Mendel, R.R., Schwarz, G.
The history of animal and plant sulfite oxidase.
(2023) Molecules, 28 (19)

Liebsch, F., Eggersmann, F.R., Merkler, Y., Kloppenburg, P., Schwarz, G.
Automated image analysis reveals different localization of synaptic gephyrin C4 splice variants.
(2023) eNeuro, 10(1)

Kožich, V., Schwahn, B.C., Sokolová, J., Křížková, M., Ditroi, T., Krijt, J., Khalil, Y., Křížek, T., Vaculíková-Fantlová, T., Stibůrková, B., Mills, P., Clayton, P., Barvíková, K., Blessing, H., Sykut-Cegielska, J., Dionisi-Vici, C., Gasperini, S., García-Cazorla, Á., Haack, T.B., Honzík, T., Ješina, P., Kuster, A., Laugwitz, L., Martinelli, D., Porta, F., Santer, R., Schwarz, G., Nagy, P.
Human ultrarare genetic disorders of sulfur metabolism demonstrate redundancies in H2S homeostasis.
(2022) Redox Biol, 58

Johannes, L., Fu, C.Y., Schwarz, G.
Molybdenum Cofactor Deficiency in Humans.
(2022) Molecules, 27(20)

Macha, A., Liebsch, F., Fricke, S., Hetsch, F., Neuser, F., Johannes, L., Kress, V., Djémié, T., Santamaria-Araujo, J.S., Vilain, C., Aeby, A., Van Bogaert, P., Dejanovic, B., Weckhuysen, S., Meier, J.C., Schwarz, G.
Biallelic gephyrin variants lead to impaired GABAergic inhibition in a patient with developmental and epileptic encephalopathy.
(2022) Hum Mol Gen, 31(6)

Macabrey, D., Joniová, J., Gasser, Q., Bechelli, C., Longchamp, A., Urfer, S., Lambelet, M., Fu, C.Y., Schwarz, G., Wagnières, G., Déglise, S., Allagnat, F.
Sodium thiosulfate, a source of hydrogen sulfide, stimulates endothelial cell proliferation and neovascularization.
(2022) Front Cardiovasc Med, 9

Salscheider, S.L., Gerlich, S., Cabrera-Orefice, A., Peker, E., Rothemann, R.A., Murschall, L.M., Finger, Y., Szczepanowska, K., Ahmadi, Z.A., Guerrero-Castillo, S., Erdogan, A., Becker, M., Ali, M., Habich, M., Petrungaro, C., Burdina, N., Schwarz, G., Klußmann, M., Neundorf, I., Stroud, D.A., Ryan, M.T., Trifunovic, A., Brandt, U., Riemer, J.
AIFM1 is a component of the mitochondrial disulfide relay that drives complex I assembly through efficient import of NDUFS5.
(2022) EMBO J, 1 (17)

Koehler, F.C., Fu, C.Y., Späth, M.R., Hoyer-Allo, K.J.R., Bohl, K., Göbel, H., Lackmann, J.W., Grundmann, F., Osterholt, T., Gloistein, C., Steiner, J.D., Antebi, A., Benzing, T., Schermer, B., Schwarz, G., Burst, V., Müller, R.U.
A systematic analysis of diet-induced nephroprotection reveals overlapping changes in cysteine catabolism.
(2022) Transl Res, S.1931-5244(22)00023-8

Pristup, J., Schaeffeler, E., Arjune, S., Hofmann, U., Santamaria-Araujo, J.A., Leuthold, P., Friedrich, N., Nauck, M., Mayr, S., Haag, M., Muerdter, T., Marner, F.J., Relling, M.V., Evans, W.E., Schwarz, G., Schwab, M.
Molybdenum cofactor catabolism unravels the physiological role of the drug metabolizing enzyme thiopurine S-methyltransferase
(2022) Clinical Pharmacology and Therapeutics, 112 (4)

Maiber, L., Koprivova, A., Bender, D., Kopriva, S., Fischer-Schrader, K.
Characterization of the amidoxime reducing components ARC1 and ARC2 from Arabidopsis thaliana
(2022) FEBS Journal, 289 (18)

Eh, M., Kaczmarek, A.T., Schwarz, G. Bender, D.
Molecular mechanism of intramolecular electron transfer in dimeric sulfite oxidase
(2022) Journal of Biological Chemistry, 298(3)

Kaczmarek, A.T., Bender, D., Gehling, T., Kohl, J.B., Daimagüler, H.S., Santamaria-Araujo, J.A., Liebau, M.C., Koy, A., Cirak, S., Schwarz, G.
A defect in molybednum cofactor binding causes an attenuated form of sulfite oxidase deficiency
(2022) Journal of Inherited Metabolic Disease, 45 (2), pp. 169-182

Macha, A., Liebsch, F., Fricke, S., Hetsch, F., Neuser, F., Johannes, L., Kress, V., Djémié, T., Santamaria-Araujo, J.A., Vilain, C., Aeby, A., Van Bogaert, P., Dejanovic, B., Weckhuysen, S., Meier, J.C., Schwarz, G.
Biallelic gephyrin variants impair GABAergic inhibition in a patient with epileptic encephalopathy.
(2022) Human Molecular Genetics, 38 (6), pp.901-913

Misko, A., Mahtani, K., Abbott, J., Schwarz, G., Atwal, P.
Molybdenum cofactor deficiency
(2021) in: Adam, M.P., Ardinger, H.H., Pagaon, R.A., et al., editors. Gene Reviews™ (Internet). University of Washington, Seattle

