Fatty acid alterations and carboxylase deficiencies in the skin of biotin-deficient rats.
Full-thickness biopsies of haired and alopecic skin of biotin-deficient rats had less subcutaneous fat and showed lipophilic follicular plugging, vascular engorgement, epidermal hyperplasia, and abnormal keratinization. Mean activities of the three mitochondrial biotin-dependent carboxylases in the skin of biotin-deficient animals were reduced to 3-18% of control whereas the cytosolic enzyme, acetyl-CoA carboxylase, was reduced only to 38-61%. The total fatty acid content of haired and alopecic skin of deficient rats was 30% of those in the corresponding skin sites of control animals. Skin from deficient rats contained less of several long-chain fatty acids (16:0, 16:1, 18:0, 18:1, and 18:2) and more very-long-chain fatty acid, particularly 24:1 and 26:1. These alterations in fatty acids in biotin-deficient rats suggest that the skin findings in biotin and biotinidase deficiencies in humans may be due to similar fatty acid changes.
Proud
V K
VK
Department of Human Genetics, Medical College of Virginia, Richmond 23298-0033.
Rizzo
W B
WB
Patterson
J W
JW
Heard
G S
GS
Wolf
B
B
D000818:Animals; D001710:Biotin / Q000008:administration & dosage / Q000172:deficiency*; D002265:Carboxylic Ester Hydrolases / Q000378:metabolism*; D005227:Fatty Acids / Q000378:metabolism*; D051381:Rats; D011919:Rats, Inbred Strains; D012867:Skin / Q000201:enzymology / Q000378:metabolism* / Q000473:pathology
D005227:Fatty Acids; D001710:Biotin; D002265:Carboxylic Ester Hydrolases