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Prof. Dr. med. Georg Wolfgang Josef Auburger

Zentrum für Neurologie und Neurochirurgie, ZNN
Klinik für Neurologie
Experimentelle Neurologie
Haus 89, Heinrich Hoffmann Strasse 7
D – 60528 Frankfurt am Main

Tel +49 (0)69 6301 7428
Fax +49 (0)69 6301 7142



Scientific Focus

Brain degeneration is a frequent problem at old age, but its prevention is currently impossible.
Microscopic studies have revealed quite specific patterns and hallmarks of neurodegeneration.
Hypothesis-driven biochemical and molecular research are making only slow progress to elucidate underlying causes and mechanisms.

Many of these diseases may be inherited in a monogenic manner in exceptional families,
allowing hypothesis-free genetic approaches:
– We define the chromosomal localization and identify the mutations of the disease gene;
– We study the subcellular localization and the protein and the molecular interactions of the mutant disease protein;
– We generate and characterize mouse mutants, which define the function of the disease protein
or which are engineered to model and study initial stages of the disease process.
– We survey expression changes across all genes within tissues from patients or mutant animals.
– We define biomarkers to quantify disease severity and progression,
in order to validate the benefit of preventive therapies.

Clinically, the group is focused on
– the SCA2 variant of Cerebellar Ataxia,
– the PARK6 / PARK1 / PARK4 variants of Parkinson’s Disease.

Functionally, the team has accumulated evidence that mitochondrial quality control after cell stress is crucial for neurodegeneration, so we are exploring the bioenergetic and inflammatory consequences.


Our group uses a wide spectrum of techniques, including

– the analysis of tissues and (transfected) cultured cells with mutation screens, molecular biology, global transcriptome and proteome studies, immunohistochemistry and fluorescent microscopy, biochemistry of protein interactions;

– the analysis of patients / mice with neurological motor or behaviour tests, cooperating with experts for electro-physiological tests or for brain imaging analysis.

Selected Publications

On SCA2 / polyglutamine disorders:

Orozco-Diaz G, Nodarse-Fleites A, Cordoves-Sagaz R, Auburger G (1990) Autosomal dominant cerebellar ataxia: Clinical analysis of 263 patients from a homogeneous population in Holguin, Cuba. Neurology, 40:1369-1375

Gispert S, Twells R, Orozco G, Brice A, Weber J, Heredero L, Scheufler K, Riley B, Allotey R, Nothers C, Hillermann R, Lunkes A, Khati C, Stevanin G, Hernandez A, Magariño C, Klockgether T, Durr A, Chneiweiss H, Enczmann J, Farrall M, Beckmann J, Mullan M, Wernet P, Agid Y, Freund H-J, Williamson R, Auburger G§, Chamberlain S§ (1993) Chromosomal assignment of the second (Cuban) locus for autosomal dominant cerebellar ataxia (SCA2) to human chromosome 12q23-24.1. Nat Genet, 4:295-299.

Pulst S, Nechiporuk A, Nechiporuk T, Gispert S, Chen X, Lopes-Cendes I, Pearlman S, Starkman S, Orozco G, Lunkes A, de Jong P, Rouleau G, Auburger G, Korenberg JR, Figueroa C, Sahba S (1996) Moderate expansion of a normally biallelic trinucleotide repeat in Spinocerebellar ataxia 2. Nat Genet, 14:269-276.

Estrada R, Galarraga J, Orozco G, Nodarse A, Auburger G (1999) Spinocerebellar ataxia 2 (SCA2): Morphometric analyses in 11 autopsies. Acta Neuropathol, 97:306-310.

Nussbaum R, Auburger G (2000) Neurodegeneration in the polyglutamine diseases: Act1, Scene 1. Nat Neurosci, 3:103-104.

Velázquez-Pérez L, Seifried C, Santos-Falcón N, Abele M, Ziemann U, Almaguer LE, Martínez-Góngora E, Sánchez-Cruz G, Canales N, Pérez-González R, Velázquez-Manresa M, Viebahn B, Stuckrad-Barre S, Fetter M, Klockgether T, Auburger G (2004) Saccade velocity is controlled by polyglutamine size in Spinocerebellar ataxia type 2. Ann Neurol, 56 (3):444-7.

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