The world's first wiki where authorship really matters (Nature Genetics, 2008). Due credit and reputation for authors. Imagine a global collaborative knowledge base for original thoughts. Search thousands of articles and collaborate with scientists around the globe.

wikigene or wiki gene protein drug chemical gene disease author authorship tracking collaborative publishing evolutionary knowledge reputation system wiki2.0 global collaboration genes proteins drugs chemicals diseases compound
Hoffmann, R. A wiki for the life sciences where authorship matters. Nature Genetics (2008)



Gene Review

HACL1  -  2-hydroxyacyl-CoA lyase 1

Homo sapiens

Synonyms: 2-HPCL, 2-hydroxyphytanoyl-CoA lyase, HPCL, HPCL2, HSPC279, ...
Welcome! If you are familiar with the subject of this article, you can contribute to this open access knowledge base by deleting incorrect information, restructuring or completely rewriting any text. Read more.

Disease relevance of HACL1

  • Very recently, a nontumorigenic liver epithelial cell line (HACL-1) with a finite life-span and expressing a number of hepatocyte-specific markers was established from a human hepatocellular adenoma in our laboratory [1].
  • The effect of thiamine deficiency on 2-HPCL and alpha-oxidation has not been studied, nor have possible adverse effects of deficient alpha-oxidation been considered in the pathogenesis of diseases associated with thiamine shortage, such as thiamine-responsive megaloblastic anemia (TRMA) [2].

High impact information on HACL1

  • Purification, molecular cloning, and expression of 2-hydroxyphytanoyl-CoA lyase, a peroxisomal thiamine pyrophosphate-dependent enzyme that catalyzes the carbon-carbon bond cleavage during alpha-oxidation of 3-methyl-branched fatty acids [3].
  • To analyze the role of mutated p53 in the immortalization of human liver cells, we transfected HACL-1 cells with an expression vector containing a human p53 complementary DNA mutated at the third base of codon 249 and analyzed the consequences of this gene transfer on the growth properties of this cell line [1].
  • One peptide, HIPDVITY, is encoded by squalene synthase, and the other one, QFADVIVLF, is encoded by 2-hydroxyphytanoyl-CoA lyase [4].
  • The HACL-1 cells have a finite life span (i.e., they proliferate for a period of 2 months and then senesce), show cell-cell contact inhibition, do not grow in soft agar, are not tumorigenic when injected in nude mice, and possess a normal diploid karyotype [5].
  • The identification of 2-hydroxyphytanoyl-CoA lyase (2-HPCL), a thiamine pyrophosphate (TPP)-dependent peroxisomal enzyme involved in the alpha-oxidation of phytanic acid and of 2-hydroxy straight chain fatty acids, pointed towards a role of TPP in these processes [2].

Anatomical context of HACL1


Associations of HACL1 with chemical compounds

  • Concentrations of this steroid were then measured using radioimmunoassay upon which analysis of HPCL and gas chromatograms permitted the calculation of the individual pregnenolone ester contributions within the samples [9].
  • Selected food items in edible form were analyzed for the coenzyme Q content by HPCL with UV-detection, and their contribution to the total intake calculated [10].
  • We have studied the second step in the alpha-oxidation pathway, which involves conversion of 2-hydroxyphytanoyl-CoA to pristanal catalysed by 2-hydroxyphytanoyl-CoA lyase [11].
  • METHODS: The Leiden mutation was measured by the method of polymerase chain reaction, the retinoid level in the patient's serum was measured by high liquid cromathografic method (HPCL) [12].

Other interactions of HACL1


Analytical, diagnostic and therapeutic context of HACL1

  • METHODS: 5HT (platelet rich plasma, platelet poor plasma, urine, HPLC with electrochemical detection) and 5HIAA (plasma, urine, HPCL with electrochemical detection) levels were evaluated in 14 conservatively treated (CT) and 12 hemodialysed (HD) patients with CRI and were compared to those of 60 healthy volunteers (HV) [14].
  • Cell fractionation studies demonstrate that in rat liver 2-hydroxyphytanoyl-CoA lyase is localised in peroxisomes [11].


