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The Copper Metabolism MURR1 domain protein 1 (COMMD1) modulates the aggregation of misfolded protein species in a client-specific manner.

Vonk WI, Kakkar V, Bartuzi P, Jaarsma D, Berger R, Hofker MH, Klomp LW, Wijmenga C, Kampinga HH, van de Sluis B.

PLoS One. 2014 Apr 1;9(4):e92408. doi: 10.1371/journal.pone.0092408. eCollection 2014.


D-serine influences synaptogenesis in a p19 cell model.

Fuchs SA, Roeleveld MW, Klomp LW, Berger R, de Koning TJ.

JIMD Rep. 2012;6:47-53. doi: 10.1007/8904_2011_116. Epub 2012 Feb 1.


Monitoring bile acid transport in single living cells using a genetically encoded Förster resonance energy transfer sensor.

van der Velden LM, Golynskiy MV, Bijsmans IT, van Mil SW, Klomp LW, Merkx M, van de Graaf SF.

Hepatology. 2013 Feb;57(2):740-52. doi: 10.1002/hep.26012. Epub 2013 Jan 8.


Liver-specific Commd1 knockout mice are susceptible to hepatic copper accumulation.

Vonk WI, Bartuzi P, de Bie P, Kloosterhuis N, Wichers CG, Berger R, Haywood S, Klomp LW, Wijmenga C, van de Sluis B.

PLoS One. 2011;6(12):e29183. doi: 10.1371/journal.pone.0029183. Epub 2011 Dec 22.


Clusterin and COMMD1 independently regulate degradation of the mammalian copper ATPases ATP7A and ATP7B.

Materia S, Cater MA, Klomp LW, Mercer JF, La Fontaine S.

J Biol Chem. 2012 Jan 20;287(4):2485-99. doi: 10.1074/jbc.M111.302216. Epub 2011 Nov 30.


Increased concentrations of both NMDA receptor co-agonists D-serine and glycine in global ischemia: a potential novel treatment target for perinatal asphyxia.

Fuchs SA, Peeters-Scholte CM, de Barse MM, Roeleveld MW, Klomp LW, Berger R, de Koning TJ.

Amino Acids. 2012 Jul;43(1):355-63. doi: 10.1007/s00726-011-1086-9. Epub 2011 Sep 23.


A sensitive and simple ultra-high-performance-liquid chromatography-tandem mass spectrometry based method for the quantification of D-amino acids in body fluids.

Visser WF, Verhoeven-Duif NM, Ophoff R, Bakker S, Klomp LW, Berger R, de Koning TJ.

J Chromatogr A. 2011 Oct 7;1218(40):7130-6. doi: 10.1016/j.chroma.2011.07.087. Epub 2011 Aug 3.


The copper-transporting capacity of ATP7A mutants associated with Menkes disease is ameliorated by COMMD1 as a result of improved protein expression.

Vonk WI, de Bie P, Wichers CG, van den Berghe PV, van der Plaats R, Berger R, Wijmenga C, Klomp LW, van de Sluis B.

Cell Mol Life Sci. 2012 Jan;69(1):149-63. doi: 10.1007/s00018-011-0743-1. Epub 2011 Jun 11.


Activation of bile salt nuclear receptor FXR is repressed by pro-inflammatory cytokines activating NF-κB signaling in the intestine.

Gadaleta RM, Oldenburg B, Willemsen EC, Spit M, Murzilli S, Salvatore L, Klomp LW, Siersema PD, van Erpecum KJ, van Mil SW.

Biochim Biophys Acta. 2011 Aug;1812(8):851-8. doi: 10.1016/j.bbadis.2011.04.005. Epub 2011 Apr 22.


Clusterin (apolipoprotein J), a molecular chaperone that facilitates degradation of the copper-ATPases ATP7A and ATP7B.

Materia S, Cater MA, Klomp LW, Mercer JF, La Fontaine S.

J Biol Chem. 2011 Mar 25;286(12):10073-83. doi: 10.1074/jbc.M110.190546. Epub 2011 Jan 17.


Farnesoid X receptor activation inhibits inflammation and preserves the intestinal barrier in inflammatory bowel disease.

Gadaleta RM, van Erpecum KJ, Oldenburg B, Willemsen EC, Renooij W, Murzilli S, Klomp LW, Siersema PD, Schipper ME, Danese S, Penna G, Laverny G, Adorini L, Moschetta A, van Mil SW.

Gut. 2011 Apr;60(4):463-72. doi: 10.1136/gut.2010.212159. Epub 2011 Jan 17.


