Novel biallelic FA2H mutations in a Japanese boy with fatty acid hydroxylase-associated neurodegeneration

2020 ◽  
Vol 42 (2) ◽  
pp. 217-221
Author(s):  
Masahiro Kawaguchi ◽  
Takayuki Sassa ◽  
Hiroyuki Kidokoro ◽  
Tomohiko Nakata ◽  
Kohji Kato ◽  
...  
Keyword(s):  
Fatty Acid ◽  
Fatty Acid Hydroxylase

scholarly journals LeTetR Positively Regulates 3-Hydroxylation of the Antifungal HSAF and Its Analogs in Lysobacter enzymogenes OH11

Molecules ◽  
2020 ◽  
Vol 25 (10) ◽  
pp. 2286
Author(s):  
Lingjun Yu ◽  
Vimmy Khetrapal ◽  
Fengquan Liu ◽  
Liangcheng Du
Keyword(s):  
Fatty Acid ◽  
Antifungal Activity ◽  
Biocontrol Agent ◽  
Biosynthetic Gene Cluster ◽  
E Coli ◽  
Lysobacter Enzymogenes ◽  
Heat Stable ◽  
Fatty Acid Hydroxylase ◽  
Hydroxylase Gene ◽  
Antibiotic Compounds

The biocontrol agent Lysobacter enzymogenes OH11 produces several structurally distinct antibiotic compounds, including the antifungal HSAF (Heat Stable Antifungal Factor) and alteramides, along with their 3-dehydroxyl precursors (3-deOH). We previously showed that the 3-hydroxylation is the final step of the biosynthesis and is also a key structural moiety for the antifungal activity. However, the procedure through which OH11 regulates the 3-hydroxylation is still not clear. In OH11, the gene orf3232 was predicted to encode a TetR regulator (LeTetR) with unknown function. Here, we deleted orf3232 and found that the LeTetR mutant produced very little HSAF and alteramides, while the 3-deOH compounds were not significantly affected. The production of HSAF and alteramides was restored in orf3232-complemented mutant. qRT-PCR showed that the deletion of orf3232 impaired the transcription of a putative fatty acid hydroxylase gene, orf2195, but did not directly affect the expression of the HSAF biosynthetic gene cluster (hsaf). When an enzyme extract from E. coli expressing the fatty acid hydroxylase gene, hsaf-orf7, was added to the LeTetR mutant, the production of HSAF and alteramides increased by 13–14 fold. This study revealed a rare function of the TetR family regulator, which positively controls the final step of the antifungal biosynthesis and thus controls the antifungal activity of the biocontrol agent.

scholarly journals The CYPome of Sorangium cellulosum So ce56 and Identification of CYP109D1 as a New Fatty Acid Hydroxylase

2010 ◽  
Vol 17 (12) ◽  
pp. 1295-1305 ◽  
Author(s):  
Yogan Khatri ◽  
Frank Hannemann ◽  
Kerstin M. Ewen ◽  
Dominik Pistorius ◽  
Olena Perlova ◽  
...  
Keyword(s):  
Fatty Acid ◽  
Sorangium Cellulosum ◽  
Fatty Acid Hydroxylase ◽  
Sorangium Cellulosum So Ce56

Role of Leu188 in the Fatty Acid Hydroxylase Activity of CYP102A1 from Bacillus megaterium

2016 ◽  
Vol 133 ◽  
pp. 35-42 ◽  
Author(s):  
Hyun-Hee Jang ◽  
Sun-Mi Shin ◽  
Sang Hoon Ma ◽  
Ga-Young Lee ◽  
Young Hee Joung ◽  
...  
Keyword(s):  
Fatty Acid ◽  
Bacillus Megaterium ◽  
Hydroxylase Activity ◽  
Fatty Acid Hydroxylase

Cloning and Functional Characterization of CYP94A2, a Medium Chain Fatty Acid Hydroxylase from Vicia sativa

1999 ◽  
Vol 261 (1) ◽  
pp. 156-162 ◽  
Author(s):  
Renaud Le Bouquin ◽  
Franck Pinot ◽  
Irène Benveniste ◽  
Jean-Pierre Salaün ◽  
Francis Durst
Keyword(s):  
Fatty Acid ◽  
Functional Characterization ◽  
Chain Fatty Acid ◽  
Medium Chain Fatty Acid ◽  
Vicia Sativa ◽  
Medium Chain ◽  
Fatty Acid Hydroxylase

