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Gene Review

SCP2  -  sterol carrier protein 2

Homo sapiens

Synonyms: NLTP, NSL-TP, Non-specific lipid-transfer protein, Propanoyl-CoA C-acyltransferase, SCP-2, ...
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Disease relevance of SCP2


High impact information on SCP2

  • Collectively, our results suggest that SCP2 is involved in promoter clearance during steroid-activated transcription [6].
  • Ser 5 phosphorylation of the RNA polymerase II CTD, a process necessary for initiation of transcription elongation, occurred significantly earlier in SCP2-silenced than parental LNCaP cells [6].
  • In cultured skin fibroblasts, the thiolytic activity of SCPx was deficient, and no SCPx protein could be detected by western blotting [7].
  • The predicted effects of the different disease-causing amino acid changes on protein structure were determined using the crystal structures of the (3R)-hydroxyacyl-coenzyme A (CoA) dehydrogenase unit of rat DBP and the 2-enoyl-CoA hydratase 2 unit and liganded sterol carrier protein 2-like unit of human DBP [8].
  • Intracellular dissemination of peroxidative stress. Internalization, transport, and lethal targeting of a cholesterol hydroperoxide species by sterol carrier protein-2-overexpressing hepatoma cells [9].

Biological context of SCP2


Anatomical context of SCP2


Associations of SCP2 with chemical compounds

  • One hypothesis has been that sterol carrier protein-2 (SCP2, also known as the non-specific lipid transfer protein) acts in cholesterol transport through the cytoplasm [18].
  • Recent studies employing gene targeting in mice showed, however, that mice lacking SCP2 and the related putative sterol carrier known as SCPx, develop a defect in peroxisomal beta-oxidation [18].
  • In addition, diminished peroxisomal alpha-oxidation of phytanic acid (3,7,11, 15-tetramethylhexadecanoic acid) in these null mice was attributed to the absence of SCP2 which has a number of properties supporting a function as carrier for fatty acyl-CoAs rather than for sterols [18].
  • In addition, 8-Br-cAMP and phorbol myristate acetate were found to increase SCPx promoter activity in a host cell-specific manner [12].
  • Sterol carrier protein-2, a new fatty acyl coenzyme A-binding protein [19].