Expanding the clinical spectrum of 3-phosphoglycerate dehydrogenase deficiency.

Tabatabaie L, Klomp LW, Rubio-Gozalbo ME, Spaapen LJ, Haagen AA, Dorland L, de Koning TJ.

J Inherit Metab Dis. 2011 Feb;34(1):181-4. doi: 10.1007/s10545-010-9249-5. Epub 2010 Nov 27.


Familial cholestasis: progressive familial intrahepatic cholestasis, benign recurrent intrahepatic cholestasis and intrahepatic cholestasis of pregnancy.

van der Woerd WL, van Mil SW, Stapelbroek JM, Klomp LW, van de Graaf SF, Houwen RH.

Best Pract Res Clin Gastroenterol. 2010 Oct;24(5):541-53. doi: 10.1016/j.bpg.2010.07.010. Review.


Heteromeric interactions required for abundance and subcellular localization of human CDC50 proteins and class 1 P4-ATPases.

van der Velden LM, Wichers CG, van Breevoort AE, Coleman JA, Molday RS, Berger R, Klomp LW, van de Graaf SF.

J Biol Chem. 2010 Dec 17;285(51):40088-96. doi: 10.1074/jbc.M110.139006. Epub 2010 Oct 14.


Raised hepatic bile acid concentrations during pregnancy in mice are associated with reduced farnesoid X receptor function.

Milona A, Owen BM, Cobbold JF, Willemsen EC, Cox IJ, Boudjelal M, Cairns W, Schoonjans K, Taylor-Robinson SD, Klomp LW, Parker MG, White R, van Mil SW, Williamson C.

Hepatology. 2010 Oct;52(4):1341-9. doi: 10.1002/hep.23849.


Biochemical and cellular functions of P4 ATPases.

van der Velden LM, van de Graaf SF, Klomp LW.

Biochem J. 2010 Oct 1;431(1):1-11. doi: 10.1042/BJ20100644. Review.


Cu,Zn superoxide dismutase maturation and activity are regulated by COMMD1.

Vonk WI, Wijmenga C, Berger R, van de Sluis B, Klomp LW.

J Biol Chem. 2010 Sep 10;285(37):28991-9000. doi: 10.1074/jbc.M110.101477. Epub 2010 Jul 1.


A flippase-independent function of ATP8B1, the protein affected in familial intrahepatic cholestasis type 1, is required for apical protein expression and microvillus formation in polarized epithelial cells.

Verhulst PM, van der Velden LM, Oorschot V, van Faassen EE, Klumperman J, Houwen RH, Pomorski TG, Holthuis JC, Klomp LW.

Hepatology. 2010 Jun;51(6):2049-60. doi: 10.1002/hep.23586.


COMMD1 disrupts HIF-1alpha/beta dimerization and inhibits human tumor cell invasion.

van de Sluis B, Mao X, Zhai Y, Groot AJ, Vermeulen JF, van der Wall E, van Diest PJ, Hofker MH, Wijmenga C, Klomp LW, Cho KR, Fearon ER, Vooijs M, Burstein E.

J Clin Invest. 2010 Jun;120(6):2119-30. doi: 10.1172/JCI40583. Epub 2010 May 10.


Bile acids and their nuclear receptor FXR: Relevance for hepatobiliary and gastrointestinal disease.

Gadaleta RM, van Mil SW, Oldenburg B, Siersema PD, Klomp LW, van Erpecum KJ.

Biochim Biophys Acta. 2010 Jul;1801(7):683-92. doi: 10.1016/j.bbalip.2010.04.006. Epub 2010 Apr 23. Review.


Clusterin facilitates COMMD1 and I-kappaB degradation to enhance NF-kappaB activity in prostate cancer cells.

Zoubeidi A, Ettinger S, Beraldi E, Hadaschik B, Zardan A, Klomp LW, Nelson CC, Rennie PS, Gleave ME.

Mol Cancer Res. 2010 Jan;8(1):119-30. doi: 10.1158/1541-7786.MCR-09-0277. Epub 2010 Jan 12.


Liver disease associated with canalicular transport defects: current and future therapies.

Stapelbroek JM, van Erpecum KJ, Klomp LW, Houwen RH.

J Hepatol. 2010 Feb;52(2):258-71. doi: 10.1016/j.jhep.2009.11.012. Epub 2009 Nov 21. Review.


L-serine synthesis in the central nervous system: a review on serine deficiency disorders.

Tabatabaie L, Klomp LW, Berger R, de Koning TJ.

Mol Genet Metab. 2010 Mar;99(3):256-62. doi: 10.1016/j.ymgme.2009.10.012. Epub 2009 Oct 25. Review.