Flavocytochrome P450 BM3 mutant W1046A is a NADH-dependent fatty acid hydroxylase: Implications for the mechanism of electron transfer in the P450 BM3 dimer

2011 ◽  
Vol 507 (1) ◽  
pp. 75-85 ◽  
Author(s):  
Hazel M. Girvan ◽  
Adrian J. Dunford ◽  
Rajasekhar Neeli ◽  
Idorenyin S. Ekanem ◽  
Timothy N. Waltham ◽  
...  
Keyword(s):  
Electron Transfer ◽  
Fatty Acid ◽  
P450 Bm3 ◽  
Fatty Acid Hydroxylase

P450 Jα : A New, Robust and α‐Selective Fatty Acid Hydroxylase Displaying Unexpected 1‐Alkene Formation

2020 ◽  
Vol 26 (68) ◽  
pp. 15910-15921
Author(s):  
Julia Armbruster ◽  
Mathilde Steinmassl ◽  
Christina A. Müller Bogotá ◽  
Gabriele Berg ◽  
Bernd Nidetzky ◽  
...  
Keyword(s):  
Fatty Acid ◽  
Fatty Acid Hydroxylase

scholarly journals PPAR/RXR Regulation of Fatty Acid Metabolism and Fatty Acid -Hydroxylase (CYP4) Isozymes: Implications for Prevention of Lipotoxicity in Fatty Liver Disease

PPAR Research ◽  
2009 ◽  
Vol 2009 ◽  
pp. 1-20 ◽  
Author(s):  
James P. Hardwick ◽  
Douglas Osei-Hyiaman ◽  
Homer Wiland ◽  
Mohamed A. Abdelmegeed ◽  
Byoung-Joon Song
Keyword(s):  
Gene Expression ◽  
Fatty Acids ◽  
Fatty Acid ◽  
Fatty Liver ◽  
Transport Proteins ◽  
Chain Fatty Acid ◽  
Fatty Acid Transport ◽  
Acid Transport ◽  
Fatty Acid Hydroxylase ◽  
Fatty Acid Transport Proteins

Fatty liver disease is a common lipid metabolism disorder influenced by the combination of individual genetic makeup, drug exposure, and life-style choices that are frequently associated with metabolic syndrome, which encompasses obesity, dyslipidemia, hypertension, hypertriglyceridemia, and insulin resistant diabetes. Common to obesity related dyslipidemia is the excessive storage of hepatic fatty acids (steatosis), due to a decrease in mitochondria -oxidation with an increase in both peroxisomal -oxidation, and microsomal -oxidation of fatty acids through peroxisome proliferator activated receptors (PPARs). How steatosis increases PPAR activated gene expression of fatty acid transport proteins, peroxisomal and mitochondrial fatty acid -oxidation and -oxidation of fatty acids genes regardless of whether dietary fatty acids are polyunsaturated (PUFA), monounsaturated (MUFA), or saturated (SFA) may be determined by the interplay of PPARs and HNF4 with the fatty acid transport proteins L-FABP and ACBP. In hepatic steatosis and steatohepatitis, the -oxidation cytochrome P450CYP4Agene expression is increased even with reduced hepatic levels of PPAR. Although numerous studies have suggested the role ethanol-inducibleCYP2E1in contributing to increased oxidative stress,Cyp2e1-null mice still develop steatohepatitis with a dramatic increase inCYP4Agene expression. This strongly implies thatCYP4Afatty acid -hydroxylase P450s may play an important role in the development of steatohepatitis. In this review and tutorial, we briefly describe how fatty acids are partitioned by fatty acid transport proteins to either anabolic or catabolic pathways regulated by PPARs, and we explore how medium-chain fatty acid (MCFA)CYP4Aand long-chain fatty acid (LCFA)CYP4F-hydroxylase genes are regulated in fatty liver. We finally propose a hypothesis that increasedCYP4Aexpression with a decrease inCYP4Fgenes may promote the progression of steatosis to steatohepatitis.

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