Dr Brian Gibberd (1931–2006): a pioneering clinician in Refsum's disease

Author:

Wierzbicki A.S.12,Lloyd M.D.3

Affiliation:

1. Refsum's Disease Clinic, Chelsea and Westminster Hospital, London SW10 9NH, U.K.

2. St Thomas' Hospital, London SE1 7EH, U.K.

3. Department of Pharmacy and Clinical Pharmacology, University of Bath, Claverton Down, Bath BA2 7AY, U.K.

Abstract

Branched-chain fatty acids are common components of the human diet (phytanic acid) or are produced endogenously (bile acids), and are also used as medicines (ibuprofen). Owing to their branched-chain structure, they are metabolized in peroxisomes. In the case of phytanic acid, the presence of a 3-methyl group prevents β-oxidation, and instead it undergoes one round of α-oxidation to allow further metabolism. Defects in this process give rise to neurological diseases and cancer. Dr Brian F. Gibberd was one of the first U.K. physicians to recognize the importance of these peroxisomal metabolic pathways in clinical medicine, and pioneered their study. This obituary recognizes his many achievements in neurology and especially in the treatment of peroxisomal disorders. The following four papers from this mini-symposium entitled ‘Advances in peroxisomal α-, β- and ω-oxidation’ describe work done in this area as part of a collaborative study in which Dr Gibberd played a key role. This work was presented as part of the Cardiovascular Bioscience focused topic at the Life Sciences 2007 conference, and this mini-symposium was dedicated to Dr Gibberd and his important contributions to this field.

Publisher

Portland Press Ltd.

Subject

Biochemistry

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