GERAMY - Netzwek für Leichtketten (AL-) Amyloidose

Die Leichtketten- (AL-) Amyloidose ist die häufigste Form der systemischen Amyloidosen. Sie ist eine seltene Proteinfaltungs-Erkrankung mit schlechter Prognose. Die AL-Amyloidose wird meist durch eine monoklonale Plasmazell-Erkrankung des Knochenmarks verursacht; hierbei kommt es zur Produktion aymloidbildender Leichtkettenproteine, die in verschiedenen Organen wie Herz, Niere, Leber, Magen-Darm-Trakt und Nervensystem akkumulieren.

Eine Frühdiagnose zu stellen ist sehr schwierig, da die initialen Symptome unspezifisch sind. Todesursache ist meistens Herzversagen aufgrund exzessiver kardialer Amyloid-Ablagerungen. Bis heute besteht die einzige Evidenz-basierte Therapie darin, die Produktion der Amyloid-Vorläuferproteine durch Chemotherapie des zugrundeliegenden B-Zell Klons zu stoppen. Die zytostatische Therapie ist jedoch häufig mit starken Nebenwirkungen verbunden, insbesondere da bei den meisten Amyloidose-Patienten mehrere Organfunktionen eingeschränkt sind. Derzeit existiert keine geprüfte effektive, auf den Amyloidabbau gerichtete Behandlung für Patienten mit AL-Amyloidose.

Projekte

Prospektiv-randomisierte Studie zur Therapie mit EGCG vs. Placebo bei Patienten mit Herzamyloidose (PD Dr. S. Schönland, Heidelberg)
Genomische Analyse monoklonaler Plasmazellen zur Verbesserung des Verständnisses der Amyloid-Genese und Prognose bei Leichtketten-Amyloidose (Prof. A. Jauch, Heidelberg)
Klassifikation der systemischen Amyloidosen des Amyloid Registers Kiel mit Hilfe von Tissue-MALDI Imaging (Prof. C. Röcken, Kiel)
Strukturelle Homogenität und Polymorphismus von AL Amyloidfibrillen (Prof. Dr. M.Fändrich, Ulm)
MAS Festkörper-NMR Untersuchungen von Leichtketten Amyloidfibrillen und deren Wechselwirkung mit Kleinmolekülen (Prof. B. Reif, TU München)
In-vitro Untersuchung zur sequenzspezifischen Therapie (Prof. Bieschke, Berlin und St. Louis)
Identifizierung neuer modulierender Moleküle für die Leichtketten-Aggregation (Prof. E. Wanker, Berlin)

Kontakt

Priv.-Doz. Dr. med. Stefan Schönland
Universitäts-Klinikum Heidelberg
Medizinische Klinik V (Hämatologie / Amyloidose-Zentrum Heidelberg)
Im Neuenheimer Feld 410
69120 Heidelberg
Tel.: 06221-56 8001
Fax: 06221-56 4659
E-Mail: stefan.schoenland@med.uni-heidelberg.de
Website: www.amyloidose-zentrum.de

Publikationen

1. Januar 2019 (Geramy)
GERAMY publication list

Details
4. April 2013 (Geramy)
Experimental determination of microsecond reorientation correlation times in protein solutions.

J Phys Chem B. 2013 Apr 4;117(13):3548-53. doi: 10.1021/jp312561f. Epub 2013 Mar 25.

Ravera E, Parigi G, Mainz A, Religa TL, Reif B, Luchinat C.
1. April 2013 (Geramy)
A Y2H-seq approach defines the human protein methyltransferase interactome.

Nat Methods. 2013 Apr;10(4):339-42. doi: 10.1038/nmeth.2397. Epub 2013 Mar 3.

Weimann M, Grossmann A, Woodsmith J, Özkan Z, Birth P, Meierhofer D, Benlasfer N, Valovka T, Timmermann B, Wanker EE, Sauer S, Stelzl U.
1. März 2013 (Geramy)
ATTR amyloid in the carpal tunnel ligament is frequently of wildtype transthyretin origin.

Amyloid. 2013 Mar;20(1):1-6.

Giova Z, Urban P, Meliss RR, Haag J, Axmann HD, Siebert F, Beckker K, Radtke HG, Röcken C.
1. März 2013 (Geramy)
Dynamic circadian protein-protein interaction networks predict temporal organization of cellular functions.

PLoS Genet. 2013 Mar;9(3):e1003398. doi: 10.1371/journal.pgen.1003398. Epub 2013 Mar 28.

