X-ray Crystallography Core Laboratory

Publications

2014

PubMed Montemayor EJ, Katolik A, Clark NE, Taylor AB, Schuermann JP, Combs DJ, Johnsson R, Holloway SP, Stevens SW, Damha MJ, Hart PJ. (2014). Structural basis of lariat RNA recognition by the intron debranching enzyme Dbr1. Nucleic Acids Res. 42, 10845-10855. PDB: 4PEF, 4PEG, 4PEH, 4PEI, NAR Breakthrough Article.

 

PubMed Nanyes DR, Junco SE, Taylor AB, Robinson AK, Patterson NL, Shivarajpur A, Halloran J, Hale SM, Kaur Y, Hart PJ, Kim CA. (2014). Multiple polymer architectures of human polyhomeotic homolog 3 sterile alpha motif. Proteins. (in press). PDB: 4PZN, 4PZO

PubMed Kannan TR, Krishnan M, Ramasamy K, Becker A, Pakhomova ON, Hart PJ, Baseman JB. (2014). Functional mapping of community-acquired respiratory distress syndrome (CARDS) toxin of Mycoplasma pneumoniae defines regions with ADP-ribosyltransferase, vacuolating and receptor-binding activities. Mol. Microbiol. 93, 568-581.

PubMed Ayers JI, Xu G, Pletnikova O, Troncoso JC, Hart PJ, Borchelt DR. (2014). Conformational specificity of the C4F6 SOD1 antibody; low frequency of reactivity in sporadic ALS cases. Acta Neuropathol. Commun. 2, 55.

PubMed Gleason JE, Galaleldeen, A, Peterson RL, Taylor AB, Holloway SP, Waninger-Saroni J, Cormack BP, Cabelli DE, Hart PJ, Culotta VC. (2014). Candida albicans SOD5 represents the prototype of an unprecedented class of Cu-only superoxide dismutases required for pathogen defense. Proc. Natl. Acad. Sci. 111, 5866-5871. PDB: 4N3T, 4N3U

PubMed Katolik A, Johnsson R, Montemayor E, Lackey JG, Hart PJ, Damha MJ. (2014). Regiospecific Solid-phase Synthesis of Branched Oligoribonucleotides that Mimic Intronic Lariat RNA Intermediates. J. Org. Chem. 79, 963-975.

PubMed Ivanova MI, Sievers SA, Guenther EL, Johnson LM, Winkler DD, Galaleldeen A, Sawaya MR, Hart PJ, Eisenberg DS. (2014). Aggregation-triggering segments of SOD1 fibril formation support a common pathway for familial and sporadic ALS. Proc. Natl. Acad. Sci. 111, 197-201.

2013

PubMed Valentim CL, Cioli D, Chevalier FD, Cao X, Taylor AB, Holloway SP, Pica-Mattoccia L, Guidi A, Basso A, Tsai IJ, Berriman M, Carvalho-Queiroz C, Almeida M, Aguilar H, Frantz DE, Hart PJ, LoVerde PT, Anderson TJ. (2013). Genetic and molecular basis of drug resistance and species-specific drug action in schistosome parasites. Science. 342, 1385-1389. PDB: 4MUA, 4MUB

PubMed  Morales KA, Yang Y, Long Z, Li P, Taylor AB, Hart PJ, Igumenova TI. (2013). Cd2+ as a Ca2+ Surrogate in Protein-membrane Interactions: Isostructural but not Isofunctional. J. Am. Chem. Soc. 135, 12980-12983.PDB: 4L1L

PubMedGalaleldeen A, Taylor AB, Chen D, Schuermann JP, Holloway SP, Hou S, Gong S, Zhong G, Hart PJ. (2013). Structure of the Chlamydia trachomatis Immunodominant Antigen Pgp3. J. Biol. Chem. 288, 22068-22079. PDB: 4JDM, 4JDN, 4JDO in ASBMB Today September 2013.