Kaczmarek, A.T., Bahlmann, N., Thaqi, B., May, P., Schwarz, G.
Machine learning-based identification and characterization of 15 novel pathogenic SUOX missense mutations
(2021) Molecular Genetics and Metabolism, 134 (1-2), pp. 188-194

Mayr, S.J., Mendel, R.R., Schwarz, G.
Molybdenum cofactor biology, evolution and deficiency
(2021) Biochimica et Biophysica Acta (BBA) - Molecular Cell Research, 1868(1):118883

Mellis, A.T., Misko, A.L., Arjune, S., Liang, Y., Erdelyi, K., Ditroi, T., Kaczmarek, A.T., Nagy, P., Schwarz, G.
The role of glutamate oxaloacetate transaminases in sulfite biosynthesis and H2S metabolism
(2021) Redox Biology, 38:101800

Mellis, A.T., Roeper, J., Misko, A.L., Kohl, J.B., Schwarz, G.
Sulfite alters the mitochondrial network in molybdenum cofactor deficiency
(2021) Frontiers in Genetics, 11:594828

Bender, D., Kaczmarek, A.T., Kuester, S., Burlina, A.B., Schwarz, G.
Oxygen and nitrite reduction by heme-deficient sulphite oxidase in a patient with mild sulphite oxidase deficiency
(2020) Journal of Inherited Metabolic Disease, 43(4), pp. 748-757

van Schie, M.M.C.H., Kaczmarek, A.T., Tieves, F., Gomez de Santos, P., Paul, C.E. Arends, I.W.C.E., Alcalde, M., Schwarz, G., Hollmann, F.
Selective Oxyfunctionalisation Reactions Driven by Sulfite Oxidase-Catalysed In Situ Generation of H2O2
(2020) ChemCatChem, 12(12), pp. 3186-3189

Mayr, S.J., Röper, J., Schwarz, G.
Alternative splicing of the bicistronic gene molybdenum cofactor synthesis 1 (MOCS1) uncovers a novel mitochondrial protein maturation mechanism
(2020) Journal of Biological Chemistry, 295(10), pp. 3029-3039

Zivanovic, J., Kouroussis, E., Kohl, J.B., Adhikari, B., Bursac, B., Schott-Roux, S., Petrovic, D., Miljkovic, J.L., Thomas-Lopez, D., Jung, Y., Miler, M., Mitchell, S., Milosevic, V., Gomes, J.E., Benhar, M., Gonzalez-Zorn, B., Ivanovic-Burmazovic, I., Torregrossa, R., Mitchell, J.R., Whiteman, M., Schwarz, G., Snyder, S.H., Paul, B.D., Carroll, K.S., Filipovic, M.R.
Selective persulfide detection reveals evolutionarily conserved antiaging effects of S-sulfhydration
(2020) Cell Metabolism, 31(1), pp. 1152-1170.e13

Bender D., Kaczmarek, A.T., Niks, D., Hille, R., Schwarz, G.
Mechanism of nitrite-dependent NO synthesis by human sulfite oxidase
(2019) Biochemical Journal, 476(12), pp. 1805-1815.

Bender D., Kaczmarek, A.T., Santamaria-Araujo, J.A., Stueve, B., Waltz, S., Bartsch, D., Kurian, L.,
Cirak, S., Schwarz, G.
Impaired mitochondrial maturation of sulfite oxidase in a patient with severe sulfite oxidase deficiency
(2019) Human Molecular Genetics, 28(17), pp. 2885-2899.

Kaczmarek, A.T., Strampraad, M.J.F., Hagedoorn, P.-L., Schwarz, G.
Reciprocal regulation of sulfite oxidation and nitrite reduction by mitochondrial sulfite oxidase
(2019) Nitric Oxide - Biology and Chemistry, 89, pp. 22-31.

Mohn, M.A., Thaqi, B., Fischer-Schrader, K.
Isoform-specific NO synthesis by Arabidopsis thaliana nitrate reductase.
(2019) Plants, 8 (3) e67.

Nagy, P., Schwarz, G., Kopriva, S.
Highlighted mechanistic aspects in the chemical biology of reactive sulfur species
(2019) British Journal of Pharmacology, 176 (4), pp. 511-513. Editorial

Kohl, J.B., Mellis, A.-T., Schwarz, G.
Homeostatic impact of sulfite and hydrogen sulfide on cysteine catabolism
(2019) British Journal of Pharmacology, 176 (4), pp. 554-570. Review

Bender, D., Schwarz, G.
Nitrite-dependent nitric oxide synthesis by molybdenum enzymes
(2018) FEBS Letters, 592 (12), pp. 2126-2139. Review

Lubout, C.M.A., Derks, T.G.J., Meiners, L., Erwich, J.J., Bergman, K.A., Lunsing, R.J., Schwarz, G., Veldman, A., van Spronsen, F.J.
Molybdenum cofactor deficiency type A: Prenatal monitoring using MRI
(2018) European Journal of Paediatric Neurology, 22 (3), pp. 536-540.

Mayr, S.J., Sass, J.O., Vry, J., Kirschner, J., Mader, I., Hövener, J.-B., Reiss, J., Santamaria-Araujo, J.A., Schwarz, G., Grünert, S.C.
A mild case of molybdenum cofactor deficiency defines an alternative route of MOCS1 protein maturation
(2018) Journal of Inherited Metabolic Disease, 41 (2), pp. 187-196.