Wallach T, Schellenberg K, Maier B, Kalathur RK, Porras P, Wanker EE, Futschik ME, Kramer A.
18. Februar 2013 (Geramy)
Epigallocatechin-3-gallate: a useful, effective and safe clinical approach for targeted prevention and individualised treatment of neurological diseases?

EPMA J. 2013 Feb 18;4(1):5. doi: 10.1186/1878-5085-4-5.

Mähler A, Mandel S, Lorenz M, Ruegg U, Wanker EE, Boschmann M, Paul F.
1. Februar 2013 (Geramy)
Development and application of a DNA microarray-based yeast two-hybrid system.

Nucleic Acids Res. 2013 Feb 1;41(3):1496-507. doi: 10.1093/nar/gks1329. Epub 2012 Dec 28.

Suter B, Fontaine JF, Yildirimman R, Raskó T, Schaefer MH, Rasche A, Porras P, Vázquez-Álvarez BM, Russ J, Rau K, Foulle R, Zenkner M, Saar K, Herwig R, Andrade-Navarro MA, Wanker EE.
1. Februar 2013 (Geramy)
Protein-RNA interfaces probed by 1H-detected MAS solid-state NMR spectroscopy.

Angew Chem Int Ed Engl. 2013 Feb 18;52(8):2345-9. doi: 10.1002/anie.201208024. Epub 2013 Jan 18.

Asami S, Rakwalska-Bange M, Carlomagno T, Reif B.
1. Januar 2013 (Geramy)
Translation of HTT mRNA with expanded CAG repeats is regulated by the MID1-PP2A protein complex.

Nat Commun. 2013;4:1511. doi: 10.1038/ncomms2514.

Krauss S, Griesche N, Jastrzebska E, Chen C, Rutschow D, Achmüller C, Dorn S, Boesch SM, Lalowski M, Wanker E, Schneider R, Schweiger S.
1. Januar 2013 (Geramy)
A functional deficiency of TERA/VCP/p97 contributes to impaired DNA repair in multiple polyglutamine diseases.

Nat Commun. 2013;4:1816. doi: 10.1038/ncomms2828.

Fujita K, Nakamura Y, Oka T, Ito H, Tamura T, Tagawa K, Sasabe T, Katsuta A, Motoki K, Shiwaku H, Sone M, Yoshida C, Katsuno M, Eishi Y, Murata M, Taylor JP, Wanker EE, Kono K, Tashiro S, Sobue G, La Spada AR, Okazawa H.
22. Oktober 2012 (Geramy)
New Criteria for Response to Treatment in Immunoglobulin Light Chain Amyloidosis Based on Free Light Chain Measurement and Cardiac Biomarkers: Impact on Survival Outcomes.

J Clin Oncol. 2012 Oct 22.

Palladini G, Dispenzieri A, Gertz MA, Kumar S, Wechalekar A, Hawkins PN, Schönland S, Hegenbart U, Comenzo R, Kastritis E, Dimopoulos MA, Jaccard A, Klersy C, Merlini G.
1. Oktober 2012 (Geramy)
Optimal degree of protonation.

J Biomol NMR. 2012 Oct;54(2):155-68. Epub 2012 Aug 23.PubMed PMID: 22915373.

Asami S, Szekely K, Schanda P, Meier BH, Reif B.
18. September 2012 (Geramy)
Longitudinal left ventricular function for prediction of survival in systemic light-chain amyloidosis: incremental value compared with clinical and biochemical markers.

J Am Coll Cardiol. 2012 Sep 18;60(12):1067-76. doi: 10.1016/j.jacc.2012.04.043. Epub 2012 Aug 8.

Buss SJ, Emami M, Mereles D, Korosoglou G, Kristen AV, Voss A, Schellberg D, Zugck C, Galuschky C, Giannitsis E, Hegenbart U, Ho AD, Katus HA, Schonland SO, Hardt SE.
1. September 2012 (Geramy)
In support of the BMRB.

Nat Struct Mol Biol. 2012 Sep;19(9):854-60. doi: 10.1038/nsmb.2371. PubMed PMID: 22955930.