Reduced expression of ATP7B affected by Wilson disease-causing mutations is rescued by pharmacological folding chaperones 4-phenylbutyrate and curcumin.

van den Berghe PV, Stapelbroek JM, Krieger E, de Bie P, van de Graaf SF, de Groot RE, van Beurden E, Spijker E, Houwen RH, Berger R, Klomp LW.

Hepatology. 2009 Dec;50(6):1783-95. doi: 10.1002/hep.23209.


Folding defects in P-type ATP 8B1 associated with hereditary cholestasis are ameliorated by 4-phenylbutyrate.

van der Velden LM, Stapelbroek JM, Krieger E, van den Berghe PV, Berger R, Verhulst PM, Holthuis JC, Houwen RH, Klomp LW, van de Graaf SF.

Hepatology. 2010 Jan;51(1):286-96. doi: 10.1002/hep.23268.


New developments in the regulation of intestinal copper absorption.

van den Berghe PV, Klomp LW.

Nutr Rev. 2009 Nov;67(11):658-72. doi: 10.1111/j.1753-4887.2009.00250.x. Review.


Posttranslational regulation of copper transporters.

van den Berghe PV, Klomp LW.

J Biol Inorg Chem. 2010 Jan;15(1):37-46. doi: 10.1007/s00775-009-0592-7. Epub 2009 Oct 8. Review.


COMMD1 Promotes pVHL and O2-Independent Proteolysis of HIF-1alpha via HSP90/70.

van de Sluis B, Groot AJ, Vermeulen J, van der Wall E, van Diest PJ, Wijmenga C, Klomp LW, Vooijs M.

PLoS One. 2009 Oct 5;4(10):e7332. doi: 10.1371/journal.pone.0007332.


The copper connection.

Linn FH, van Erpecum KJ, Klomp LW, Houwen RH.

Eur J Neurol. 2009 Oct;16(10):1073-4. doi: 10.1111/j.1468-1331.2009.02732.x. Epub 2009 Jun 29. No abstract available.


ATP8B1 is essential for maintaining normal hearing.

Stapelbroek JM, Peters TA, van Beurden DH, Curfs JH, Joosten A, Beynon AJ, van Leeuwen BM, van der Velden LM, Bull L, Oude Elferink RP, van Zanten BA, Klomp LW, Houwen RH.

Proc Natl Acad Sci U S A. 2009 Jun 16;106(24):9709-14. doi: 10.1073/pnas.0807919106. Epub 2009 May 28.


Novel mutations in 3-phosphoglycerate dehydrogenase (PHGDH) are distributed throughout the protein and result in altered enzyme kinetics.

Tabatabaie L, de Koning TJ, Geboers AJ, van den Berg IE, Berger R, Klomp LW.

Hum Mutat. 2009 May;30(5):749-56. doi: 10.1002/humu.20934.


Nuclear-cytosolic transport of COMMD1 regulates NF-kappaB and HIF-1 activity.

Muller PA, van de Sluis B, Groot AJ, Verbeek D, Vonk WI, Maine GN, Burstein E, Wijmenga C, Vooijs M, Reits E, Klomp LW.

Traffic. 2009 May;10(5):514-27. doi: 10.1111/j.1600-0854.2009.00892.x. Epub 2009 Feb 11.


Role of transition metals in the pathogenesis of amyotrophic lateral sclerosis.

Vonk WI, Klomp LW.

Biochem Soc Trans. 2008 Dec;36(Pt 6):1322-8. doi: 10.1042/BST0361322. Review.


COMMD1 expression is controlled by critical residues that determine XIAP binding.

Maine GN, Mao X, Muller PA, Komarck CM, Klomp LW, Burstein E.

Biochem J. 2009 Jan 15;417(2):601-9. doi: 10.1042/BJ20080854.


Novel perspectives in mammalian copper metabolism through the use of genome-wide approaches.

Muller PA, Klomp LW.

Am J Clin Nutr. 2008 Sep;88(3):821S-5S.


ATOX1: a novel copper-responsive transcription factor in mammals?

Muller PA, Klomp LW.

Int J Biochem Cell Biol. 2009 Jun;41(6):1233-6. doi: 10.1016/j.biocel.2008.08.001. Epub 2008 Aug 7. Review.


Two mass-spectrometric techniques for quantifying serine enantiomers and glycine in cerebrospinal fluid: potential confounders and age-dependent ranges.

Fuchs SA, de Sain-van der Velden MG, de Barse MM, Roeleveld MW, Hendriks M, Dorland L, Klomp LW, Berger R, de Koning TJ.

