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Transglutaminase activity is related to CAG repeat length in patients with Huntington’s disease

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Abstract

Huntington’s disease (HD) is a neurodegenerative disorder associated with CAG repeat expansion. We measured transglutaminase (TGase) activity in lymphocytes from 35 HD patients and from healthy individuals to ascertain whether it was altered in this condition. TGase activity was above maximum control levels in 25% of HD patients; it was correlated with the age of the patient and inversely correlated with the CAG repeat length. These results suggest that: (1) HD could be biochemically heterogeneous, and (2) the length of the CAG repeat expansion/TGase ratio could be important in the manifestation of HD.

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Authors and Affiliations

  1. Laboratorio di Biochimica e Biologia Molecolare, Stazione Zoologica “Anton Dohrn”, Villa Comunale, I-80121 Naples, Italy Tel.: +39-81-5833216; Fax: +39-81-7641355, , , , , , IT

    L. Cariello, Laura Zanetti & Roberto Di Lauro

  2. Centro di Endocrinologia ed Oncologia Sperimentale (C.E.O.S.) del C.N.R c/o Dipartimento di Biologia e Patologia Cellulare e Molecolare, Universita’ di Napoli Federico II, Via S. Pansini 5, I-80131 Naples, Italy, , , , , , IT

    T. de Cristofaro & S. Varrone

  3. Dipartimento di Scienze Neurologiche, Universita’ di Napoli Federico II, Via S. Pansini 5, I-80131 Naples, Italy, , , , , , IT

    Teresa Cuomo, Luigi Di Maio & Giuseppe Campanella

  4. Dipartimento di Matematica e Applicazioni, Universita’ di Napoli Federico II, Complesso Universitario Monte S.Angelo, Via Cintia, I-80126 Naples, Italy, , , , , , IT

    Silvana Rinaldi

  5. Dipartimento di S.A.V.A., Universita’ del Molise, I-86100 Campobasso, Italy, , , , , , IT

    Paolo Zanetti

Authors
  1. L. Cariello
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  2. T. de Cristofaro
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  3. Laura Zanetti
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  4. Teresa Cuomo
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  5. Luigi Di Maio
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  6. Giuseppe Campanella
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  7. Silvana Rinaldi
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  8. Paolo Zanetti
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  9. Roberto Di Lauro
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  10. S. Varrone
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Additional information

Received: 25 March 1996 / Revised: 23 June 1996

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Cariello, L., de Cristofaro, T., Zanetti, L. et al. Transglutaminase activity is related to CAG repeat length in patients with Huntington’s disease. Hum Genet 98, 633–635 (1996). https://doi.org/10.1007/s004390050273

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  • DOI: https://doi.org/10.1007/s004390050273

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