Phytanoyl-CoA (BioDeep_00000004833)

   

human metabolite Endogenous


代谢物信息卡片


{[(2R,3S,4R,5R)-5-(6-amino-9H-purin-9-yl)-4-hydroxy-2-({[hydroxy({hydroxy[3-hydroxy-2,2-dimethyl-3-({2-[(2-{[(3S,7R,11R)-3,7,11,15-tetramethylhexadecanoyl]sulfanyl}ethyl)carbamoyl]ethyl}carbamoyl)propoxy]phosphoryl}oxy)phosphoryl]oxy}methyl)oxolan-3-yl]oxy}phosphonic acid

化学式: C41H74N7O17P3S (1061.4075)
中文名称:
谱图信息: 最多检出来源 not specific(not specific) 0%

分子结构信息

SMILES: CC(C)CCCC(C)CCCC(C)CCCC(C)CC(=O)SCCNC(=O)CCNC(=O)C(C(C)(C)COP(=O)(O)OP(=O)(O)OCC1C(C(C(O1)N2C=NC3=C(N=CN=C32)N)O)OP(=O)(O)O)O
InChI: InChI=1S/C41H74N7O17P3S/c1-26(2)11-8-12-27(3)13-9-14-28(4)15-10-16-29(5)21-32(50)69-20-19-43-31(49)17-18-44-39(53)36(52)41(6,7)23-62-68(59,60)65-67(57,58)61-22-30-35(64-66(54,55)56)34(51)40(63-30)48-25-47-33-37(42)45-24-46-38(33)48/h24-30,34-36,40,51-52H,8-23H2,1-7H3,(H,43,49)(H,44,53)(H,57,58)(H,59,60)(H2,42,45,46)(H2,54,55,56)

描述信息

Phytanoyl CoA is a coenzyme A derivative of phytanic acid. Phytanic acid is present in human diet or in animal tissues where it may be derived from chlorophyll in plant extracts. Specifically it is an epimeric metabolite of the isoprenoid side chain of chlorophyll. Owing to the presence of its epimeric beta-methyl group, phytanic acid cannot be metabolized by beta-oxidation. Instead, it is metabolized in peroxisomes via alpha-oxidation to give pristanic acid, which is then oxidized by beta-oxidation. PhyH (phytanoyl-CoA 2-hydroxylase) catalyses hydroxylation of phytanoyl-CoA. Mutations of PhyH can lead to phytanic acid accumulation. High levels of phytanic acid are found in patients suffering from Refsums syndrome. This inherited neurological disorder is characterized by an accumulation of phytanic acid in blood and tissues. Clinically it is characterized by adult onset retinitis pigmentosa, anosmia, sensory neuropathy, and phytanic acidaemia. This disorder has been found to be related to deficiency in the α-oxidation pathway in the liver. (PMID: 17956235). Phytanoyl CoA and other branched-chain fatty acid CoA products are potent inducers of the peroxisome proliferator-activated receptor PPARalpha, a nuclear receptor that enhances transcription of peroxisomal enzymes mediating beta-oxidation of these potentially toxic fatty acids (PMID: 16768463). Pyruvate dehydrogenase and 2-oxoglutarate dehydrogenase are strongly inhibited by phytanoyl-CoA. Decreased activity of these important mitochondrial metabolism complexes might therefore contribute to neurological symptoms upon accumulation of phytanic acid in Refsum disease (PMID: 16737698). [HMDB]
Phytanoyl CoA is a coenzyme A derivative of phytanic acid. Phytanic acid is present in human diet or in animal tissues where it may be derived from chlorophyll in plant extracts. Specifically it is an epimeric metabolite of the isoprenoid side chain of chlorophyll. Owing to the presence of its epimeric beta-methyl group, phytanic acid cannot be metabolized by beta-oxidation. Instead, it is metabolized in peroxisomes via alpha-oxidation to give pristanic acid, which is then oxidized by beta-oxidation. PhyH (phytanoyl-CoA 2-hydroxylase) catalyses hydroxylation of phytanoyl-CoA. Mutations of PhyH can lead to phytanic acid accumulation. High levels of phytanic acid are found in patients suffering from Refsums syndrome. This inherited neurological disorder is characterized by an accumulation of phytanic acid in blood and tissues. Clinically it is characterized by adult onset retinitis pigmentosa, anosmia, sensory neuropathy, and phytanic acidaemia. This disorder has been found to be related to deficiency in the α-oxidation pathway in the liver. (PMID: 17956235). Phytanoyl CoA and other branched-chain fatty acid CoA products are potent inducers of the peroxisome proliferator-activated receptor PPARalpha, a nuclear receptor that enhances transcription of peroxisomal enzymes mediating beta-oxidation of these potentially toxic fatty acids (PMID: 16768463). Pyruvate dehydrogenase and 2-oxoglutarate dehydrogenase are strongly inhibited by phytanoyl-CoA. Decreased activity of these important mitochondrial metabolism complexes might therefore contribute to neurological symptoms upon accumulation of phytanic acid in Refsum disease (PMID: 16737698).