Clin Chem. 2008 Sep;54(9):1443-50. doi: 10.1373/clinchem.2007.100412. Epub 2008 Jul 7.


Elevated glutathione levels confer cellular sensitization to cisplatin toxicity by up-regulation of copper transporter hCtr1.

Chen HH, Song IS, Hossain A, Choi MK, Yamane Y, Liang ZD, Lu J, Wu LY, Siddik ZH, Klomp LW, Savaraj N, Kuo MT.

Mol Pharmacol. 2008 Sep;74(3):697-704. doi: 10.1124/mol.108.047969. Epub 2008 Jun 3.


Transcription factor Sp1 plays an important role in the regulation of copper homeostasis in mammalian cells.

Song IS, Chen HH, Aiba I, Hossain A, Liang ZD, Klomp LW, Kuo MT.

Mol Pharmacol. 2008 Sep;74(3):705-13. doi: 10.1124/mol.108.046771. Epub 2008 May 15.


Cerebrospinal fluid D-serine and glycine concentrations are unaltered and unaffected by olanzapine therapy in male schizophrenic patients.

Fuchs SA, De Barse MM, Scheepers FE, Cahn W, Dorland L, de Sain-van der Velden MG, Klomp LW, Berger R, Kahn RS, de Koning TJ.

Eur Neuropsychopharmacol. 2008 May;18(5):333-8. doi: 10.1016/j.euroneuro.2007.12.002. Epub 2008 Feb 7.


[From gene to disease; Menkes disease: copper deficiency due to an ATP7A-gene defect].

Aldenhoven M, Klomp LW, van Hasselt PM, de Koning TJ, Visser G.

Ned Tijdschr Geneeskd. 2007 Oct 13;151(41):2266-70. Review. Dutch.


Distinct Wilson's disease mutations in ATP7B are associated with enhanced binding to COMMD1 and reduced stability of ATP7B.

de Bie P, van de Sluis B, Burstein E, van de Berghe PV, Muller P, Berger R, Gitlin JD, Wijmenga C, Klomp LW.

Gastroenterology. 2007 Oct;133(4):1316-26. Epub 2007 Jul 25.


COMMD1: a novel protein involved in the proteolysis of proteins.

van de Sluis B, Groot AJ, Wijmenga C, Vooijs M, Klomp LW.

Cell Cycle. 2007 Sep 1;6(17):2091-8. Epub 2007 Jun 22.


Molecular pathogenesis of Wilson and Menkes disease: correlation of mutations with molecular defects and disease phenotypes.

de Bie P, Muller P, Wijmenga C, Klomp LW.

J Med Genet. 2007 Nov;44(11):673-88. Epub 2007 Aug 23. Review.


Human copper transporter 2 is localized in late endosomes and lysosomes and facilitates cellular copper uptake.

van den Berghe PV, Folmer DE, Malingré HE, van Beurden E, Klomp AE, van de Sluis B, Merkx M, Berger R, Klomp LW.

Biochem J. 2007 Oct 1;407(1):49-59.


Increased activity of hypoxia-inducible factor 1 is associated with early embryonic lethality in Commd1 null mice.

van de Sluis B, Muller P, Duran K, Chen A, Groot AJ, Klomp LW, Liu PP, Wijmenga C.

Mol Cell Biol. 2007 Jun;27(11):4142-56. Epub 2007 Mar 19.


Variation of linker length in ratiometric fluorescent sensor proteins allows rational tuning of Zn(II) affinity in the picomolar to femtomolar range.

van Dongen EM, Evers TH, Dekkers LM, Meijer EW, Klomp LW, Merkx M.

J Am Chem Soc. 2007 Mar 28;129(12):3494-5. Epub 2007 Mar 3. No abstract available.


Gene expression profiling of liver cells after copper overload in vivo and in vitro reveals new copper-regulated genes.

Muller P, van Bakel H, van de Sluis B, Holstege F, Wijmenga C, Klomp LW.

J Biol Inorg Chem. 2007 May;12(4):495-507. Epub 2007 Jan 9.


D-serine in the developing human central nervous system.

Fuchs SA, Dorland L, de Sain-van der Velden MG, Hendriks M, Klomp LW, Berger R, de Koning TJ.

Ann Neurol. 2006 Oct;60(4):476-80.


Ratiometric fluorescent sensor proteins with subnanomolar affinity for Zn(II) based on copper chaperone domains.

van Dongen EM, Dekkers LM, Spijker K, Meijer EW, Klomp LW, Merkx M.

J Am Chem Soc. 2006 Aug 23;128(33):10754-62.


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