Markley JL, Akutsu H, Asakura T, Baldus M, Boelens R, Bonvin A, Kaptein R, Bax
A, Bezsonova I, Gryk MR, Hoch JC, Korzhnev DM, Maciejewski MW, Case D, Chazin WJ,
Cross TA, Dames S, Kessler H, Lange O, Madl T, Reif B, Sattler M, Eliezer D,
Fersht A, Forman-Kay J, Kay LE, Fraser J, Gross J, Kortemme T, Sali A, Fujiwara
T, Gardner K, Luo X, Rizo-Rey J, Rosen M, Gil RR, Ho C, Rule G, Gronenborn AM,
Ishima R, Klein-Seetharaman J, Tang P, van der Wel P, Xu Y, Grzesiek S, Hiller S,
Seelig J, Laue ED, Mott H, Nietlispach D, Barsukov I, Lian LY, Middleton D,
Blumenschein T, Moore G, Campbell I, Schnell J, Vakonakis IJ, Watts A, Conte MR,
Mason J, Pfuhl M, Sanderson MR, Craven J, Williamson M, Dominguez C, Roberts G,
Günther U, Overduin M, Werner J, Williamson P, Blindauer C, Crump M, Driscoll P,
Frenkiel T, Golovanov A, Matthews S, Parkinson J, Uhrin D, Williams M, Neuhaus D,
Oschkinat H, Ramos A, Shaw DE, Steinbeck C, Vendruscolo M, Vuister GW, Walters
KJ, Weinstein H, Wüthrich K, Yokoyama S.
24. August 2012 (Geramy)
Structural properties of EGCG-induced, nontoxic Alzheimer’s disease Aβ oligomers.

J Mol Biol. 2012 Aug 24;421(4-5):517-24. Epub 2012 Jan 28. PubMed PMID: 22300765.

Lopez del Amo JM, Fink U, Dasari M, Grelle G, Wanker EE,Bieschke J, Reif B.
1. August 2012 (Geramy)
Amyloidose in Pneuomologie.

Pneumologie. 2012 Aug;66(8):483-92. Epub 2012 Jun 12. German.

Hagmeyer L, Stieglitz S, Röcken C, Randerath W.
1. August 2012 (Geramy)
Identification of human proteins that misfolding and proteotoxicity of pathogenic ataxin-1.

Petrakis S, Raskó T, Russ J, Friedrich RP, Stroedicke M, Riechers SP, Muehlenberg K, Möller A, Reinhardt A, Vinayagam A, Schaefer MH, Boutros M, Tricoire H, Andrade-Navarro MA, Wanker EE. modify PLoS Genet. 2012 Aug;8(8):e1002897. doi: 10.1371/journal.pgen.1002897.
18. Mai 2012 (Geramy)
Substrate transport activation is mediated through second periplasmic loop of transmembrane protein MalF in maltose transport complex of Escherichia coli.

J Biol Chem. 2012 May 18;287(21):17040-9.

Jacso T, Schneider E, Rupp B, Reif B.
1. Mai 2012 (Geramy)
Amyloidose des Herzens.

Der Pathologe. 2012 May;33(3):236-44

Kristen AV, Röcken C.
1. Mai 2012 (Geramy)
Evolution and function of CAG/polyglutamine repeats in protein-protein interaction networks.

Nucleic Acids Res. 2012 May;40(10):4273-87. doi: 10.1093/nar/gks011. Epub 2012 Jan 28.

Schaefer MH, Wanker EE, Andrade-Navarro MA.
10. April 2012 (Geramy)
Phosphorylation-regulated axonal dependent transport of syntaxin 1 is mediated by a Kinesin-1 adapter.

Chua JJ, Butkevich E, Worseck JM, Kittelmann M, Grønborg M, Behrmann E, Stelzl U, Pavlos NJ, Lalowski MM, Eimer S, Wanker EE, Klopfenstein DR, Jahn R.

Proc Natl Acad Sci U S A. 2012 Apr 10;109(15):5862-7. doi: 10.1073/pnas.1113819109.
1. März 2012 (Geramy)
Ultra-high resolution in MAS solid-state NMR of perdeuterated proteins: implications for structure and dynamics.

J Magn Reson. 2012 Mar;216:1-12.

Reif B.
14. Februar 2012 (Geramy)
HIPPIE: Integrating protein interaction networks with experiment based quality scores.

Schaefer MH, Fontaine JF, Vinayagam A, Porras P, Wanker EE, Andrade-Navarro MA.

PLoS One. 2012;7(2):e31826. Epub 2012 Feb 14.
12. Januar 2012 (Geramy)
Immunohistochemistry in the classification of systemic forms of amyloidosis: a systematic investigation of 117 patients.

2012 Jan 12;119(2):488-93. Epub 2011 Nov 21

Schönland S, Hegenbart U, Bochtler T, Mangatter A, Hansberg O, Ho AD, Lohse P, Röcken C.
1. Januar 2012 (Geramy)
Green Tea Halts Progression of Cardiac Transthyretin Amyloidosis – an Observational Report.