PubMed  Gaweska, HM, Taylor AB, Hart PJ, Fitzpatrick PF. (2013). The Structure of the Flavoprotein Tryptophan-2-Monooxygenase, a Key Enzyme in the Formation of Galls in Plants. Biochemistry. 52, 2620-2626. PDB: 4IV9

PubMed Junco SE, Wang R, Gaipa JC, Taylor AB, Schirf V, Gearhart MD, Bardwell VJ, Demeler B, Hart, PJ, Kim CA. (2013). Structure of the polycomb group protein PCGF1 in complex with BCOR reveals basis for binding selectivity of PCGF homologs. Structure. 21, 665-671. PDB: 4HPL, 4HPM

PubMed  Aguirre JD, Clark HM, McIlvin M, Vazquez C, Palmere SL, Grab D, Seshu J, Hart PJ, Saito M, Culotta VC. (2013). A Manganese-rich Environment Supports Superoxide Dismutase Activity in a Lyme Disease Pathogen, Borrelia burgdorferi. J. Biol. Chem. 288, 8468-8478.

2012

PubMed  Adachi MS, Taylor AB, Hart PJ, Fitzpatrick PF. (2012). Mechanistic and Structural Analyses of the Roles of Active Site Residues in the Yeast Polyamine Oxidase Fms1: Characterization of the N195A and D94N Enzymes. Biochemistry. 51, 8690-8697. PDB: 4GDP

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PubMed  Fang H, Caranto JD, Mendoza R, Taylor AB, Hart PJ, Kurtz Jr DM. (2012). Histidine ligand variants of a flavo-diiron protein: Effects on structure and activities. J. Biol. Inorg. Chem. 17,1231-1239. PDB: 4DIK, 4DIL

 


PubMed  Biris N, Yang Y, Taylor AB, Tomashevski A, Guo M, Hart PJ, Diaz-Griffero F, Ivanov DN. (2012). Structure of the rhesus monkey TRIM5α PRYSPRY domain, the HIV capsid recognition module. Proc. Natl. Acad. Sci. 109, 13278-13283. PDB: 3UV9, 2LM3 in Protein Data Bank Molecule of the Month July 2013

PubMed  Adachi MS, Taylor AB, Hart PJ, Fitzpatrick PF. (2012). Mechanistic and Structural Analyses of the Role of His67 in the Yeast Polyamine Oxidase Fms1. Biochemistry. 51, 4888-4897. PDB: 4ECH

PubMed  Bouldin SD, Darch MA, Hart PJ, Outten CE. (2012). Redox properties of the disulfide bond of human Cu,Zn superoxide dismutase and the effects of human glutaredoxin 1. Biochem J. 446, 59-67.

PubMed  Leitch JM, Li CX, Baron JA, Matthews LM, Cao X, Hart PJ, Culotta VC. (2012). Post-translational modification of Cu/Zn superoxide dismutase under anaerobic conditions. Biochemistry. 51, 677-685.

2011

PubMed  Pau VPT, Smith FJ, Taylor AB, Parfenova LV, Samakai E, Callaghan MM, Abarca-Heidemann K, Hart PJ, and Rothberg BS. (2011). Structure and function of Multiple Ca2+-binding sites in a K+ channel RCK domain. Proc. Natl. Acad. Sci. 108, 17684-17689. PDB: 3RBX, 3RBZ

PubMed  Childers BM, Cao X, Weber GG, Demeler B, Hart PJ, Klose KE. (2011). N-terminal residues of the Vibrio cholerae virulence regulatory protein ToxT involved in dimerization and modulation by fatty acids. J. Biol. Chem. 286, 28644-28655.

PubMed  Chinai JM, Taylor AB, Ryno LM, Hargreaves ND, Morris CA, Hart PJ, and Urbach AR. (2011). Molecular Recognition of Insulin by a Synthetic Receptor. J. Am. Chem. Soc. 133, 8810-8813. PDB: 3Q6E

2010

PubMed  Chen D, Lei L, Lu C, Galaleldeen A, Hart PJ, and Zhong G. (2010). Characterization of Pgp3, a Chlamydia trachomatis plasmid-encoded immunodominant antigen. J Bacteriol. 192, 6017-6024.

