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Gene Review

Scp2  -  sterol carrier protein 2, liver

Mus musculus

Synonyms: AA409774, AA409893, C76618, C79031, NSL-TP, ...
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Disease relevance of Scp2

  • Scp2 deficiency did not prevent gallstone formation and may be compensated in part by hepatic up-regulation of liver fatty acid-binding protein [1].
  • Our results suggest that overexpression of SCP2 contributes to biliary cholesterol hypersecretion and the pathogenesis of gallstones in genetically susceptible mice [2].
  • Complete deficiency of SCP2 and SCPx was associated with marked alterations in gene expression, peroxisome proliferation, hypolipidemia, impaired body weight control, and neuropathy [3].
  • We have cloned the cytosolic/peroxisomal form of mouse SCP-2 into the Escherichia coli expression vector pKK233-2 and have expressed and purified recombinant mouse SCP-2, Mr 13,034 [4].
  • Recombinant adenovirus Ad.rSCP2 was used to overexpress SCP-2 in livers of mice [5].

High impact information on Scp2


Chemical compound and disease context of Scp2

  • Although expression of sterol carrier protein (SCP)-2, SCP-x, or liver FABP (L-FABP) in transfected L cells reduced [(3)H]phytanic acid uptake (57-87%) and lipid accumulation (21-27%), nevertheless [(3)H]phytanic acid oxidation was inhibited (74-100%) and phytanic acid toxicity was enhanced in the order L-FABP >> SCP-x > SCP-2 [8].

Biological context of Scp2

  • Assignment of the mouse sterol carrier protein gene (Scp2) to chromosome 4 [9].
  • In this report we show that a mouse cDNA (785 base pairs) encodes a precursor form of SCP-2 containing a N-terminal presequence and an additional C-terminal residue [4].
  • In summary, these data showed for the first time that SCP-2 and SCP-x stimulate oxidation and esterification of branched-chain as well as straight-chain fatty acids in intact cells [10].
  • These effects suggest a key regulatory role for SCP-2 in hepatic lipid metabolism and the existence of a reciprocal relationship between the fluxes of lipids across the sinusoidal and canalicular membranes [5].
  • Cloning, expression and sequences of mouse sterol-carrier protein-x-encoding cDNAs and a related pseudogene [11].

Anatomical context of Scp2


Associations of Scp2 with chemical compounds

  • We hypothesized that Scp2 deficiency may alter biliary lipid secretion and hepatic cholesterol metabolism [1].
  • Early reports indicates cross-reactivity of brain proteins with antisera against two native liver sterol transfer proteins, sterol carrier protein-2 (SCP-2) and the liver form of fatty acid-binding protein (L-FABP) [15].
  • Our results demonstrate that the SCPx thiolase is critical for beta-oxidation of the steroid side chain in conversion of cholesterol into bile acids [16].
  • Recently, gene targeting of the SCP2/SCPx gene has shown in mice that the SCPx beta-ketothiolase is involved in peroxisomal beta-oxidation of 2-methyl-branched chain fatty acids like pristanic acid [16].
  • In contrast, SCP-2 increased biliary lipid secretion and the proportion of hydrophobic PC molecular species in bile [5].