Grünewald, N., Jan, A., Salvatico, C., Kress, V., Renner, M., Triller, A., Specht, C.G., Schwarz, G.
Sequences flanking the gephyrin-binding site of glyrβ tune receptor stabilization at synapses
(2018) eNeuro, 5 (1), art. no. e0042-17.2018,

Kumar, A., Dejanovic, B., Hetsch, F., Semtner, M., Fusca, D., Arjune, S., Santamaria-Araujo, J.A., Winkelmann, A., Ayton, S., Bush, A.I., Kloppenburg, P., Meier, J.C., Schwarz, G., Belaidi, A.A.
S-sulfocysteine/NMDA receptor–dependent signaling underlies neurodegeneration in molybdenum cofactor deficiency
(2017) Journal of Clinical Investigation, 127 (12), pp. 4365-4378.

Kalimuthu, P., Belaidi, A.A., Schwarz, G., Bernhardt, P.V.
Chitosan-Promoted Direct Electrochemistry of Human Sulfite Oxidase
(2017) Journal of Physical Chemistry B, 121 (39), pp. 9149-9159.

Kalimuthu, P., Belaidi, A.A., Schwarz, G., Bernhardt, P.V.
Mediated Catalytic Voltammetry of Holo and Heme-Free Human Sulfite Oxidases
(2017) ChemElectroChem, 4 (4), pp. 947-956.

Schwarz, G.
7201633808;
Molybdenum - From basic metabolism to neurobiology and blood pressure regulation [Molybdän – vom Grundstoffwechsel zur Neurobiologie und Blutdruckregulation]
(2017) BioSpektrum, 23 (1), pp. 18-21.

Kappler, U., Schwarz, G.
The Sulfite Oxidase Family of Molybdenum Enzymes
(2017) RSC Metallobiology, 2017-January (5), pp. 240-273. Book Chapter

Belaidi, A.A., Schwarz, G.
Molybdenum Cofactor in Humans: Health, Disease, and Treatment
(2016) Molecular, Genetic, and Nutritional Aspects of Major and Trace Minerals, pp. 399-410. Book Chapter

Jakubiczka-Smorag, J., Santamaria-Araujo, J.A., Metz, I., Kumar, A., Hakroush, S., Brueck, W., Schwarz, G., Burfeind, P., Reiss, J., Smorag, L.
Mouse model for molybdenum cofactor deficiency type B recapitulates the phenotype observed in molybdenum cofactor deficient patients
(2016) Human Genetics, 135 (7), pp. 813-826.

Kalimuthu, P., Belaidi, A.A., Schwarz, G., Bernhardt, P.V.
Low Potential Catalytic Voltammetry of Human Sulfite Oxidase
(2016) Electrochimica Acta, 199, pp. 280-289.

Schwarz, G.
Molybdenum cofactor and human disease
(2016) Current Opinion in Chemical Biology, 31, pp. 179-187. Review

Lal, D., Reinthaler, E.M., Dejanovic, B., May, P., Thiele, H., Lehesjoki, A.-E., Schwarz, G., Riesch, E., Ikram, M.A., Van Duijn, C.M., Uitterlinden, A.G., Hofman, A., Steinböck, H., Gruber-Sedlmayr, U., Neophytou, B., Zara, F., Hahn, A., Gormley, P., Becker, F., Weber, Y.G., Cilio, M.R., Kunz, W.S., Krause, R., Zimprich, F., Lemke, J.R., Nürnberg, P., Sander, T., Lerche, H., Neubauer, B.A., Palotie, A., Ruppert, A.-K., Suls, A., Siren, A., Koeleman, B., Haberlandt, E., Ronen, G.M., Caglayan, H., Hjalgrim, H., Muhle, H., Schulz, H., Helbig, I., Altmüller, J., Geldner, J., Schubert, J., Jabbari, K., Everett, K., Feucht, M., Balestri, M., Nothnagel, M., Striano, P., Møller, R.S., Nabbout, R., Balling, R., Baulac, S., Bianchi, A., La Neve, A., Minetti, C., Giuseppe, C.
Evaluation of presumably disease causing SCN1A variants in a cohort of common epilepsy syndromes
(2016) PLoS ONE, 11 (3), art. no. e0150426,

Dejanovic, B., Djémié, T., Grünewald, N., Suls, A., Kress, V., Hetsch, F., Craiu, D., Zemel, M., Gormley, P., Lal, D., Myers, C.T., Mefford, H.C., Palotie, A., Helbig, I., Meier, J.C., De Jonghe, P., Weckhuysen, S., Schwarz, G.
Simultaneous impairment of neuronal and metabolic function of mutated gephyrin in a patient with epileptic encephalopathy
(2015) EMBO Molecular Medicine, 7 (12), pp. 1580-1594.

Schwahn, B.C., Van Spronsen, F.J., Belaidi, A.A., Bowhay, S., Christodoulou, J., Derks, T.G., Hennermann, J.B., Jameson, E., König, K., McGregor, T.L., Font-Montgomery, E., Santamaria-Araujo, J.A., Santra, S., Vaidya, M., Vierzig, A., Wassmer, E., Weis, I., Wong, F.Y., Veldman, A., Schwarz, G.
Efficacy and safety of cyclic pyranopterin monophosphate substitution in severe molybdenum cofactor deficiency type A: A prospective cohort study
(2015) The Lancet, 386 (10007), pp. 1955-1963.

Imhof, T., Gruenewald, N., Schwarz, G., Noack, M.J., Koch, M.
Modified amelogenin is a new and versatile nanomaterial for biomedical applications
(2015) Biotechnology and Bioengineering, 112 (8), pp. 1708-1713.

Wang, J., Krizowski, S., Fischer-Schrader, K., Niks, D., Tejero, J., Sparacino-Watkins, C., Wang, L., Ragireddy, V., Frizzell, S., Kelley, E.E., Zhang, Y., Basu, P., Hille, R., Schwarz, G., Gladwin, M.T.
Sulfite Oxidase Catalyzes Single-Electron Transfer at Molybdenum Domain to Reduce Nitrite to Nitric Oxide
(2015) Antioxidants and Redox Signaling, 23 (4), pp. 283-294.

