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Islet β-cell area and hormone expression are unaltered in Huntington’s disease

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Abstract

Neurodegenerative disorders are often associated with metabolic alterations. This has received little attention, but might be clinically important because it can contribute to symptoms and influence the course of the disease. Patients with Huntington’s disease (HD) exhibit increased incidence of diabetes mellitus (DM). This is replicated in mouse models of HD, e.g., the R6/2 mouse, in which DM is primarily caused by a deficiency of β-cells with impaired insulin secretion. Pancreatic tissue from HD patients has previously not been studied and, thus, the pathogenesis of DM in HD is unclear. To address this issue, we examined pancreatic tissue sections from HD patients at different disease stages. We found that the pattern of insulin immunostaining, levels of insulin transcripts and islet β-cell area were similar in HD patients and controls. Further, there was no sign of amyloid deposition in islets from HD patients. Thus, our data show that pancreatic islets in HD patients appear histologically normal. Functional studies of HD patients with respect to insulin secretion and islet function are required to elucidate the pathogenesis of DM in HD. This may lead to a better understanding of HD and provide novel therapeutic targets for symptomatic treatment in HD.

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Acknowledgments

The authors wish to thank Doris Persson and Britt-Marie Nilsson for expert technical assistance. This research was supported by the Swedish Research Council (M2006-6238, 14196, 4499 and 20403), Novo Nordisk, Crafoord, Tore Nilson, Åke Wiberg, Albert Påhlsson, Magnus Bergwall, Fredrik and Ingrid Thuring, Swedish Diabetes and The Swedish Medical Association Foundations, The Swedish Royal Physiographic Society, and the Medical Faculty in Lund.

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Authors and Affiliations

  1. Unit of Molecular Metabolism, Department of Experimental Medical Science, Lund University, CRC 91-11, 205 02, Malmö, Sweden

    Karl Bacos & Hindrik Mulder

  2. Neuronal Survival Unit, Department of Experimental Medical Science, Lund University, BMC A10, 221 84, Lund, Sweden

    Maria Björkqvist & Patrik Brundin

  3. Translational Neuroendocrine Research Unit, Department of Experimental Medical Science, BMC D11, 221 84, Lund, Sweden

    Åsa Petersén

  4. Department of Pathology, Univeristy Hospital, 221 85, Lund, Sweden

    Lena Luts

  5. Department of Neurology, Leiden University Medical Center, P.O.Box 9600, 2300 RC, Leiden, The Netherlands

    Marion L. C. Maat-Schieman & Raymund A. C. Roos

  6. Unit of Neuroendocrine Cell Biology, Department of Experimental Medical Science, Lund University, BMC B11, 221 84, Lund, Sweden

    Frank Sundler & Nils Wierup

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  1. Karl Bacos
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  2. Maria Björkqvist
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Correspondence to Karl Bacos.

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Bacos, K., Björkqvist, M., Petersén, Å. et al. Islet β-cell area and hormone expression are unaltered in Huntington’s disease. Histochem Cell Biol 129, 623–629 (2008). https://doi.org/10.1007/s00418-008-0393-z

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