Other interactions of SCP2


Analytical, diagnostic and therapeutic context of SCP2


  1. Structure-activity studies of human sterol carrier protein 2. Seedorf, U., Scheek, S., Engel, T., Steif, C., Hinz, H.J., Assmann, G. J. Biol. Chem. (1994) [Pubmed]
  2. Sterol carrier protein-2 is involved in cholesterol transfer from the endoplasmic reticulum to the plasma membrane in human fibroblasts. Puglielli, L., Rigotti, A., Greco, A.V., Santos, M.J., Nervi, F. J. Biol. Chem. (1995) [Pubmed]
  3. Utilization of sterol carrier protein-2 by phytanoyl-CoA 2-hydroxylase in the peroxisomal alpha oxidation of phytanic acid. Mukherji, M., Kershaw, N.J., Schofield, C.J., Wierzbicki, A.S., Lloyd, M.D. Chem. Biol. (2002) [Pubmed]
  4. Probing the ligand binding sites of fatty acid and sterol carrier proteins: effects of ethanol. Schroeder, F., Myers-Payne, S.C., Billheimer, J.T., Wood, W.G. Biochemistry (1995) [Pubmed]
  5. Overexpression of sterol carrier protein-2 mRNA in patients with cholesterol gallstones. Cui, N.Q., Zhang, S.K., Cui, Y.F., Li, D.H., Chen, C., Wu, X.Z. HBPD INT (2005) [Pubmed]
  6. Small carboxyl-terminal domain phosphatase 2 attenuates androgen-dependent transcription. Thompson, J., Lepikhova, T., Teixido-Travesa, N., Whitehead, M.A., Palvimo, J.J., Jänne, O.A. EMBO J. (2006) [Pubmed]
  7. Mutations in the gene encoding peroxisomal sterol carrier protein X (SCPx) cause leukencephalopathy with dystonia and motor neuropathy. Ferdinandusse, S., Kostopoulos, P., Denis, S., Rusch, H., Overmars, H., Dillmann, U., Reith, W., Haas, D., Wanders, R.J., Duran, M., Marziniak, M. Am. J. Hum. Genet. (2006) [Pubmed]
  8. Mutational spectrum of D-bifunctional protein deficiency and structure-based genotype-phenotype analysis. Ferdinandusse, S., Ylianttila, M.S., Gloerich, J., Koski, M.K., Oostheim, W., Waterham, H.R., Hiltunen, J.K., Wanders, R.J., Glumoff, T. Am. J. Hum. Genet. (2006) [Pubmed]
  9. Intracellular dissemination of peroxidative stress. Internalization, transport, and lethal targeting of a cholesterol hydroperoxide species by sterol carrier protein-2-overexpressing hepatoma cells. Kriska, T., Levchenko, V.V., Korytowski, W., Atshaves, B.P., Schroeder, F., Girotti, A.W. J. Biol. Chem. (2006) [Pubmed]
  10. The integrin-linked kinase (ILK) suppresses anoikis. Attwell, S., Roskelley, C., Dedhar, S. Oncogene (2000) [Pubmed]
  11. Holo-sterol carrier protein-2. (13)C NMR investigation of cholesterol and fatty acid binding sites. Stolowich, N., Frolov, A., Petrescu, A.D., Scott, A.I., Billheimer, J.T., Schroeder, F. J. Biol. Chem. (1999) [Pubmed]
  12. Human sterol carrier protein x/sterol carrier protein 2 gene has two promoters. Ohba, T., Holt, J.A., Billheimer, J.T., Strauss, J.F. Biochemistry (1995) [Pubmed]
  13. Regulation of sterol carrier protein gene expression by the forkhead transcription factor FOXO3a. Dansen, T.B., Kops, G.J., Denis, S., Jelluma, N., Wanders, R.J., Bos, J.L., Burgering, B.M., Wirtz, K.W. J. Lipid Res. (2004) [Pubmed]
  14. The structure of the human sterol carrier protein X/sterol carrier protein 2 gene (SCP2). Ohba, T., Rennert, H., Pfeifer, S.M., He, Z., Yamamoto, R., Holt, J.A., Billheimer, J.T., Strauss, J.F. Genomics (1994) [Pubmed]
  15. Peroxisomal fatty acid oxidation disorders and 58 kDa sterol carrier protein X (SCPx). Activity measurements in liver and fibroblasts using a newly developed method. Ferdinandusse, S., Denis, S., van Berkel, E., Dacremont, G., Wanders, R.J. J. Lipid Res. (2000) [Pubmed]
  16. Role of the sterol carrier protein-2 N-terminal membrane binding domain in sterol transfer. Huang, H., Gallegos, A.M., Zhou, M., Ball, J.M., Schroeder, F. Biochemistry (2002) [Pubmed]
  17. Steroidogenic acute regulatory protein binds cholesterol and modulates mitochondrial membrane sterol domain dynamics. Petrescu, A.D., Gallegos, A.M., Okamura, Y., Strauss, J.F., Schroeder, F. J. Biol. Chem. (2001) [Pubmed]
  18. Sterol carrier protein-2. Seedorf, U., Ellinghaus, P., Roch Nofer, J. Biochim. Biophys. Acta (2000) [Pubmed]
  19. Sterol carrier protein-2, a new fatty acyl coenzyme A-binding protein. Frolov, A., Cho, T.H., Billheimer, J.T., Schroeder, F. J. Biol. Chem. (1996) [Pubmed]
  20. Peroxisomal lipid degradation via beta- and alpha-oxidation in mammals. Mannaerts, G.P., Van Veldhoven, P.P., Casteels, M. Cell Biochem. Biophys. (2000) [Pubmed]
  21. Peroxisomal beta-oxidation enzymes. Hashimoto, T. Cell Biochem. Biophys. (2000) [Pubmed]
  22. Fusion and fission, the evolution of sterol carrier protein-2. Edqvist, J., Blomqvist, K. J. Mol. Evol. (2006) [Pubmed]
  23. A second isoform of 3-ketoacyl-CoA thiolase found in Caenorhabditis elegans, which is similar to sterol carrier protein x but lacks the sequence of sterol carrier protein 2. Bun-Ya, M., Maebuchi, M., Hashimoto, T., Yokota, S., Kamiryo, T. Eur. J. Biochem. (1997) [Pubmed]
  24. Effects of hypophysectomy and human chorionic gonadotrophin on Leydig cell function in mature rats. Stocco, D.M., Teerds, K.J., van Noort, M., Rommerts, F.F. J. Endocrinol. (1990) [Pubmed]
  25. Peroxisome proliferators and retinoids affect JEG-3 choriocarcinoma cell function. Matsuo, H., Strauss, J.F. Endocrinology (1994) [Pubmed]
  26. Involvement of corticotropin-releasing factor and adrenocorticotropic hormone in the ovarian maturation, seawater acclimation, and induced spawning of Liza ramada. Mousa, S.A., Mousa, M.A. Gen. Comp. Endocrinol. (2006) [Pubmed]
  27. Purification of nonspecific lipid transfer protein (sterol carrier protein 2) from human liver and its deficiency in livers from patients with cerebro-hepato-renal (Zellweger) syndrome. van Amerongen, A., Helms, J.B., van der Krift, T.P., Schutgens, R.B., Wirtz, K.W. Biochim. Biophys. Acta (1987) [Pubmed]
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