Belaidi, A.A., Röper, J., Arjune, S., Krizowski, S., Trifunovic, A., Schwarz, G.
Oxygen reactivity of mammalian sulfite oxidase provides a concept for the treatment of sulfite oxidase deficiency
(2015) Biochemical Journal, 469 (2), pp. 211-221.

Reinthaler, E.M., Dejanovic, B., Lal, D., Semtner, M., Merkler, Y., Reinhold, A., Pittrich, D.A., Hotzy, C., Feucht, M., Steinböck, H., Gruber-Sedlmayr, U., Ronen, G.M., Neophytou, B., Geldner, J., Haberlandt, E., Muhle, H., Ikram, M.A., Van Duijn, C.M., Uitterlinden, A.G., Hofman, A., Altmüller, J., Kawalia, A., Toliat, M.R., Nürnberg, P., Lerche, H., Nothnagel, M., Thiele, H., Sander, T., Meier, J.C., Schwarz, G., Neubauer, B.A., Zimprich, F.
Rare variants in γ-aminobutyric acid type A receptor genes in rolandic epilepsy and related syndromes
(2015) Annals of Neurology, 77 (6), pp. 972-986.

Winkelmann, A., You, X., Grünewald, N., Häussler, U., Krestel, H., Haas, C.A., Schwarz, G., Chen, W., Meier, J.C.
Identification of a new genomic hot spot of evolutionary diversification of protein function
(2015) PLoS ONE, 10 (5), art. no. e0125413,

Edwards, M., Roeper, J., Allgood, C., Chin, R., Santamaria, J., Wong, F., Schwarz, G., Whitehall, J.
Investigation of molybdenum cofactor deficiency due to MOCS2 deficiency in a newborn baby
(2015) Meta Gene, 3, pp. 43-49.

Arjune, S., Schwarz, G., Belaidi, A.A.
Involvement of the Cys-Tyr cofactor on iron binding in the active site of human cysteine dioxygenase
(2015) Amino Acids, 47 (1), pp. 55-63. Cited 8 times.

Kalimuthu, P., Fischer-Schrader, K., Schwarz, G., Bernhardt, P.V.
A sensitive and stable amperometric nitrate biosensor employing Arabidopsis thaliana nitrate reductase
(2015) Journal of Biological Inorganic Chemistry, 20 (2), pp. 385-393.

Chi, J.-C., Roeper, J., Schwarz, G., Fischer-Schrader, K.
Dual binding of 14-3-3 protein regulates Arabidopsis nitrate reductase activity
(2015) Journal of Biological Inorganic Chemistry, 20 (2), pp. 277-286.

Winkelmann, A., Maggio, N., Eller, J., Caliskan, G., Semtner, M., Häussler, U., Jüttner, R., Dugladze, T., Smolinsky, B., Kowalczyk, S., Chronowska, E., Schwarz, G., Rathjen, F.G., Rechavi, G., Haas, C.A., Kulik, A., Gloveli, T., Heinemann, U., Meier, J.C.
Changes in neural network homeostasis trigger neuropsychiatric symptoms
(2014) Journal of Clinical Investigation, 124 (2), pp. 696-711.

Fröhlich, M., Dejanovic, B., Kashkar, H., Schwarz, G., Nussberger, S.
S-palmitoylation represents a novel mechanism regulating the mitochondrial targeting of BAX and initiation of apoptosis
(2014) Cell Death and Disease, 5 (2), art. no. e1057,

Klein, E.L., Belaidi, A.A., Raitsimring, A.M., Davis, A.C., Krämer, T., Astashkin, A.V., Neese, F., Schwarz, G., Enemark, J.H.
Pulsed electron paramagnetic resonance spectroscopy of 33S- labeled molybdenum cofactor in catalytically active bioengineered sulfite oxidase
(2014) Inorganic Chemistry, 53 (2), pp. 961-971.

Hagmann, H., Bossung, V., Ali Belaidi, A., Fridman, A., Karumanchi, S.A., Thadhani, R., Schermer, B., Mallmann, P., Schwarz, G., Benzing, T., Brinkkoetter, P.T.
Low-molecular weight heparin increases circulating sFlt-1 levels and enhances urinary elimination
(2014) PLoS ONE, 9 (1), art. no. e85258.

Havarushka, N., Fischer-Schrader, K., Lamkemeyer, T., Schwarz, G.
Structural basis of thermal stability of the tungsten cofactor synthesis protein MoaB from Pyrococcus furiosus
(2014) PLoS ONE, 9 (1), art. no. e86030,

Tejada-Jiménez, M., Schwarz, G.
Molybdenum and tungsten
(2014) RSC Metallobiology, 2014-January (2), pp. 223-259. Review

Dejanovic, B., Schwarz, G.
Neuronal nitric oxide synthase-dependent s-nitrosylation of gephyrin regulates gephyrin clustering at GABAergic synapses
(2014) Journal of Neuroscience, 34 (23), pp. 7763-7768.

Dejanovic, B., Semtner, M., Ebert, S., Lamkemeyer, T., Neuser, F., Lüscher, B., Meier, J.C., Schwarz, G.
Palmitoylation of Gephyrin Controls Receptor Clustering and Plasticity of GABAergic Synapses
(2014) PLoS Biology, 12 (7), art. no. e1001908, pp. 1-16.

