested by a significant increase in plasma membrane cholesterol content in both cultured CF cells and in nasal tissue excised from cftr(-/-) mice. Impaired intracellular cholesterol movement is predicted to stimulate cholesterol synthesis, a hypothesis supported by the observation of increased de novo cholesterol synthesis in lung and liver of cftr(-/-) mice compared with controls. Furthermore, pharmacological inhibition of cholesterol transport is sufficient to cause CF-like elevation in cytokine production in wild-type cells in response to bacterial challenge but has no effect in CF cells. These data demonstrate via multiple methods in both cultured and in vivo models that cellular cholesterol homeostasis is inherently altered in CF. This perturbation of cholesterol homeostasis represents a potentially important process in CF pathogenesis.

Altered cholesterol homeostasis in cultured and in vivo models of cystic fibrosis. Publishing Authors By Initials

NM White,D Jiang,JD Burgess,IR Bederman,SF Previs,TJ Kelley,

For similar peptides: intracellular signaling peptides and proteins: adaptor proteins, signal transducing: smad proteins: smad proteins, receptor-regulated: smad3 protein research abstracts see: peptides: intracellular signaling peptides and proteins: adaptor proteins, signal transducing: smad proteins: smad proteins, receptor-regulated: smad3 protein research

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Altered cholesterol homeostasis in cultured and in vivo models of cystic fibrosis. Journal Published:

PUBLICATION TYPE: Research Support, Non-U.S. Gov

Journal: American journal of physiology. Lung cellular and

VOLUME: 292

Page Numbers: L476-86

Journal Abbreviation: Am. J. Physiol. Lung Cell Mol.

ISSN: 1040-0605

DAY: 3

MONTH: 11

YEAR: 2006

Altered cholesterol homeostasis in cultured and in vivo models of cystic fibrosis. Information

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LANGUAGE: eng

NlmUniqueID: 100901229

Altered cholesterol homeostasis in cultured and in vivo models of cystic fibrosis. Keywords Mesh Terms:

KEYWORDS: Smad3 Protein

MESH TERMS: metabolism

Chemical & Substance for Abstract: Altered cholesterol homeostasis in cultured and in vivo models of cystic fibrosis. Information

Substance Name: Nitric Oxide Synthase Type II

Registry Number: EC 1.14.13.39

Grant and Affiliation Information for Altered cholesterol homeostasis in cultured and in vivo models of cystic fibrosis.

AFFILIATION: Department of Pediatrics and Pharmacology, Case Western Reserve University and Rainbow Babies and Children's Hospital, Cleveland, Ohio 44106, USA.

Country: United States

AGENCY: United States NIDDK

GRANT: P30-DK-27651

ACRONYM: DK

MEDLINETA: Am J Physiol Lung Cell Mol Phy

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