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Gene Review

Phyh  -  phytanoyl-CoA hydroxylase

Mus musculus

Synonyms: AI256161, AI265699, Ln1, Lnap1, Lupus nephritis-associated peptide 1, ...
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Disease relevance of Phyh


High impact information on Phyh

  • We previously reported that bone morphogenetic proteins (BMPs), members of the transforming growth factor superfamily, together with the basement membrane glycoprotein laminin-1 (Ln-1), promote proliferation of fetal pancreatic cells and formation of colonies containing peripheral insulin-positive cells [2].
  • It was determined that brown fat and brown adipocytes are sites of high gene expression of phytanoyl-CoA hydroxylase, the enzyme required for initiation of peroxisomal alpha-oxidation of phytanic acid [3].
  • Previously, we identified a novel neuron-specific protein (PAHX-AP1) that binds to Refsum disease gene product (PAHX), and we developed transgenic (TG) mice that overexpress heart-targeted PAHX-AP1 [4].

Analytical, diagnostic and therapeutic context of Phyh

  • By RT-PCR, receptors for BMP (BMPR) (excepting BMPR-1B) and Ln-1 were expressed in the fetal pancreas between E13.5 and E17 [2].
  • To understand the structural basis of laminins' functions, we examined the motion of laminin-1 (Ln-1) in physiological buffers using atomic force microscopy [5].
  • Similarly, quantitative PCR demonstrated a 4-fold increase in phytanoyl-CoA alpha hydroxylase (PAHX) and fatty acyl-CoA oxidase (FACO) mRNA levels during differentiation [6].


  1. Cardiac Characteristics of Transgenic Mice Overexpressing Refsum Disease Gene-Associated Protein within the Heart. Koh, J.T., Choi, H.H., Ahn, K.Y., Kim, J.U., Kim, J.H., Chun, J.Y., Baik, Y.H., Kim, K.K. Biochem. Biophys. Res. Commun. (2001) [Pubmed]
  2. Convergence of bone morphogenetic protein and laminin-1 signaling pathways promotes proliferation and colony formation by fetal mouse pancreatic cells. Jiang, F.X., Harrison, L.C. Exp. Cell Res. (2005) [Pubmed]
  3. Phytanic acid, but not pristanic acid, mediates the positive effects of phytol derivatives on brown adipocyte differentiation. Schluter, A., Giralt, M., Iglesias, R., Villarroya, F. FEBS Lett. (2002) [Pubmed]
  4. Changes underlying arrhythmia in the transgenic heart overexpressing Refsum disease gene-associated protein. Koh, J.T., Jeong, B.C., Kim, J.H., Ahn, Y.K., Lee, H.S., Baik, Y.H., Kim, K.K. Biochem. Biophys. Res. Commun. (2004) [Pubmed]
  5. Structures and dynamic motion of laminin-1 as observed by atomic force microscopy. Chen, C.H., Clegg, D.O., Hansma, H.G. Biochemistry (1998) [Pubmed]
  6. Sequential ordered fatty acid alpha oxidation and Delta9 desaturation are major determinants of lipid storage and utilization in differentiating adipocytes. Su, X., Han, X., Yang, J., Mancuso, D.J., Chen, J., Bickel, P.E., Gross, R.W. Biochemistry (2004) [Pubmed]
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