  1. The human p53 gene mutated at position 249 per se is not sufficient to immortalize human liver cells. Schleger, C., Becker, R., Oesch, F., Steinberg, P. Hepatology (1999) [Pubmed]
  2. Thiamine pyrophosphate: an essential cofactor for the alpha-oxidation in mammals--implications for thiamine deficiencies? Sniekers, M., Foulon, V., Mannaerts, G.P., Van Maldergem, L., Mandel, H., Gelb, B.D., Casteels, M., Van Veldhoven, P.P. Cell. Mol. Life Sci. (2006) [Pubmed]
  3. Purification, molecular cloning, and expression of 2-hydroxyphytanoyl-CoA lyase, a peroxisomal thiamine pyrophosphate-dependent enzyme that catalyzes the carbon-carbon bond cleavage during alpha-oxidation of 3-methyl-branched fatty acids. Foulon, V., Antonenkov, V.D., Croes, K., Waelkens, E., Mannaerts, G.P., Van Veldhoven, P.P., Casteels, M. Proc. Natl. Acad. Sci. U.S.A. (1999) [Pubmed]
  4. A human CTL recognizes a caspase-8-derived peptide on autologous HLA-B*3503 molecules and two unrelated peptides on allogeneic HLA-B*3501 molecules. Mandruzzato, S., Stroobant, V., Demotte, N., van der Bruggen, P. J. Immunol. (2000) [Pubmed]
  5. Establishment and characterization of a nontumorigenic cell line derived from a human hepatocellular adenoma expressing hepatocyte-specific markers. Schleger, C., Heck, R., Niketeghad, F., Schirmacher, P., Radaeva, S., Oesch, F., Dienes, H.P., Bannasch, P., Steinberg, P. Exp. Cell Res. (1997) [Pubmed]
  6. Identification of pristanal dehydrogenase activity in peroxisomes: conclusive evidence that the complete phytanic acid alpha-oxidation pathway is localized in peroxisomes. Jansen, G.A., van den Brink, D.M., Ofman, R., Draghici, O., Dacremont, G., Wanders, R.J. Biochem. Biophys. Res. Commun. (2001) [Pubmed]
  7. Purification and characterization of defensins from cystic fibrosis sputum. Soong, L.B., Ganz, T., Ellison, A., Caughey, G.H. Inflamm. Res. (1997) [Pubmed]
  8. Metabolism of dextromethorphan in human liver microsomes: a rapid HPCL assay to monitor cytochrome P450 2D6 activity. Vielnascher, E., Spatzenegger, M., Mayrhofer, A., Klinger, P., Jäger, W. Die Pharmazie. (1996) [Pubmed]
  9. Presence of fatty acid esters of pregnenolone in follicular fluid from women undergoing follicle stimulation. Roy, R., Bélanger, A. Steroids (1989) [Pubmed]
  10. The coenzyme Q10 content of the average Danish diet. Weber, C., Bysted, A., Hłlmer, G. International journal for vitamin and nutrition research. Internationale Zeitschrift für Vitamin- und Ernährungsforschung. Journal international de vitaminologie et de nutrition. (1997) [Pubmed]
  11. Phytanic acid alpha-oxidation: identification of 2-hydroxyphytanoyl-CoA lyase in rat liver and its localisation in peroxisomes. Jansen, G.A., Verhoeven, N.M., Denis, S., Romeijn, G., Jakobs, C., ten Brink, H.J., Wanders, R.J. Biochim. Biophys. Acta (1999) [Pubmed]
  12. Leiden mutation (as genetic) and environmental (retinoids) sequences in the acute and chronic inflammatory and premalignant colon disease in human gastrointestinal tract. Mózsik, G., Nagy, Z., Nagy, A., Rumi, G., Karádi, O., Czimmer, J., Matus, Z., Tóth, G., Pár, A. J. Physiol. Paris (2001) [Pubmed]
  13. Phytanic acid alpha-oxidation in man: identification of 2-hydroxyphytanoyl-CoA lyase, a peroxisomal enzyme with normal activity in Zellweger syndrome. Jansen, G.A., Denis, S., Verhoeven, N.M., Jakobs, C., Wanders, R.J. J. Inherit. Metab. Dis. (2000) [Pubmed]
  14. Serotonin and 5-hydroxyindole-acetic acid. Sebekova, K., Spustova, V., Opatrny, K., Dzurik, R. Bratislavské lekárske listy. (2001) [Pubmed]
WikiGenes - Universities