同义名列表

14 个代谢物同义名

{[(2R,3S,4R,5R)-5-(6-amino-9H-purin-9-yl)-4-hydroxy-2-({[hydroxy({hydroxy[3-hydroxy-2,2-dimethyl-3-({2-[(2-{[(3S,7R,11R)-3,7,11,15-tetramethylhexadecanoyl]sulfanyl}ethyl)carbamoyl]ethyl}carbamoyl)propoxy]phosphoryl}oxy)phosphoryl]oxy}methyl)oxolan-3-yl]oxy}phosphonic acid; [(2R,3S,4R,5R)-5-(6-aminopurin-9-yl)-4-hydroxy-2-[({hydroxy[hydroxy(3-hydroxy-2,2-dimethyl-3-({2-[(2-{[(3S,7R,11R)-3,7,11,15-tetramethylhexadecanoyl]sulfanyl}ethyl)carbamoyl]ethyl}carbamoyl)propoxy)phosphoryl]oxyphosphoryl}oxy)methyl]oxolan-3-yl]oxyphosphonic acid; [(2S,3S,4R,5R)-5-(6-aminopurin-9-yl)-4-hydroxy-2-[[hydroxy-[hydroxy-[3-hydroxy-2,2-dimethyl-3-[2-[2-[(3S,7S,11R)-3,7,11,15-tetramethylhexadecanoyl]sulfanylethylcarbamoyl]ethylcarbamoyl]propoxy]phosphoryl]oxy-phosphoryl]oxymethyl]oxolan-3-yl]oxyphosphonic acid; (3S,7S,11S,15-tetraethylhexadecanoate)-coenzyme A; 3,7,11,15-Tetramethyl hexadecanoyl coenzyme A; Phytanoyl-coenzyme A; (Acyl-CoA); [M+H]+;; 3,7,11,15-Tetramethyl hexadecanoyl CoA; Phytanoyl coenzyme A; Phytanoyl-coenzyme A; Phytanyl coenzyme A; Phytanoyl CoA; Phytanoyl-CoA; CID 60210075; Phytanyl CoA



数据库引用编号

15 个数据库交叉引用编号

分类词条

相关代谢途径

Reactome(0)

BioCyc(0)

PlantCyc(0)

代谢反应

15 个相关的代谢反应过程信息。

Reactome(0)

BioCyc(0)

WikiPathways(0)

Plant Reactome(0)

INOH(0)

PlantCyc(0)

COVID-19 Disease Map(0)

PathBank(15)

PharmGKB(0)

