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Title:
Neurodegenerative disorders associated with diabetes mellitus | Journal of Molecular Medicine
Description:
More than 20 syndromes among the significant and increasing number of degenerative diseases of neuronal tissues are known to be associated with diabetes mellitus, increased insulin resistance and obesity, disturbed insulin sensitivity, and excessive or impaired insulin secretion. This review briefly presents such syndromes, including Alzheimer disease, ataxia-telangiectasia, Down syndrome/trisomy 21, Friedreich ataxia, Huntington disease, several disorders of mitochondria, myotonic dystrophy, Parkinson disease, Prader-Willi syndrome, Werner syndrome, Wolfram syndrome, mitochondrial disorders affecting oxidative phosphorylation, and vitamin B1 deficiency/inherited thiamine-responsive megaloblastic anemia syndrome as well as their respective relationship to malignancies, cancer, and aging and the nature of their inheritance (including triplet repeat expansions), genetic loci, and corresponding functional biochemistry. Discussed in further detail are disturbances of glucose metabolism including impaired glucose tolerance and both insulin-dependent and non-insulin-dependent diabetes caused by neurodegeneration in humans and mice, sometimes accompanied by degeneration of pancreatic beta-cells. Concordant mouse models obtained by targeted disruption (knock-out), knock-in, or transgenic overexpression of the respective transgene are also described. Preliminary conclusions suggest that many of the diabetogenic neurodegenerative disorders are related to alterations in oxidative phosphorylation (OXPHOS) and mitochondrial nutrient metabolism, which coincide with aberrant protein precipitation in the majority of affected individuals.
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Keywords {🔍}

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Topics {✒️}

thiamine-responsive megaloblastic anaemia thiamine-responsive megaloblastic anemia month download article/chapter globular c-terminal domain thiamine-dependent erythroid hypoplasia verbindung mit sklerodermie ataxia-telangiectasia protein-deficient mice vieweg & sohn klinik der leberkrankheiten n-acetyl aspartate levels bardet-biedl syndrome sibs thiol-dependent manner resulting phosphofructo-1-kinase/muscle subtype altered beta-cell characteristics muscle-specific chloride channel alpha-synuclein gene identified hôtel-dieu de paris des troubles cérébelleux laurence-moon-biedl syndrome insulin-degrading enzyme regulates islet amyloid polypeptide tissue-specific knockout mice machado-joseph disease mutations von kleist-retzow gaa triplet-repeat expansion ragged-red fiber disease metabolic profile increased body-mass index full article pdf early-onset diabetes mellitus population-based cohort study van den broek amyloid beta-protein über katarakt islet amyloid formation complications neurologiques dans van de pol increased beta-cell apoptosis insulin-dependent diabetes mellitus insulin-dependent diabetes caused mckusick-nathans institute late-onset parkinson disease mckusick va privacy choices/manage cookies maternally transmitted diabetes bardet-biedl syndrome progressive lenticular degeneration abnormal glucose tolerance progressive motor deficits heteroplasmic mtdna mutation

Questions {❓}

Ashcroft F, Rorsman P (2004) Type 2 diabetes mellitus: not quite exciting enough?
Bertram L, Tanzi RE (2004) Alzheimer’s disease: one disorder, too many genes?
Clark A, Jones LC, de Koning E, Hansen BC, Matthews DR (2001) Decreased insulin secretion in type 2 diabetes: a problem of cellular mass or function?
Feigenbaum AB, Bergeron C, Richardson R, Wherret J, Robinson B, Weksberg R (1994) Premature atherosclerosis with photomyoclonic epilepsy, deafness, diabetes mellitus, nephropathy, and neurodegenerative disorder in two brothers: a new syndrome?
Martin GM (1997) The Werner mutation: does it lead to a “public” or “private” mechanism of aging?
Reaven GM, Bernstein R, Davis B, Olefsky JM (1976) Nonketotic diabetes mellitus: insulin deficiency or insulin resistance?
Viana MB, Carvalho RI (1978) Thiamine-responsive megaloblastic anemia, sensorineural deafness, and diabetes mellitus: a new syndrome?

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