LMPD Database

LMP000928

UniProt Annotations

Entry Information
Gene Nameelongation of very long chain fatty acids (FEN1/Elo2, SUR4/Elo3, yeast)-like 3
Protein EntryELOV3_MOUSE
UniProt IDO35949
SpeciesMouse
Comments
Comment typeDescription
Catalytic ActivityA very-long-chain acyl-CoA + malonyl-CoA = CoA + a very-long-chain 3-oxoacyl-CoA + CO(2).
Disruption PhenotypeMutant mice grow normally and are fertile. They display a sparse hair coat, a hyperplastic pilosebaceous system and their hair lipid content is disturbed with exceptionally high levels of eicosenoic acid (20:1). In the triglyceride fraction, fatty acids longer than 20 carbon atoms are almost undetectable. As a result, mice exhibited a severe defect in water repulsion and increased trans-epidermal water loss. When exposed to cold stress, mutants exhibit a significantly reduced VLCFA elongation activity in brown adipose tissue, but only during the initial phase. Cold-acclimated mutants are equally efficient as normal mice at elongating fatty acids. Mutant mice are lean and resistant to diet-induced weight gain, they show normal food intake but increased metabolic rate, and show reduced hepatic lipogenesis and triglycerides synthesis. {ECO:0000269|PubMed:14581464, ECO:0000269|PubMed:16326704, ECO:0000269|PubMed:20605947}.
FunctionCondensing enzyme that elongates saturated and monounsaturated very long chain fatty acids (VLCFAs) of up to 24 carbon atoms. Participates in the formation of certain VLCFA and triglycerides in certain cells of the hair follicles and the sebaceous glands, required for skin barrier function. Critical enzyme for lipid accumulation and metabolic activity in brown adipocytes during the early phase of the tissue recruitment. Play a role in lipid storage and in resistance to diet-induced obesity. {ECO:0000269|PubMed:10791983, ECO:0000269|PubMed:14581464, ECO:0000269|PubMed:16326704, ECO:0000269|PubMed:20605947}.
InductionStrongly up-regulated in brown adipose tissue in conditions of brown fat recruitment, such as cold stress, perinatal development and after diet-induced thermogenesis. A synergistic action of both catecholamines and glucocorticoids is required for the induction.
SimilarityBelongs to the ELO family. {ECO:0000305}.
Subcellular LocationEndoplasmic reticulum membrane {ECO:0000269|PubMed:10429212}; Multi-pass membrane protein {ECO:0000269|PubMed:10429212}.
Tissue SpecificityExpressed in brown adipose tissue and liver. In the skin, strong expressed in the cells of the inner layer of the outer root sheath of the hair follicles and in the sebocytes of the sebaceous glands. Hardly detectable in the epidermis and not at all in fibroblasts. {ECO:0000269|PubMed:14581464, ECO:0000269|PubMed:9395518}.