1 个相关的物种来源信息

在这里通过桑基图来展示出与当前的这个代谢物在我们的BioDeep知识库中具有相关联信息的其他代谢物。在这里进行关联的信息来源主要有：

  • PubMed: 来源于PubMed文献库中的文献信息，我们通过自然语言数据挖掘得到的在同一篇文献中被同时提及的相关代谢物列表，这个列表按照代谢物同时出现的文献数量降序排序，取前10个代谢物作为相关研究中关联性很高的代谢物集合展示在桑基图中。
  • NCBI Taxonomy: 通过文献数据挖掘，得到的代谢物物种来源信息关联。这个关联信息同样按照出现的次数降序排序，取前10个代谢物作为高关联度的代谢物集合展示在桑吉图上。
  • Chemical Taxonomy: 在物质分类上处于同一个分类集合中的其他代谢物
  • Chemical Reaction: 在化学反应过程中，存在为当前代谢物相关联的生化反应过程中的反应底物或者反应产物的关联代谢物信息。

点击图上的相关代谢物的名称，可以跳转到相关代谢物的信息页面。

亚细胞结构定位 关联基因列表
Cytoplasm 6 ACSBG1, DYRK1A, MARK2, PHYHIP, PHYHIPL, SCP2
Peripheral membrane protein 1 CRAT
Endoplasmic reticulum membrane 1 HSP90B1
Nucleus 4 DYRK1A, HSP90B1, PPARA, SCP2
cytosol 10 ACOT6, ACOT8, ACOX3, ACSBG1, CRAT, HACL1, HSP90B1, PECR, PHYH, SCP2
dendrite 2 DYRK1A, MARK2
mitochondrial membrane 1 IVD
nucleoplasm 6 DYRK1A, HACL1, IVD, MARK2, PPARA, SCP2
Cell membrane 2 ACSBG1, GPR34
Multi-pass membrane protein 1 GPR34
mitochondrial inner membrane 2 CRAT, L2HGDH
smooth endoplasmic reticulum 1 HSP90B1
plasma membrane 4 ACSBG1, GPR34, MARK2, P2RY10
Membrane 6 ACOX3, GPR34, HSP90B1, L2HGDH, MARK2, SCP2
axon 1 DYRK1A
extracellular exosome 1 HSP90B1
endoplasmic reticulum 4 ACSBG1, CRAT, HSP90B1, SCP2
perinuclear region of cytoplasm 1 HSP90B1
mitochondrion 6 CRAT, IVD, L2HGDH, MARK2, PECR, SCP2
protein-containing complex 2 HSP90B1, SCP2
intracellular membrane-bounded organelle 1 HACL1
extracellular region 1 HSP90B1
Mitochondrion matrix 1 IVD
mitochondrial matrix 1 IVD
cytoplasmic vesicle 1 ACSBG1
midbody 1 HSP90B1
Mitochondrion inner membrane 1 CRAT
Matrix side 1 CRAT
focal adhesion 1 HSP90B1
Peroxisome 6 ACOX3, CRAT, HACL1, PECR, PHYH, SCP2
Peroxisome matrix 1 ACOT8
peroxisomal matrix 7 ACOT6, ACOT8, ACOX3, CRAT, HACL1, PHYH, SCP2
peroxisomal membrane 1 PECR
collagen-containing extracellular matrix 1 HSP90B1
lateral plasma membrane 1 MARK2
nuclear speck 1 DYRK1A
chromatin 1 PPARA
Chromosome 1 SCP2
cytoskeleton 1 DYRK1A
[Isoform 1]: Mitochondrion 1 CRAT
condensed chromosome, centromeric region 1 SCP2
actin filament 1 MARK2
lateral element 1 SCP2
Cell projection, dendrite 1 MARK2
Melanosome 1 HSP90B1
Nucleus speckle 1 DYRK1A
sperm plasma membrane 1 HSP90B1
Microsome 1 ACSBG1
endoplasmic reticulum lumen 1 HSP90B1
[Isoform 2]: Cytoplasm 1 ACOT6
9+0 non-motile cilium 1 PHYH
Sarcoplasmic reticulum lumen 1 HSP90B1
ribonucleoprotein complex 1 DYRK1A
synaptonemal complex 1 SCP2
endocytic vesicle lumen 1 HSP90B1
endoplasmic reticulum chaperone complex 1 HSP90B1
[Isoform SCP2]: Peroxisome 1 SCP2
[Isoform SCPx]: Peroxisome 1 SCP2
[Isoform 2]: Peroxisome 1 CRAT
microtubule bundle 1 MARK2
[Isoform 1]: Peroxisome 1 ACOT6