Physical interactions of Scp2


Other interactions of Scp2


Analytical, diagnostic and therapeutic context of Scp2


  1. Disruption of the sterol carrier protein 2 gene in mice impairs biliary lipid and hepatic cholesterol metabolism. Fuchs, M., Hafer, A., Münch, C., Kannenberg, F., Teichmann, S., Scheibner, J., Stange, E.F., Seedorf, U. J. Biol. Chem. (2001) [Pubmed]
  2. Sterol carrier protein 2 participates in hypersecretion of biliary cholesterol during gallstone formation in genetically gallstone-susceptible mice. Fuchs, M., Lammert, F., Wang, D.Q., Paigen, B., Carey, M.C., Cohen, D.E. Biochem. J. (1998) [Pubmed]
  3. Defective peroxisomal catabolism of branched fatty acyl coenzyme A in mice lacking the sterol carrier protein-2/sterol carrier protein-x gene function. Seedorf, U., Raabe, M., Ellinghaus, P., Kannenberg, F., Fobker, M., Engel, T., Denis, S., Wouters, F., Wirtz, K.W., Wanders, R.J., Maeda, N., Assmann, G. Genes Dev. (1998) [Pubmed]
  4. cDNA sequence and bacterial expression of mouse liver sterol carrier protein-2. Moncecchi, D., Pastuszyn, A., Scallen, T.J. J. Biol. Chem. (1991) [Pubmed]
  5. Hepatic overexpression of sterol carrier protein-2 inhibits VLDL production and reciprocally enhances biliary lipid secretion. Amigo, L., Zanlungo, S., Miquel, J.F., Glick, J.M., Hyogo, H., Cohen, D.E., Rigotti, A., Nervi, F. J. Lipid Res. (2003) [Pubmed]
  6. Sterol carrier protein 2 gene transfer changes lipid metabolism and enterohepatic sterol circulation in mice. Zanlungo, S., Amigo, L., Mendoza, H., Miquel, J.F., Vío, C., Glick, J.M., Rodríguez, A., Kozarsky, K., Quiñones, V., Rigotti, A., Nervi, F. Gastroenterology (2000) [Pubmed]
  7. Fluorescence and multiphoton imaging resolve unique structural forms of sterol in membranes of living cells. McIntosh, A.L., Gallegos, A.M., Atshaves, B.P., Storey, S.M., Kannoju, D., Schroeder, F. J. Biol. Chem. (2003) [Pubmed]
  8. Expression of fatty acid binding proteins inhibits lipid accumulation and alters toxicity in L cell fibroblasts. Atshaves, B.P., Storey, S.M., Petrescu, A., Greenberg, C.C., Lyuksyutova, O.I., Smith, R., Schroeder, F. Am. J. Physiol., Cell Physiol. (2002) [Pubmed]
  9. Assignment of the mouse sterol carrier protein gene (Scp2) to chromosome 4. Welch, C.L., Xia, Y.R., Billheimer, J.T., Strauss 3rd, J.F., Lusis, A.J. Mamm. Genome (1996) [Pubmed]
  10. Sterol carrier protein-2/sterol carrier protein-x expression differentially alters fatty acid metabolism in L cell fibroblasts. Atshaves, B.P., Storey, S.M., Schroeder, F. J. Lipid Res. (2003) [Pubmed]
  11. Cloning, expression and sequences of mouse sterol-carrier protein-x-encoding cDNAs and a related pseudogene. Seedorf, U., Raabe, M., Assmann, G. Gene (1993) [Pubmed]
  12. Sterol carrier protein-2 selectively alters lipid composition and cholesterol dynamics of caveolae/lipid raft vs nonraft domains in L-cell fibroblast plasma membranes. Atshaves, B.P., Gallegos, A.M., McIntosh, A.L., Kier, A.B., Schroeder, F. Biochemistry (2003) [Pubmed]
  13. Sterol carrier protein-2 expression alters plasma membrane lipid distribution and cholesterol dynamics. Gallegos, A.M., Atshaves, B.P., Storey, S.M., McIntosh, A.L., Petrescu, A.D., Schroeder, F. Biochemistry (2001) [Pubmed]
  14. Sterol carrier protein-2 localization in endoplasmic reticulum and role in phospholipid formation. Starodub, O., Jolly, C.A., Atshaves, B.P., Roths, J.B., Murphy, E.J., Kier, A.B., Schroeder, F. Am. J. Physiol., Cell Physiol. (2000) [Pubmed]
  15. Effects of chronic ethanol consumption on sterol transfer proteins in mouse brain. Myers-Payne, S.C., Fontaine, R.N., Loeffler, A., Pu, L., Rao, A.M., Kier, A.B., Wood, W.G., Schroeder, F. J. Neurochem. (1996) [Pubmed]
  16. Aberrant oxidation of the cholesterol side chain in bile acid synthesis of sterol carrier protein-2/sterol carrier protein-x knockout mice. Kannenberg, F., Ellinghaus, P., Assmann, G., Seedorf, U. J. Biol. Chem. (1999) [Pubmed]
  17. Sterol carrier protein-2 directly interacts with caveolin-1 in vitro and in vivo. Zhou, M., Parr, R.D., Petrescu, A.D., Payne, H.R., Atshaves, B.P., Kier, A.B., Ball, J.M., Schroeder, F. Biochemistry (2004) [Pubmed]
  18. Stability of fatty acyl-coenzyme A thioester ligands of hepatocyte nuclear factor-4alpha and peroxisome proliferator-activated receptor-alpha. Schroeder, F., Huang, H., Hostetler, H.A., Petrescu, A.D., Hertz, R., Bar-Tana, J., Kier, A.B. Lipids (2005) [Pubmed]
  19. Sterol carrier protein-2: not just for cholesterol any more. Murphy, E.J. Mol. Cell. Biochem. (2002) [Pubmed]
  20. Peroxisomal beta-oxidation enzyme gene expression in the developing mouse brain. Knoll, A., Salles, J., Sargueil, F., Cassagne, C., Garbay, B. Neurosci. Lett. (2000) [Pubmed]
  21. Effect of membrane lipids on the lactosylceramide molecular species specificity of CMP-N-acetylneuraminate:lactosylceramide sialyltransferase. Kadowaki, H., Grant, M.A., Williams, L.A. J. Lipid Res. (1993) [Pubmed]
  22. Structure and chromosomal assignment of the murine sterol carrier protein 2 gene (Scp2) and two related pseudogenes by in situ hybridization. Raabe, M., Seedorf, U., Hameister, H., Ellinghaus, P., Assmann, G. Cytogenet. Cell Genet. (1996) [Pubmed]
  23. Characterization of a sterol carrier protein 2/3-oxoacyl-CoA thiolase from the cotton leafworm (Spodoptera littoralis): a lepidopteran mechanism closer to that in mammals than that in dipterans. Takeuchi, H., Chen, J.H., Jenkins, J.R., Bun-Ya, M., Turner, P.C., Rees, H.H. Biochem. J. (2004) [Pubmed]
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