Institute of Genetic Medicine

Staff Profile

Professor Volker Straub

Harold Macmillan Professor of Medicine

Background

Volker Straub is The Harold Macmillan Professor of Medicine and Professor of Neuromuscular Genetics at the Institute of Genetic Medicine at Newcastle University, United Kingdom. He is the director of the university’s John Walton Muscular Dystrophy Research Centre and holds honorary clinical appointments with the Newcastle upon Tyne Hospitals NHS Foundation Trust and the North Tees and Hartlepool NHS Foundation Trust.


Volker was trained as a pediatric neurologist at the University of Düsseldorf and the University of Essen in Germany. He wrote his PhD thesis on Duchenne muscular dystrophy (DMD) and worked as a postdoctoral research fellow in Dr Kevin Campbell’s laboratory at the Howard Hughes Medical Institute at the University of Iowa in Iowa City, Iowa, USA, on limb girdle muscular dystrophies (LGMD).

 

Volker has a long-standing interest in the pathogenesis of genetic muscle diseases, with research using zebrafish and mouse models. His current research also involves the application of magnetic resonance imaging, next generation sequencing and other –omics technologies for the characterization of primary neuromuscular disorders.

 

One of Volker’s main interests in muscle diseases is around translational research. He was the co-founder of the EU FP6 funded network of excellence for genetic neuromuscular diseases, TREAT-NMD (www.treat-nmd.eu), which he coordinated together with Kate Bushby. Volker is a long standing core member of the TREAT-NMD TACT committee (http://www.treat-nmd.eu/resources/tact/introduction/), which offers guidance on the translation and development path of therapeutics programs in rare neuromuscular diseases.

 

Volker is the CI/ PI for a number of natural history and interventional trials in DMD, LGMD, Pompe disease, spinal muscular atrophy and other NMDs. Volker also partners with the Broad Institute and MIT to lead the MYO-SEQ study, a pan European next generation sequencing programme targeted at patients with limb girdle weakness of unknown origin (https://myo-seq.org). Volker recently chaired the European MYO-MRI COST Action (BM1304) to develop applications of MR imaging and spectroscopy techniques in neuromuscular disease. He is part of the steering committee of the MRC Centre for neuromuscular diseases and an executive board member of the World Muscle Society. He is an author on >300 peer-reviewed publications.

 

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Research

Research Interests

Neuromuscular research

The majority of neuromuscular diseases are rare genetic conditions. They either affect the anterior horn cell, the peripheral nerve, the neuromuscular junction or the muscle cell itself. The neuromuscular research group at the Institute of Human Genetics has both a clinical and a research interest in neuromuscular diseases. In our specialized clinics we follow more than a thousand local patients with a broad spectrum of neuromuscular conditions most of which show progressive muscle weakness and wasting. Even for experts in the field it is becoming increasingly difficult to distinguish and accurately diagnose all forms of neuromuscular diseases on clinical grounds alone, as there is currently a still growing number of about 200 different disease entities. This urges a collaborative effort among clinicians and scientists who work on diagnosis, pathogenesis, and on therapeutic approaches in neuromuscular diseases.

A main interest of the neuromuscular team is around translational research. Together with Professor Bushby (see separate entry) Professor Straub is coordinating a network of excellence for translational research in rare inherited neuromuscular diseases funded by the European Commission. The ultimate goal of the network, called TREAT-NMD (www.treat-nmd.eu) is to accelerate the development of curative treatments for patients with neuromuscular diseases. To reach this goal the network is addressing the fragmentation currently hindering translational research for cutting edge therapies in these diseases. Preparing the patient population for trials involves the development of integrated databases and biobanks, standardised protocols for diagnosis, standards of care and validated outcome measures. The network is supported by a programme of education and outreach.

Within the neuromuscular research group Professor Straub has a long-standing interest in the pathogenesis of muscular dystrophies. Mechanisms of muscle fibre damage and repair are studied in zebrafish and mouse models for the diseases. The application of contrast enhanced MRI and quantitative MRI gives an insight into the dynamic processes underlying muscle fibre de- and regeneration. Professor Straub is an IHG executive board member, an executive board member of the World Muscle Society and the R&D Director of the North Tees and Hartlepool NHS Foundation Trust.

Our work is supported by the EU, the Muscular Dystrophy Campaign, the Association Francaise contre les Myopathies, the British Heart Foundation, the German Federal Ministry of Education and Research, the Department of Health and the MRC.

Co-workers
Elizabeth Greally BSc
Muscular Dystrophy Campaign Research Technician
Alison Blain BSc PhD
Research Associate

Publications