Glucose transportation in the brain and its impairment in Huntington disease: one more shade of the energetic metabolism failure? View Full Text


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Article Info

DATE

2017-04-10

AUTHORS

Veronica Morea, Eris Bidollari, Gianni Colotti, Annarita Fiorillo, Jessica Rosati, Lidia De Filippis, Ferdinando Squitieri, Andrea Ilari

ABSTRACT

Huntington’s disease (HD) or Huntington’s chorea is the most common inherited, dominantly transmitted, neurodegenerative disorder. It is caused by increased CAG repeats number in the gene coding for huntingtin (Htt) and characterized by motor, behaviour and psychiatric symptoms, ultimately leading to death. HD patients also exhibit alterations in glucose and energetic metabolism, which result in pronounced weight loss despite sustained calorie intake. Glucose metabolism decreases in the striatum of all the subjects with mutated Htt, but affects symptom presentation only when it drops below a specific threshold. Recent evidence points at defects in glucose uptake by the brain, and especially by neurons, as a relevant component of central glucose hypometabolism in HD patients. Here we review the main features of glucose metabolism and transport in the brain in physiological conditions and how these processes are impaired in HD, and discuss the potential ability of strategies aimed at increasing intracellular energy levels to counteract neurological and motor degeneration in HD patients. More... »

PAGES

1147-1157

References to SciGraph publications

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  • Identifiers

    URI

    http://scigraph.springernature.com/pub.10.1007/s00726-017-2417-2

    DOI

    http://dx.doi.org/10.1007/s00726-017-2417-2

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    PUBMED

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    45 brain
    46 calorie intake
    47 central glucose hypometabolism
    48 chorea
    49 components
    50 conditions
    51 death
    52 defects
    53 degeneration
    54 disease
    55 disorders
    56 energetic metabolism
    57 energetic metabolism failure
    58 energy levels
    59 evidence points
    60 failure
    61 features
    62 genes
    63 glucose
    64 glucose hypometabolism
    65 glucose metabolism
    66 glucose transportation
    67 glucose uptake
    68 huntingtin
    69 hypometabolism
    70 impairment
    71 intake
    72 intracellular energy levels
    73 levels
    74 loss
    75 main features
    76 metabolism
    77 metabolism failure
    78 more shade
    79 motor
    80 motor degeneration
    81 neurodegenerative disorders
    82 neurons
    83 number
    84 patients
    85 physiological conditions
    86 point
    87 potential ability
    88 presentation
    89 process
    90 pronounced weight loss
    91 psychiatric symptoms
    92 recent evidence points
    93 relevant components
    94 repeat number
    95 shade
    96 specific threshold
    97 strategies
    98 striatum
    99 subjects
    100 sustained calorie intake
    101 symptom presentation
    102 symptoms
    103 threshold
    104 transport
    105 transportation
    106 uptake
    107 weight loss
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