Publication: Mitochondrial cholesterol in health and disease
Authors
Garcia-Ruiz, Carmen ; Mari, Monserrat ; Colell, Ana ; Morales, Albert ; Caballero, Francisco ; Montero, Joan ; Terrones, Ohiana ; Basañez, Gorka ; Fernández-Checa, José C.
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Publisher
Murcia : F. Hernández
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DOI
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info:eu-repo/semantics/article
Description
Abstract
Cholesterol is a critical component of
biological membranes, which not only plays an essential
role in determining membrane physical properties, but
also in the regulation of multiple signaling pathways.
Cells satisfy their need for cholesterol either by uptake
from nutrients and lipoproteins or de novo synthesis
from acetyl-CoA. The latter process occurs in the
endoplasmic reticulum, where transcription factors that
regulate the expression of enzymes involved in the de
novo cholesterol synthesis reside. Cholesterol is
distributed to different membranes most prominently to
plasma membrane, where it participates in the physical
organization of specific membrane domains.
Mitochondria, however, are considered cholesterol-poor
organelles, and obtain their cholesterol load by the action
of specialized proteins involved in its delivery from
extramitochondrial sources and trafficking within
mitochondrial membranes. Although mitochondrial
cholesterol fulfills vital physiological functions, such as
the synthesis of bile acids in the liver or the formation of
steroid hormones in specialized tissues, recent evidence
indicates that the accumulation of cholesterol in
mitochondria may be a key step in disease progression,
including steatohepatitis, carcinogenesis or Alzheimer
disease.
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