Keusekotten, K., Bade, V.N., Meyer-Teschendorf, K., Sriramachandran, A.M., Fischer-Schrader, K., Krause, A., Horst, C., Schwarz, G., Hofmann, K., Dohmen, R.J., Praefcke, G.J.K.
Multivalent interactions of the SUMO-interaction motifs in RING finger protein 4 determine the specificity for chains of the SUMO
(2014) Biochemical Journal, 457 (1), pp. 207-214. Cited 15 times.

Dejanovic, B., Lal, D., Catarino, C.B., Arjune, S., Belaidi, A.A., Trucks, H., Vollmar, C., Surges, R., Kunz, W.S., Motameny, S., Altmüller, J., Köhler, A., Neubauer, B.A., EPICURE Consortium, Nürnberg, P., Noachtar, S., Schwarz, G., Sander, T.
Exonic microdeletions of the gephyrin gene impair GABAergic synaptic inhibition in patients with idiopathic generalized epilepsy
(2014) Neurobiology of Disease, 67, pp. 88-96.

Kalimuthu, P., Fischer-Schrader, K., Schwarz, G., Bernhardt, P.V.
Mediated electrochemistry of nitrate reductase from Arabidopsis thaliana
(2013) Journal of Physical Chemistry B, 117 (25), pp. 7569-7577.

Clinch, K., Watt, D.K., Dixon, R.A., Baars, S.M., Gainsford, G.J., Tiwari, A., Schwarz, G., Saotome, Y., Storek, M., Belaidi, A.A., Santamaria-Araujo, J.A.
Synthesis of cyclic pyranopterin monophosphate, a biosynthetic intermediate in the molybdenum cofactor pathway
(2013) Journal of Medicinal Chemistry, 56 (4), pp. 1730-1738.

Belaidi, A.A., Schwarz, G.
Metal insertion into the molybdenum cofactor: Product-substrate channelling demonstrates the functional origin of domain fusion in gephyrin
(2013) Biochemical Journal, 450 (1), pp. 149-157.

Kowalczyk, S., Winkelmann, A., Smolinsky, B., Förstera, B., Neundorf, I., Schwarz, G., Meier, J.C.
Direct binding of GABA<inf>A</inf> receptor β2 and β3 subunits to gephyrin
(2013) European Journal of Neuroscience, 37 (4), pp. 544-554.

Schwarz, G., Belaidi, A.A.
Molybdenum in human health and disease
(2013) Metal Ions in Life Sciences, 13, pp. 415-450.

Belaidi, A.A., Schwarz, G.
37036821500;7201633808;
Molybdenum cofactor deficiency: Metabolic link between taurine and S-sulfocysteine
(2013) Advances in Experimental Medicine and Biology, 776, pp. 13-19.

Klein, J.M., Busch, J.D., Pottings, C., Baker, M.J., Langers, T., Schwarz, G.
The mitochondrial amidoxime-reducing component (mARC1) is a novel signal-anchored protein of the outer mitochondrial membrane
(2012) Journal of Biological Chemistry, 287 (51), pp. 42795-42803.

Klein, J.M., Schwarz, G.
Cofactor-dependent maturation of mammalian sulfite oxidase links two mitochondrial import pathways
(2012) Journal of Cell Science, 125 (20), pp. 4876-4855.

Hitzert, M.M., Bos, A.F., Bergman, K.A., Veldman, A., Schwarz, G., Santamaria-Araujo, J.A., Heiner-Fokkema, R., Sival, D.A., Lunsing, R.J., Arjune, S., Kosterink, J.G.W., Van Spronsen, F.J.
Favorable outcome in a newborn with molybdenum cofactor type A deficiency treated with cPMP
(2012) Pediatrics, 130 (4), pp. e1005-e1010.

Herweg, J., Schwarz, G.
Splice-specific glycine receptor binding, folding, and phosphorylation of the scaffolding protein gephyrin
(2012) Journal of Biological Chemistry, 287 (16), pp. 12645-12656.

Lambeck, I.C., Fischer-Schrader, K., Niks, D., Roeper, J., Chi, J.-H., Hille, R., Schwarz, G.
Molecular mechanism of 14-3-3 protein-mediated inhibition of plant nitrate reductase
(2012) Journal of Biological Chemistry, 287 (7), pp. 4562-4571.

Santamaria-Araujo, J.A., Wray, V., Schwarz, G.
Structure and stability of the molybdenum cofactor intermediate cyclic pyranopterin monophosphate
(2012) Journal of Biological Inorganic Chemistry, 17 (1), pp. 113-122.

Belaidi, A.A., Arjune, S., Santamaria-Araujo, J.A., Sass, J.O., Schwarz, G.
Molybdenum cofactor deficiency: A new HPLC method for fast quantification of s-sulfocysteine in urine and serum
(2012) JIMD Reports, 5, pp. 35-43. Book Chapter

Bevers, L.E., Schwarz, G., Hagen, W.R.
A molecular basis for tungstate selectivity in prokaryotic ABC transport systems
(2011) Journal of Bacteriology, 193 (18), pp. 4999-5001.

Veldman, A., Hennermann, J.B., Schwarz, G., Van Spronsen, F., Weis, I., Wong, F.Y., Schwahn, B.C.
Timing of cerebral developmental disruption in molybdenum cofactor deficiency
(2011) Journal of Child Neurology, 26 (8), pp. 1059-1060.

Mendel, R.R., Schwarz, G.
Molybdenum cofactor biosynthesis in plants and humans
(2011) Coordination Chemistry Reviews, 255 (9-10), pp. 1145-1158. Review

Ogino, K., Ramsden, S.L., Keib, N., Schwarz, G., Harvey, R.J., Hirata, H.
Duplicated gephyrin genes showing distinct tissue distribution and alternative splicing patterns mediate molybdenum cofactor biosynthesis, glycine receptor clustering, and escape behavior in zebrafish
(2011) Journal of Biological Chemistry, 286 (1), pp. 806-817.