Clin Res Cardiol 2012;101(10):805-13.

Kristen AV, Lehrke S, Buss S, Mereles D, Steen H, Ehlermann P, Hardt S, Giannitsis E, Schreiner R, Haberkorn U, Schnabel PA, Linke RP, Röcken C, Wanker EE, Dengler TJ, Klaus Altland K, Katus HA.
1. Januar 2012 (Geramy)
The investigation of binary PPIs with the classical yeast two-hybrid (Y2H) approach, Y2H variants, and other in vivo methods for PPI mapping.

Methods Mol Biol. 2012;812:v-vi. PubMed PMID: 22319789.

Suter B, Wanker EE.
1. Januar 2012 (Geramy)
Deuterated peptides and proteins: structure and dynamics studies by MAS solid-state NMR.

Methods Mol Biol. 2012;831:279-301. doi: 10.1007/978-1-61779-480-3_16.

Reif B.
1. Dezember 2011 (Geramy)
The role of fluorescence in situ hybridization and gene expression profiling in myeloma risk stratification.

Srp Arh Celok Lek. 2011 Dec;139 Suppl 2:84-9. PubMed PMID: 22352188.

Hose D, Seckinger A, Jauch A, Rème T, Moreaux J, Bertsch U, Neben K, Klein B, Goldschmidt H.

Weiterführende Informationen

Artikel zum Verbund GERAMY in: Verbundforschung für seltene Erkrankungen - Gemeinsam zu Diagnose und Therapie, Februar 2014

Download (PDF)

Conference of the German Network on Systemic AL Amyloidosis (20-21 November, 2015)

Mehr...

Weitere Informationen

GERAMY - Netzwek für Leichtketten (AL-) Amyloidose

1. Januar 2019 (Geramy) GERAMY publication list

4. April 2013 (Geramy) Experimental determination of microsecond reorientation correlation times in protein solutions.

1. April 2013 (Geramy) A Y2H-seq approach defines the human protein methyltransferase interactome.

1. März 2013 (Geramy) ATTR amyloid in the carpal tunnel ligament is frequently of wildtype transthyretin origin.

1. März 2013 (Geramy) Dynamic circadian protein-protein interaction networks predict temporal organization of cellular functions.

18. Februar 2013 (Geramy) Epigallocatechin-3-gallate: a useful, effective and safe clinical approach for targeted prevention and individualised treatment of neurological diseases?

1. Februar 2013 (Geramy) Development and application of a DNA microarray-based yeast two-hybrid system.

1. Februar 2013 (Geramy) Protein-RNA interfaces probed by 1H-detected MAS solid-state NMR spectroscopy.

1. Januar 2013 (Geramy) Translation of HTT mRNA with expanded CAG repeats is regulated by the MID1-PP2A protein complex.

1. Januar 2013 (Geramy) A functional deficiency of TERA/VCP/p97 contributes to impaired DNA repair in multiple polyglutamine diseases.

22. Oktober 2012 (Geramy) New Criteria for Response to Treatment in Immunoglobulin Light Chain Amyloidosis Based on Free Light Chain Measurement and Cardiac Biomarkers: Impact on Survival Outcomes.

1. Oktober 2012 (Geramy) Optimal degree of protonation.

18. September 2012 (Geramy) Longitudinal left ventricular function for prediction of survival in systemic light-chain amyloidosis: incremental value compared with clinical and biochemical markers.

1. September 2012 (Geramy) In support of the BMRB.

24. August 2012 (Geramy) Structural properties of EGCG-induced, nontoxic Alzheimer’s disease Aβ oligomers.

1. August 2012 (Geramy) Amyloidose in Pneuomologie.

1. August 2012 (Geramy) Identification of human proteins that misfolding and proteotoxicity of pathogenic ataxin-1.

18. Mai 2012 (Geramy) Substrate transport activation is mediated through second periplasmic loop of transmembrane protein MalF in maltose transport complex of Escherichia coli.

1. Mai 2012 (Geramy) Amyloidose des Herzens.

1. Mai 2012 (Geramy) Evolution and function of CAG/polyglutamine repeats in protein-protein interaction networks.

10. April 2012 (Geramy) Phosphorylation-regulated axonal dependent transport of syntaxin 1 is mediated by a Kinesin-1 adapter.

1. März 2012 (Geramy) Ultra-high resolution in MAS solid-state NMR of perdeuterated proteins: implications for structure and dynamics.

