Fatty acid transporters in skin development, function and disease.

[aniridia]

Fatty acids in the epidermis can be incorporated into complex lipids or exist in a free form , and they are crucial to proper functions of the epidermis and its appendages , such as sebaceous glands . Epidermal fatty acids can be synthesized de novo by keratinocytes or taken up from extracutaneous sources in a process that likely involves protein transporters . Several proteins that are expressed in the epidermis have been proposed to facilitate the uptake of long -chain fatty acids (LCFA ) in mammalian cells , including fatty acid translocase /CD 36 , fatty acid binding protein , and fatty acid transport protein (FATP )/very long -chain acyl-CoA synthetase . In this review , we will discuss the mechanisms by which these candidate transporters facilitate the uptake of fatty acids . We will then discuss the clinical implications of defects in these transporters and relevant animal models , including the FATP 4 animal models and ichthyosis prematurity syndrome , a congenital ichthyosis caused by FATP 4 deficiency . This article is part of a Special Issue entitled The Important Role of Lipids in the Epidermis and their Role in the Formation and Maintenance of the Cutaneous Barrier . Guest Editors : Kenneth R . Feingold and Peter Elias .

Diseases
Validation

Diseases presenting "long-chain fatty acids" symptom

adrenomyeloneuropathy

aniridia

neonatal adrenoleukodystrophy

x-linked adrenoleukodystrophy

zellweger syndrome

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