Specht, C.G., Grünewald, N., Pascual, O., Rostgaard, N., Schwarz, G., Triller, A.
Regulation of glycine receptor diffusion properties and gephyrin interactions by protein kinase C
(2011) EMBO Journal, 30 (18), pp. 3842-3853.

Förstera, B., Belaidi, A.A., Jüttner, R., Bernert, C., Tsokos, M., Lehmann, T.-N., Horn, P., Dehnicke, C., Schwarz, G., Meiere, J.C.
Irregular RNA splicing curtails postsynaptic gephyrin in the cornu ammonis of patients with epilepsy
(2010) Brain, 133 (12), pp. 3778-3794.

Lambeck, I., Chi, J.-C., Krizowski, S., Mueller, S., Mehlmer, N., Teige, M., Fischer, K., Schwarz, G.
Kinetic analysis of 14-3-3-inhibited arabidopsis thaliana nitrate reductase
(2010) Biochemistry, 49 (37), pp. 8177-8186.

Veldman, A., Santamaria-Araujo, J.A., Sollazzo, S., Pitt, J., Gianello, R., Yaplito-Lee, J., Wong, F., Ramsden, C.A., Reiss, J., Cook, I., Fairweather, J., Schwarz, G.
Successful treatment of molybdenum cofactor deficiency type a with cPMP
(2010) Pediatrics, 125 (5), pp. e1249-e1254.

Kolmos, E., Nowak, M., Werner, M., Fischer, K., Schwarz, G., Mathews, S., Schoof, H., Nagy, F., Bujnicki, J.M., Davis, S.J.
Integrating ELF4 into the circadian system through combined structural and functional studies
(2009) HFSP Journal, 3 (5), pp. 350-366.

Eichler, S.A., Förstera, B., Smolinsky, B., Jüttner, R., Lehmann, T.-N., Fähling, M., Schwarz, G., Legendre, P., Meier, J.C.
Splice-specific roles of glycine receptor α3 in the hippocampus
(2009) European Journal of Neuroscience, 30 (6), pp. 1077-1091.

Schwarz, G., Mendel, R.R., Ribbe, M.W.
Molybdenum cofactors, enzymes and pathways
(2009) Nature, 460 (7257), pp. 839-847. Review

Smolinsky, B., Eichler, S.A., Buchmeier, S., Meier, J.C., Schwarz, G.
Splice-specific functions of gephyrin in molybdenum cofactor biosynthesis
(2008) Journal of Biological Chemistry, 283 (25), pp. 17370-17379.

Fritschy, J.-M., Harvey, R.J., Schwarz, G.
Gephyrin: where do we stand, where do we go?
(2008) Trends in Neurosciences, 31 (5), pp. 257-264. Review

Bevers, L.E., Hagedoorn, P.-L., Santamaria-Araujo, J.A., Magalon, A., Hagen, W.R., Schwarz, G.
Function of MoaB proteins in the biosynthesis of the molybdenum and tungsten cofactors
(2008) Biochemistry, 47 (3), pp. 949-956.

Llamas, Á., Tejada-Jimenez, M., González-Ballester, D., Higuera, J.J., Schwarz, G., Galván, A., Fernández, E.
Chlamydomonas reinhardtii CNX1E reconstitutes molybdenum cofactor biosynthesis in Escherichia coli mutants
(2007) Eukaryotic Cell, 6 (6), pp. 1063-1067.

Lardi-Studler, B., Smolinsky, B., Petitjean, C.M., Koenig, F., Sidler, C., Meier, J.C., Fritschy, J.-M., Schwarz, G.
Vertebrate-specific sequences in the gephyrin E-domain regulate cytosolic aggregation and postsynaptic clustering
(2007) Journal of Cell Science, 120 (8), pp. 1371-1382.

Fischer, K., Llamas, A., Tejada-Jimenez, M., Schrader, N., Kuper, J., Ataya, F.S., Galvan, A., Mendel, R.R., Fernandez, E., Schwarz, G.
Function and structure of the molybdenum cofactor carrier protein from Chlamydomonas reinhardtii
(2006) Journal of Biological Chemistry, 281 (40), pp. 30186-30194.

Schwarz, G., Mendel, R.R.
Molybdenum cofactor biosynthesis and molybdenum enzymes
(2006) Annual Review of Plant Biology, 57, pp. 623-647. Review

Llamas, A., Otte, T., Multhaup, G., Mendel, R.R., Schwarz, G.
The mechanism of nucleotide-assisted molybdenum insertion into molybdopterin: A novel route toward metal cofactor assembly
(2006) Journal of Biological Chemistry, 281 (27), pp. 18343-18350.

Xiang, S., Kim, E.Y., Connelly, J.J., Nassar, N., Kirsch, J., Winking, J., Schwarz, G., Schindelin, H.
The Crystal Structure of Cdc42 in Complex with Collybistin II, a Gephyrin-interacting Guanine Nucleotide Exchange Factor
(2006) Journal of Molecular Biology, 359 (1), pp. 35-46.

Eun, Y.K., Schrader, N., Smolinsky, B., Bedet, C., Vannier, C., Schwarz, G., Schindelin, H.
Deciphering the structural framework of glycine receptor anchoring by gephyrin
(2006) EMBO Journal, 25 (6), pp. 1385-1395.