PubMed  Seetharaman SV, Taylor AB, Holloway S, Hart PJ. (2010). Structures of mouse SOD1 and human/mouse SOD1 chimeras.  Arch. Biochem. Biophys. 503, 183-190. PDB: 3GTT, 3GTV, 3LTV

PubMed  Wang R, Taylor AB, Leal BZ, Chadwell LV, Ilangova U, Robinson AK, Schirf V, Hart PJ, Lafer EM, Demeler B, Hinck AP, McEwen DG, and Kim CA. (2010). Polycomb Group Targeting Through Different Binding Partners of RING1B C-terminal Domain. Structure. 18, 966-975. PDB: 3GS2, 3IXS

PubMed  Seetharaman SV, Winkler DD, Taylor AB, Cao X, Whitson LJ, Doucette PA, Valentine JS, Schirf V, Demeler B, Carroll MC, Culotta VC, and Hart PJ. (2010). Disrupted Zinc-binding Sites in Structures of Pathogenic SOD1 Variants D124V and H80R.  Biochemistry. 49, 5714-5725.  PDB: 3H2P, 3H2Q, 3H2R

PubMed  Tormos JR, Taylor AB, Daubner SC, Hart PJ, and Fitzpatrick PF. (2010).  Identification of a Hypothetical Protein from Podospora anserina as a Nitroalkane Oxidase. Biochemistry. 49, 5035-5031. PDB: 3MKH

PubMed  Pakhomova ON, Taylor AB, Becker A, Holloway SP, Kannan TR, Baseman JB, and Hart PJ. (2010).  Crystallization of Community-acquired Respiratory Distress Syndrome Toxin from Mycoplasma pneumoniae. Acta Cryst. F66, 294-296.

PubMed  You Z, Cao X, Taylor AB, Hart PJ, and Levine, RL. (2010).  Characterization of a Covalent Polysulfane Bridge in Copper-Zinc Superoxide Dismutase. Biochemistry. 49, 1191-1198. PDB: 3K91

2009

PubMed  Galaleldeen A, Strange R, Whitson LJ, Antonyuk S, Narayana N, Taylor AB, Schuermann JP, Holloway SP, Hasnain SS, and Hart PJ.  (2009).  Structural and Biophysical Properties of Metal-Free Pathogenic SOD1 Mutants A4V and G93A.  Arch. Biochem. Biophys.  492, 40-47.  PDB: 3GZO, 3GZP, 3GZQ, 2WKO

PubMed  Konkle ME, Muellner SK, Schwander AL, Dicus MM, Pokhrel R, Britt RD, Taylor AB, and Hunsicker-Wang LM. (2009).  Effects of pH on the Rieske Protein from Thermus thermophilus: A Spectroscopic and Structural Analysis. Biochemistry. 48, 9848-9857.  PDB: 3FOU

PubMed  Tiwari A, Liba A, Sohn SH, Seetharaman SV, Bilsel O, Matthews CR, Hart PJ, Valentine JS, and Hayward LJ.  (2009).  Metal deficiency increases aberrant hydrophobicity of mutant superoxide dismutases that cause amyotrophic lateral sclerosis.  J. Biol. Chem284, 27746-27758.

PubMed  Seetharaman SV, Prudencio M, Karch C, Holloway SP, Borchelt DR, and Hart PJ. (2009).  Immature copper-zinc superoxide dismutase and familial amyotrophic lateral sclerosis. Exp. Biol. Med. 234, 1140-1154.

PubMed  Balasubramanian S, Kannan TR, Hart PJ, and Baseman JB. (2009). Amino acid changes in elongation factor Tu of Mycoplasma pneumoniae and Mycoplasma genitalium influence fibronectin binding. Infect. Immun. 77, 3533-3541.

PubMed  Leitch JM, Jensen LT, Bouldin SD, Outten CE, Hart PJ, and Culotta VC. (2009). Activation of Cu,Zn-superoxide dismutase in the absence of oxygen and the copper chaperone CCS. J. Biol. Chem. 284, 21863-21871.

PubMed  Prudencio M, Hart PJ, Borchelt DR, and Andersen PM. (2009). Variation in aggregation propensities among ALS-associated variants of SOD1: correlation to human disease. Hum. Mol. Genet. 18, 3217-3226.

  PubMed Karch CM, Prudencio M, Winkler DD, Hart PJ, and Borchelt DR. (2009). Role of mutant SOD1 disulfide oxidation and aggregation in the pathogenesis of familial ALS. Proc. Natl. Acad. Sci. 106, 7774-7779.

PubMed  Chen D, Chai J, Hart PJ, and Zhong G. (2009). Identifying catalytic residues in CPAF, a Chlamydia-secreted protease. Arch. Biochem. Biophys.  485, 16-23.