文献列表

  • Wentao Yang, Philipp Gutbrod, Katharina Gutbrod, Helga Peisker, Xiaoning Song, Anna-Lena Falz, Andreas J Meyer, Peter Dörmann. 2-Hydroxy-phytanoyl-CoA lyase (AtHPCL) is involved in phytol metabolism in Arabidopsis. The Plant journal : for cell and molecular biology. 2022 03; 109(5):1290-1304. doi: 10.1111/tpj.15632. [PMID: 34902195]
  • Wagner L Araújo, Kimitsune Ishizaki, Adriano Nunes-Nesi, Takayuki Tohge, Tony R Larson, Ina Krahnert, Ilse Balbo, Sandra Witt, Peter Dörmann, Ian A Graham, Christopher J Leaver, Alisdair R Fernie. Analysis of a range of catabolic mutants provides evidence that phytanoyl-coenzyme A does not act as a substrate of the electron-transfer flavoprotein/electron-transfer flavoprotein:ubiquinone oxidoreductase complex in Arabidopsis during dark-induced senescence. Plant physiology. 2011 Sep; 157(1):55-69. doi: 10.1104/pp.111.182188. [PMID: 21788362]
  • Tomoyuki Fukasawa, Koichiro Murashima, Tomoko Nemoto, Ichiro Matsumoto, Jinichiro Koga, Hidetoshi Kubota, Minoru Kanegae. Identification of marker genes for lipid-lowering effect of a short-chain fructooligosaccharide by DNA microarray analysis. Journal of dietary supplements. 2009; 6(3):254-62. doi: 10.1080/19390210903070822. [PMID: 22435477]
  • Veerle Foulon, Stanny Asselberghs, Wendy Geens, Guy P Mannaerts, Minne Casteels, Paul P Van Veldhoven. Further studies on the substrate spectrum of phytanoyl-CoA hydroxylase: implications for Refsum disease?. Journal of lipid research. 2003 Dec; 44(12):2349-55. doi: 10.1194/jlr.m300230-jlr200. [PMID: 12923223]
  • N M Verhoeven, R J Wanders, D S Schor, G A Jansen, C Jakobs. Phytanic acid alpha-oxidation: decarboxylation of 2-hydroxyphytanoyl-CoA to pristanic acid in human liver. Journal of lipid research. 1997 Oct; 38(10):2062-70. doi: . [PMID: 9374128]
  • P A Watkins, A E Howard, S J Gould, J Avigan, S J Mihalik. Phytanic acid activation in rat liver peroxisomes is catalyzed by long-chain acyl-CoA synthetase. Journal of lipid research. 1996 Nov; 37(11):2288-95. doi: . [PMID: 8978480]
  • K Pahan, M Khan, I Singh. Phytanic acid oxidation: normal activation and transport yet defective alpha-hydroxylation of phytanic acid in peroxisomes from Refsum disease and rhizomelic chondrodysplasia punctata. Journal of lipid research. 1996 May; 37(5):1137-43. doi: . [PMID: 8725164]
  • J E De Larco, D A Pigott. Ectopic peptides released by a human melanoma cell line that modulate the transformed phenotype. Ciba Foundation symposium. 1985; 116(?):224-40. doi: 10.1002/9780470720974.ch14. [PMID: 3000706]