Schwarz, G.
Molybdenum cofactor biosynthesis and deficiency
(2005) Cellular and Molecular Life Sciences, 62 (23), pp. 2792-2810. Review

Fischer, K., Barbier, G.G., Hecht, H.-J., Mendel, R.R., Campbell, W.H., Schwarz, G.
Structural basis of eukaryotic nitrate reduction: Crystal structures of the nitrate reductase active site
(2005) Plant Cell, 17 (4), pp. 1167-1179.

Hemann, C., Hood, B.L., Fulton, M., Hänsch, R., Schwarz, G., Mendel, R.R., Kirk, M.L., Hille, R.
Spectroscopic and kinetic studies of arabidopsis thaliana sulfite oxidase: Nature of the redox-active orbital and electronic structure contributions to catalysis
(2005) Journal of the American Chemical Society, 127 (47), pp. 16567-16577.

Godber, B.L.J., Schwarz, G., Mendel, R.R., Lowe, D.J., Bray, R.C., Eisenthal, R., Harrison, R.
Molecular characterization of human xanthine oxidoreductase: The enzyme is grossly deficient in molybdenum and substantially deficient in iron-sulphur centres
(2005) Biochemical Journal, 388 (2), pp. 501-508.

Schwarz, G., Reiss, J.
Curing of the formerly lethal molybdenum cofactor deficiency [Heilung der bisher tödlich verlaufenden molybdän-cofaktor- defizienz]
(2005) BioSpektrum, 11 (1), pp. 88-90.

Reiss, J., Bonin, M., Schwegler, H., Sass, J.O., Garattini, E., Wagner, S., Lee, H.-J., Engel, W., Riess, O., Schwarz, G.
The pathogenesis of molybdenum cofactor deficiency, its delay by maternal clearance, and its expression pattern in microarray analysis
(2005) Molecular Genetics and Metabolism, 85 (1), pp. 12-20.

Llamas, A., Mendel, R.R., Schwarz, G.
Synthesis of adenylated molybdopterin: An essential step for molybdenum insertion
(2004) Journal of Biological Chemistry, 279 (53), pp. 55241-55246.

Kuper, J., Llamas, A., Hecht, H.-J., Mendel, R.R., Schwarz, G.
Structure of the molybdopterin-bound Cnx1G domain links molybdenum and copper metabolism
(2004) Nature, 430 (7001), pp. 803-806.

Jockusch, B.M., Rothkegel, M., Schwarz, G.
Linking the synapse to the cytoskeleton: A breath-taking role for microfilaments
(2004) NeuroReport, 15 (10), pp. 1535-1538. Review

Schwarz, G., Santamaria-Araujo, J.A., Wolf, S., Lee, H.-J., Adham, I.M., Gröne, H.-J., Schwegler, H., Sass, J.O., Otte, T., Hänzelmann, P., Mendel, R.R., Engel, W., Reiss, J.
Rescue of lethal molybdenum cofactor deficiency by a biosynthetic precursor from Escherichia coli
(2004) Human Molecular Genetics, 13 (12), pp. 1249-1255.

Schrader, N., Kim, E.Y., Winking, J., Paulukat, J., Schindelin, H., Schwarz, G.
Biochemical Characterization of the High Affinity Binding between the Glycine Receptor and Gephyrin
(2004) Journal of Biological Chemistry, 279 (18), pp. 18733-18741.

Santamaria-Araujo, J.A., Fischer, B., Otte, T., Nimtz, M., Mendel, R.R., Wray, V., Schwarz, G.
The Tetrahydropyranopterin Structure of the Sulfur-free and Metal-free Molybdenum Cofactor Precursor
(2004) Journal of Biological Chemistry, 279 (16), pp. 15994-15999.

Schrader, N., Fischer, K., Theis, K., Mendel, R.R., Schwarz, G., Kisker, C.
The crystal structure of plant sulfite oxidase provides insights into sulfite oxidation in plants and animals
(2003) Structure, 11 (10), pp. 1251-1263.

Giesemann, T., Schwarz, G., Nawrotzki, R., Berhörster, K., Rothkegel, M., Schlüter, K., Schrader, N., Schindelin, H., Mendel, R.R., Kirsch, J., Jockusch, B.M.
Complex formation between the postsynaptic scaffolding protein gephyrin, profilin, and Mena: A possible link to the microfilament system
(2003) Journal of Neuroscience, 23 (23), pp. 8330-8339.

Guse, A., Stevenson, C.E.M., Kuper, J., Buchanan, G., Schwarz, G., Giordano, G., Magalon, A., Mendel, R.R., Lawson, D.M., Palmer, T.
Biochemical and structural analysis of the molybdenum cofactor biosynthesis protein MobA
(2003) Journal of Biological Chemistry, 278 (28), pp. 25302-25307.

Kuper, J., Winking, J., Hecht, H.-J., Mendel, R.R., Schwarz, G.
The active site of the molybdenum cofactor biosynthetic protein domain Cnx1G
(2003) Archives of Biochemistry and Biophysics, 411 (1), pp. 36-46.

Kuper, J., Zu Berstenhorst, S.M., Vödisch, B., Mendel, R.R., Schwarz, G., Boxer, D.H.
In vivo detection of molybdate-binding proteins using a competition assay with ModE in Escherichia coli
(2003) FEMS Microbiology Letters, 218 (1), pp. 187-193.

Lee, H.-J., Adham, I.M., Schwarz, G., Kneussel, M., Sass, J.O., Engel, W., Reiss, J.
Molybdenum cofactor-deficient mice resemble the phenotype of human patients
(2002) Human Molecular Genetics, 11 (26), pp. 3309-3317.

