PMID- 10866807
OWN - NLM
STAT- MEDLINE
DCOM- 20000816
LR  - 20190620
IS  - 0014-2956 (Print)
IS  - 0014-2956 (Linking)
VI  - 267
IP  - 13
DP  - 2000 Jul
TI  - Phytanoyl-CoA hydroxylase activity is induced by phytanic acid.
PG  - 4063-7
AB  - Phytanic acid (3,7,11,15-tetramethylhexadecanoic acid) is a branched-chain fatty 
      acid present in various dietary products such as milk, cheese and fish. In 
      patients with Refsum disease, accumulation of phytanic acid occurs due to a 
      deficiency of phytanoyl-CoA hydroxylase, a peroxisomal enzyme containing a 
      peroxisomal targeting signal 2. Recently, phytanoyl-CoA hydroxylase cDNA has been 
      isolated and functional mutations have been identified. As it has been shown that 
      phytanic acid activates the nuclear hormone receptors peroxisome 
      proliferator-activated receptor (PPAR)alpha and all three retinoid X receptors 
      (RXRs), the intracellular concentration of this fatty acid should be tightly 
      regulated. When various cell lines were grown in the presence of phytanic acid, 
      the activity of phytanoyl-CoA hydroxylase increased up to four times, depending 
      on the particular cell type. In one cell line, HepG2, no induction of 
      phytanoyl-CoA hydroxylase activity was observed. After addition of phytanic acid 
      to COS-1 cells, an increase in phytanoyl-CoA hydroxylase activity was observed 
      within 2 h, indicating a quick cell response. No stimulation of phytanoyl-CoA 
      hydroxylase was observed when COS-1 cells were grown in the presence of clofibric 
      acid, 9-cis-retinoic acid or both ligands together. This indicates that the 
      activation of phytanoyl-CoA hydroxylase is not regulated via PPARalpha or RXR. 
      However, stimulation of PPARalpha and all RXRs by clofibric acid and 
      9-cis-retinoic acid was observed in transient transfection assays. These results 
      suggest that the induction of phytanoyl-CoA hydroxylase by phytanic acid does not 
      proceed via one of the nuclear hormone receptors, RXR or PPARalpha.
FAU - Zomer, A W
AU  - Zomer AW
AD  - Hubrecht Laboratory, Netherlands Institute for Developmental Biology, Utrecht, 
      the Netherlands.
FAU - Jansen, G A
AU  - Jansen GA
FAU - Van Der Burg, B
AU  - Van Der Burg B
FAU - Verhoeven, N M
AU  - Verhoeven NM
FAU - Jakobs, C
AU  - Jakobs C
FAU - Van Der Saag, P T
AU  - Van Der Saag PT
FAU - Wanders, R J
AU  - Wanders RJ
FAU - Poll-The, B T
AU  - Poll-The BT
LA  - eng
PT  - Journal Article
PL  - England
TA  - Eur J Biochem
JT  - European journal of biochemistry
JID - 0107600
RN  - 14721-66-5 (Phytanic Acid)
RN  - 1UA8E65KDZ (Alitretinoin)
RN  - 53PF01Q249 (Clofibric Acid)
RN  - 5688UTC01R (Tretinoin)
RN  - EC 1.- (Mixed Function Oxygenases)
RN  - EC 1.14.- (PHYH protein, human)
SB  - IM
MH  - Alitretinoin
MH  - Animals
MH  - COS Cells
MH  - Cattle
MH  - Clofibric Acid/pharmacology
MH  - Enzyme Induction/drug effects
MH  - Female
MH  - Mixed Function Oxygenases/*biosynthesis
MH  - Phytanic Acid/*pharmacology
MH  - Tretinoin/pharmacology
EDAT- 2000/06/24 11:00
MHDA- 2000/08/19 11:00
CRDT- 2000/06/24 11:00
PHST- 2000/06/24 11:00 [pubmed]
PHST- 2000/08/19 11:00 [medline]
PHST- 2000/06/24 11:00 [entrez]
AID - ejb1451 [pii]
AID - 10.1046/j.1432-1327.2000.01451.x [doi]
PST - ppublish
SO  - Eur J Biochem. 2000 Jul;267(13):4063-7. doi: 10.1046/j.1432-1327.2000.01451.x.