PubMed  Winkler DD, Schuermann JP, Cao X, Holloway SP, Borchelt DR, Carroll MC, Proescher JB, Culotta VC, and Hart PJ. (2009). Structural and biophysical properties of the pathogenic SOD1 variant H46R/H48Q. Biochemistry. 48, 3436-3447. PDB: 3GQF

2008

PubMed  Schuermann JP, Jiang J, Cuellar J, Llorca O, Wang L, Gimenez LE, Jin S, Taylor AB, Demeler B, Morano KA, Hart PJ, Valpuesta JM, Lafer EM, and Sousa, R. (2008).  Structure of the Hsp110:Hsc70 nucleotide exchange machine. Mol Cell. 31, 232-243. PDB: 3C7N

PubMed  Cao X, Antonyuk S, Seetharaman SV, Whitson LJ, Taylor AB, Holloway SP, Strange RW, Doucette PA, Valentine JS, Tiwari A, Hayward LJ, Padua S, Cohlberg JA, Hasnain SS, and Hart PJ. (2008). Structures of the G85R variant of SOD1 in Familial ALS. J. Biol. Chem. 283, 16169-16177.  PDB: 2VR6, 2VR7, 2VR8, 3CQP, 3CQQ

PubMed   Taylor AB, Meyer B, Leal BZ, Kötter P, Schirf V, Demeler B, Hart PJ, Entian KD, and Wöhnert, J.  (2008). The crystal structure of Nep1 reveals an extended SPOUT-class methyltransferase fold and a pre-organized SAM-binding site.  Nucleic Acids Res. 36, 1542-1554.  PDB: 3BBD, 3BBE, 3BBH

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PubMed  Taylor AB, Hu G, Hart PJ, and McAlister-Henn L. (2008). Allosteric motions in structures of yeast NAD+-specific isocitrate dehydrogenase.  J. Biol. Chem.  283, 10872-10880.  PDB: 3BLV, 3BLW, 3BLX in Protein Data Bank Molecule of the Month September 2010, October 2012

 

 

PubMed   Groppe J, Hinck CS, Samavarchi-Tehrani P, Zubieta C, Schuermann JP, Taylor AB, Schwarz PM, Wrana JL, and Hinck, AP.  (2008). Cooperative assembly of TGF-β superfamily signaling complexes Is mediated by two disparate mechanisms and distinct modes of receptor binding.  Mol. Cell. 29, 157-168.  PDB: 2PJY

 

2007

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PubMed  Quintanar L, Stoj C, Taylor AB, Hart PJ, Kosman DJ, and Solomon EI. (2007). Shall we dance? How a multicopper oxidase chooses its electron transfer partner. Acc. Chem. Res. 40, 445-452.

 

 

PubMed   Jiang J, Maes EG, Taylor AB, Wang L, Hinck AP, Lafer EM, and Sousa, R.  (2007). Structural basis of J cochaperone binding and regulation of Hsp70. Mol. Cell.  28, 422-433. PDB: 2QW9, 2QWL, 2QWM, 2QWN, 2QWO, 2QWP, 2QWQ, 2QWR

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PubMed  Hu G, Taylor AB, McAlister-Henn L, and Hart PJ. (2007). Crystal structure of the yeast nicotinamidase Pnc1p.  Arch. Biochem. Biophys.  461, 66-75.  PDB: 2H0R

 


Galaleldeen A and Hart PJ. (2007) "Human copper-zinc superoxide dismutase and familial amyotrophic lateral sclerosis" in: Protein Reviews: Protein Misfolding, Aggregation and Conformational Diseases. (Atassi, M.Z. ed.). Springer Science + Business Media, LLC, New York, U.S.A., pp. 327-344.

PubMed   Wang J, Caruano-Yzermans A, Rodriguez A, Schuermann JP, Slunt HH, Cao X, Gitlin J, Hart PJ, and Borchelt DR. (2007). Disease-associated mutations at copper ligand histidine residues of superoxide dismutase 1 diminish the binding of copper and compromise dimer stability.  J. Biol. Chem.  282, 345-352.  PDB: 2NNX

2006

PubMed   Heitmann LM, Taylor AB, Hart PJ, and Urbach AR.  (2006).  Sequence-specific recognition and cooperative dimerization of N-terminal aromatic peptides in aqueous solution by a synthetic host.  J. Am. Chem. Soc.  128, 12574-12581.