Hänzelmann, P., Schwarz, G., Mendel, R.R.
Functionality of alternative splice forms of the first enzymes involved in human molybdenum cofactor biosynthesis
(2002) Journal of Biological Chemistry, 277 (21), pp. 18303-18312.

Mendel, R.R., Schwarz, G.
Biosynthesis and molecular biology of the molybdenum cofactor (Moco)
(2002) Metals Ions in Biological System: Molybdenum and Tungsten: Their Roles in Biological Processes, 39, pp. 317-368. Book Chapter

Eilers, T., Schwarz, G., Brinkmann, H., Witt, C., Richter, T., Nieder, J., Koch, B., Hille, R., Hänsch, R., Mendel, R.R.
Identification and biochemical characterization of Arabidopsis thaliana sulfite oxidase: A new player in plant sulfur metabolism
(2001) Journal of Biological Chemistry, 276 (50), pp. 46989-46994.

Gutzke, G., Fischer, B., Mendel, R.R., Schwarz, G.
Thiocarboxylation of Molybdopterin Synthase Provides Evidence for the Mechanism of Dithiolene Formation in Metal-binding Pterins
(2001) Journal of Biological Chemistry, 276 (39), pp. 36268-36274.

Schwarz, G., Schrader, N., Mendel, R.R., Hecht, H.-J., Schindelin, H.
Crystal structures of human gephyrin and plant Cnx1 G domains: Comparative analysis and functional implications
(2001) Journal of Molecular Biology, 312 (2), pp. 405-418.

Buchanan, G., Kuper, J., Mendel, R.R., Schwarz, G., Palmer, T.
Characterisation of the mob locus of Rhodobacter sphaeroides ws8: mobA is the only gene required for molybdopterin guanine dinucleotide synthesis
(2001) Archives of Microbiology, 176 (1-2), pp. 62-68.

Reiss, J., Gross-Hardt, S., Christensen, E., Schmidt, P., Mendel, R.R., Schwarz, G.
A mutation in the gene for the neurotransmitter receptor-clustering protein gephyrin causes a novel form of molybdenum cofactor deficiency
(2001) American Journal of Human Genetics, 68 (1), pp. 208-213.

Schwarz, G., Schulze, J., Bittner, F., Eilers, T., Kuper, J., Bollmann, G., Nerlich, A., Brinkmann, H., Mendel, R.R.
The molybdenum cofactor biosynthetic protein Cnx1 complements molybdate-repairable mutants, transfers molybdenum to the metal binding pterin, and is associated with the cytoskeleton
(2000) Plant Cell, 12 (12), pp. 2455-2471.

Kuper, J., Palmer, T., Mendel, R.R., Schwarz, G.
Mutations in the molybdenum cofactor biosynthetic protein Cnx1G from Arabidopsis thaliana define functions for molybdopterin binding, molybdenum insertion, and molybdenum cofactor stabilization
(2000) Proceedings of the National Academy of Sciences of the United States of America, 97 (12), pp. 6475-6480.

Kappler, U., Bennett, B., Rethmeier, J., Schwarz, G., Deutzmann, R., McEwan, A.G., Dahl, C.
Sulfite:cytochrome c oxidoreductase from Thiobacillus novellus. Purification, characterization, and molecular biology of a heterodimeric member of the sulfite oxidase family
(2000) Journal of Biological Chemistry, 275 (18), pp. 13202-13212.

Leimkühler, S., Angermüller, S., Schwarz, G., Mendel, R.R., Klipp, W.
Activity of the molybdopterin-containing xanthine dehydrogenase of Rhodobacter capsulatus can be restored by high molybdenum concentrations in a moeA mutant defective in molybdenum cofactor biosynthesis
(1999) Journal of Bacteriology, 181 (19), pp. 5930-5939.

Stallmeyer, B., Schwarz, G., Schulze, J., Nerlich, A., Reiss, J., Kirsch, J., Mendel, R.R.
The neurotransmitter receptor-anchoring protein gephyrin reconstitutes molybdenum cofactor biosynthesis in bacteria, plants, and mammalian cells
(1999) Proceedings of the National Academy of Sciences of the United States of America, 96 (4), pp. 1333-1338.

Mendel, R.R., Schwarz, G.
Molybdoenzymes and molybdenum cofactor in plants
(1999) Critical Reviews in Plant Sciences, 18 (1), pp. 33-69. Review

Witte, C.-P., Igeño, M.I., Mendel, R., Schwarz, G., Fernández, E.
The Chlamydomonas reinhardtii MoCo carrier protein is multimeric and stabilizes molybdopteria cofactor in a molybdate charged form
(1998) FEBS Letters, 431 (2), pp. 205-209.

Pauk, J., Hänsch, R., Schwarz, G., Nerlich, A., Monostori, T., Mészáros, A., Jenes, B., Kertész, Z., Matuz, J., Schulze, J., Mendel, R.R.
Genetic transformation of wheat (Triticum aestivum L) in Hungary
(1998) Novenytermeles, 47 (3), pp. 242-251.

Leimkühler, S., Kern, M., Solomon, P.S., McEwan, A.G., Schwarz, G., Mendel, R.R., Klipp, W.
Xanthine dehydrogenase from the phototrophic purple bacterium Rhodobacter capsulatus is more similar to its eukaryotic counterparts than to prokaryotic molybdenum enzymes
(1998) Molecular Microbiology, 27 (4), pp. 853-869.

Schwarz, G., Boxer, D.H., Mendel, R.R.
Molybdenum cofactor biosynthesis. The plant protein Cnx1 binds molybdopterin with high affinity
(1997) Journal of Biological Chemistry, 272 (43), pp. 26811-26814.