14. Februar 2012 (Geramy) HIPPIE: Integrating protein interaction networks with experiment based quality scores.

12. Januar 2012 (Geramy) Immunohistochemistry in the classification of systemic forms of amyloidosis: a systematic investigation of 117 patients.

1. Januar 2012 (Geramy) Green Tea Halts Progression of Cardiac Transthyretin Amyloidosis – an Observational Report.

1. Januar 2012 (Geramy) The investigation of binary PPIs with the classical yeast two-hybrid (Y2H) approach, Y2H variants, and other in vivo methods for PPI mapping.

1. Januar 2012 (Geramy) Deuterated peptides and proteins: structure and dynamics studies by MAS solid-state NMR.

1. Dezember 2011 (Geramy) The role of fluorescence in situ hybridization and gene expression profiling in myeloma risk stratification.

Weiterführende Informationen

Conference of the German Network on Systemic AL Amyloidosis (20-21 November, 2015)

1. Januar 2019 (Geramy)
GERAMY publication list

4. April 2013 (Geramy)
Experimental determination of microsecond reorientation correlation times in protein solutions.

1. April 2013 (Geramy)
A Y2H-seq approach defines the human protein methyltransferase interactome.

1. März 2013 (Geramy)
ATTR amyloid in the carpal tunnel ligament is frequently of wildtype transthyretin origin.

1. März 2013 (Geramy)
Dynamic circadian protein-protein interaction networks predict temporal organization of cellular functions.

18. Februar 2013 (Geramy)
Epigallocatechin-3-gallate: a useful, effective and safe clinical approach for targeted prevention and individualised treatment of neurological diseases?

1. Februar 2013 (Geramy)
Development and application of a DNA microarray-based yeast two-hybrid system.

1. Februar 2013 (Geramy)
Protein-RNA interfaces probed by 1H-detected MAS solid-state NMR spectroscopy.

1. Januar 2013 (Geramy)
Translation of HTT mRNA with expanded CAG repeats is regulated by the MID1-PP2A protein complex.

1. Januar 2013 (Geramy)
A functional deficiency of TERA/VCP/p97 contributes to impaired DNA repair in multiple polyglutamine diseases.

22. Oktober 2012 (Geramy)
New Criteria for Response to Treatment in Immunoglobulin Light Chain Amyloidosis Based on Free Light Chain Measurement and Cardiac Biomarkers: Impact on Survival Outcomes.

1. Oktober 2012 (Geramy)
Optimal degree of protonation.

18. September 2012 (Geramy)
Longitudinal left ventricular function for prediction of survival in systemic light-chain amyloidosis: incremental value compared with clinical and biochemical markers.

1. September 2012 (Geramy)
In support of the BMRB.

24. August 2012 (Geramy)
Structural properties of EGCG-induced, nontoxic Alzheimer’s disease Aβ oligomers.

1. August 2012 (Geramy)
Amyloidose in Pneuomologie.

1. August 2012 (Geramy)
Identification of human proteins that misfolding and proteotoxicity of pathogenic ataxin-1.

18. Mai 2012 (Geramy)
Substrate transport activation is mediated through second periplasmic loop of transmembrane protein MalF in maltose transport complex of Escherichia coli.

1. Mai 2012 (Geramy)
Amyloidose des Herzens.

1. Mai 2012 (Geramy)
Evolution and function of CAG/polyglutamine repeats in protein-protein interaction networks.

10. April 2012 (Geramy)
Phosphorylation-regulated axonal dependent transport of syntaxin 1 is mediated by a Kinesin-1 adapter.

1. März 2012 (Geramy)
Ultra-high resolution in MAS solid-state NMR of perdeuterated proteins: implications for structure and dynamics.

14. Februar 2012 (Geramy)
HIPPIE: Integrating protein interaction networks with experiment based quality scores.

12. Januar 2012 (Geramy)
Immunohistochemistry in the classification of systemic forms of amyloidosis: a systematic investigation of 117 patients.

1. Januar 2012 (Geramy)
Green Tea Halts Progression of Cardiac Transthyretin Amyloidosis – an Observational Report.

1. Januar 2012 (Geramy)
The investigation of binary PPIs with the classical yeast two-hybrid (Y2H) approach, Y2H variants, and other in vivo methods for PPI mapping.

1. Januar 2012 (Geramy)
Deuterated peptides and proteins: structure and dynamics studies by MAS solid-state NMR.

1. Dezember 2011 (Geramy)
The role of fluorescence in situ hybridization and gene expression profiling in myeloma risk stratification.