PubMed   Carroll MC, Outten CE, Proescher JB, Rosenfeld L, Watson WH, Whitson LJ, Hart PJ, Jensen LT, and Culotta VC. (2006). The effects of glutaredoxin and copper activation pathways on the disulfide and stability of Cu,Zn superoxide dismutase.  J. Biol. Chem.  281, 28648-28656.

PubMed   Hart PJ.  (2006).  Pathogenic superoxide dismutase structure, folding, aggregation and turnover.  Curr. Opin. Chem. Biol. 10, 131-138.

PubMed   Jiang J, Lafer EM, and Sousa R. (2006). Crystallization of a functionally intact Hsp70 chaperone. Acta Cryst. F62, 39-43.

2005

PubMed   Taylor AB, Stoj CS, Ziegler L, Kosman DJ, and Hart PJ.  (2005).  The copper-iron connection in biology: Structure of the metallo-oxidase Fet3p.  Proc. Natl. Acad. Sci. 102, 15459-15464.  PDB: 1ZPU

PubMed  Di Noto L, Whitson LJ, Cao X, Hart PJ, and Levine RL.  (2005).  Proteasomal degradation of mutant superoxide dismutases linked to amyotrophic lateral sclerosis.  J. Biol. Chem. 280, 39907-39913.

  PubMed   Jiang J, Prasad K, Lafer EM, and Sousa, R. (2005) Structural basis of interdomain communication in the Hsc70 chaperone. Mol. Cell 20, 513-524.  PDB: 1YUW

Whitson LJ and Hart PJ. (2005) "Copper-zinc superoxide dismutase and familial amyotrophic lateral sclerosis." in: Metal Ions in Biological Systems. Vol 45 (Sigel, A., Sigel, H, and Sigel, R.O.K. eds) John Wiley & Sons Ltd., Chichester, UK.

Hart PJ and Valentine JS. (2005).  "Metal-deficient copper-zinc superoxide dismutase and familial amyotrophic lateral sclerosis" in Medicinal Inorganic Chemistry.  (Sessler, J.L., Doctrow, S.R., McMurray, T.S. and Lippard, S.J. eds).  ACS Books, Washington D.C., U.S.A. pp. 348-365.

Hart PJ, Nersissian AM, and DeBeer GS.  (2005).  "Copper Proteins with Type I Sites" in Encyclopedia of Inorganic Chemistry, Vol. 2. (King, R.B., Atwood, D.A., Crabtree, R.H., Lukehart, C.M. and Scott, R.A. eds.) John Wiley & Sons, Ltd.  Chichester, U.K.

Hu G, Taylor AB, McAlister-Henn L, and Hart PJ. (2005). Crystallization and preliminary X-ray crystallographic analysis of yeast NAD+-specific isocitrate dehydrogenase. Acta Crystallogr.  F61, 486-488.

PubMed   Antonyuk S, Elam JS, Hough MA, Strange RW, Doucette PA, Rodriguez, JA, Hayward LJ, Valentine JS, Hart PJ, and Hasnain SS. (2005).  Structural consequences of the familial amyotrophic lateral sclerosis SOD1 mutant His46Arg. Protein Sci. 14, 1201-1213.

2004

PubMed   Doucette PA, Whitson LJ, Cao X, Schirf V, Demeler B, Valentine JS, Hansen JC, and Hart PJ.  (2004).  Dissociation of human copper-zinc superoxide dismutase dimers using chaotrope and reductant: Insights into the molecular basis for dimer stability. J. Biol. Chem. 279, 54558-54566.

PubMed   Laliberte J, Whitson LJ, Beaudoin J, Holloway SP, Hart PJ, and Labbe S.  (2004).  The Schizosaccharomyces pombe Pccs protein functions in both copper trafficking and metal detoxification pathways.  J. Biol. Chem.  279, 28744-28755.

PubMed   Hough MA, Grossmann JG, Antonyuk SV, Strange RW, Doucette PA, Rodriguez JA, Whitson LJ, Hart PJ, Hayward LJ, Valentine JS, and Hasnain SS. (2004).  Dimer destabilization in superoxide dismutase may result in disease-causing properties: structures of motor neuron disease mutants. Proc. Natl. Acad. Sci. U.S.A. 101, 5976-5981.

2003

PubMed   Elam JS, Taylor AB, Strange R, Antonyuk S, Doucette PA, Rodriguez JA, Hasnain SS, Hayward LJ, Valentine JS, Yeates TO, and Hart PJ. (2003).  Amyloid-like filaments and water-filled nanotubes formed by SOD1 mutants linked to familial ALS. Nat. Struct. Biol. 10, 461-467.  PDB: 1OEZ, 1OZT, 1OZU

PubMed   Elam JS, Malek K, Rodriguez JA, Doucette PA, Taylor AB, Hayward LJ, Cabelli DE, Valentine JS, and Hart PJ. (2003).  An alternative mechanism of bicarbonate-mediated peroxidation by copper-zinc superoxide dismutase.  J. Biol. Chem. 278, 21032-21039. PubMed PDB: 1P1V

PubMed   Taylor AB, Benglis Jr, DM, Dhandayuthapani S, and Hart PJ. (2003).  Structure of Mycobacterium tuberculosis methionine sulfoxide reductase A in complex with protein-bound methionine.  J. Bacteriol. 185, 4119-4126.  PDB: 1NWA

PubMed   Jiang J, Taylor AB, Prasad K, Ishikawa-Brush Y, Hart PJ, Lafer EM, and Sousa R. (2003). Structure-function analysis of the auxilin J-domain reveals an extended Hsc70 interaction interface. Biochemistry. 42, 5748-53. PDB:1NZ6

PubMed   Strange R, Antonyuk S, Hough MA, Doucette PA, Rodriguez JA, Hart PJ, Hayward LJ, Valentine JS, and Hasnain SS. (2003).  The structure of holo and metal deficient wild type human Cu,Zn superoxide dismutase and its relevance to familial amyotrophic lateral sclerosis.  J. Mol. Biol. 328, 877-91.  PDB: 1HL4, 1HL5

PubMed   Valentine JS and Hart PJ. (2003).  Misfolded CuZnSOD and ALS. Proc. Natl. Acad. Sci. U.S.A. 100, 3617-3622.

2002

PubMed   Hart PJ, Deep S, Taylor AB, Shu Z, Hinck CS, and Hinck AP.  (2002). Crystal Structure of the Human TGF-beta Type II Receptor Extracellular Domain in Complex with TGF-beta3. Nat. Struct. Biol. 9, 203-208.  PDB: 1KTZ

PubMed   Elam JS, Thomas ST, Holloway SP, Taylor AB, and Hart PJ. (2002). Copper Chaperones.  In: Advances in Protein Chemistry. (Valentine, J.S. and Gralla, E.B., eds.) Academic Press, San Diego, CA. pp. 151-219.

PubMed   Pascal JM, Hart PJ, Hecht NB, and Robertus JD. (2002). Crystal structure of TB-RBP, a novel RNA-binding and regulating protein.  J. Mol. Biol. 319, 1049-1057.  PDB: 1KEY

2001

Nersissian AM, Hart PJ, and Valentine JS. (2001). Stellacyanin, a member of the phytocyanin family of plant proteins. In: Handbook of Metalloproteins (Messerschmidt, A., Huber, R., Poulos, T., and Wieghardt, K., eds) John Wiley and Sons, Ltd.,  Chichester, England. pp. 1219-1234.

2000

PubMed   Hall LT, Sanchez RJ, Holloway SP, Zhu H, Stine JE, Lyons TJ, Demeler B, Schirf V, Hansen JC, Nersissian AM, Valentine JS, and Hart PJ. (2000). X-ray crystallographic and analytical ultracentrifugation analyses of truncated and full-length yeast copper chaperones for SOD (LYS7): a dimer-dimer model of LYS7-SOD association and copper delivery. Biochemistry. 39, 3611-3623. PDB: 1EJ8

PubMed   Zhu H, Shipp E, Sanchez RJ, Liba A, Stine JE, Hart PJ, Gralla EB, Nersissian AM, and Valentine JS.  (2000). Cobalt(2+) binding to human and tomato copper chaperone for superoxide dismutase: implications for the metal ion transfer mechanism. Biochemistry39, 5413-5421.

1999

PubMed   Valentine JS, Hart PJ, and Gralla EB. (1999). "Copper-zinc superoxide dismutase and ALS." In: Advances in Experimental Medicine and Biology: Copper Transport and its Disorders:  Molecular and Cellular Aspects (Leone, A., and Mercer, J.F.B. eds.) Plenum Press, New York, New York. pp. 193-203.