J Neural Transm Suppl. 1999;57:137-59.

Molecular misreading of genes in Down syndrome as a model for the Alzheimer type of neurodegeneration.

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Netherlands Institute for Brain Research, Amsterdam, The Netherlands. f.van.leeuwen@nih.knaw.nl

Abstract

The occurrence of +1 frameshifted proteins, such as amyloid precursor protein (APP+1) and ubiquitin-B (UBB+1) in Down syndrome (DS) has been linked to the onset of Alzheimer's disease (AD). In DS and AD patients, but also in elderly non-demented persons, these co-called +1 proteins accumulate in the neuropathological hallmarks (neurofibrillary tangles, dystrophic neurites of the neuritic plaques and neuropil threads) and may have deleterious effects on neuronal function. Frameshifts are caused by dinucleotide deletions in GAGAG motifs in messenger RNA and are now thought to be the result of unfaithful transcription of normal DNA by a novel process termed "molecular misreading". In the present review some of the critical events in molecular misreading are discussed, the emphasis being on DS.

PMID:: 10666673; [PubMed - indexed for MEDLINE]

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Review

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RNA, Messenger

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Review Molecular misreading: a new type of transcript mutation expressed during aging. [Neurobiol Aging. 2000]
Review Molecular misreading: a new type of transcript mutation expressed during aging.
van Leeuwen FW, Fischer DF, Kamel D, Sluijs JA, Sonnemans MA, Benne R, Swaab DF, Salehi A, Hol EM. Neurobiol Aging. 2000 Nov-Dec; 21(6):879-91.
Molecular misreading. A new type of transcript mutation in gerontology. [Ann N Y Acad Sci. 2000]
Molecular misreading. A new type of transcript mutation in gerontology.
van Leeuwen FW, Fischer DF, Benne R, Hol EM. Ann N Y Acad Sci. 2000 Jun; 908:267-81.
Molecular misreading: the frequency of dinucleotide deletions in neuronal mRNAs for beta-amyloid precursor protein and ubiquitin B. [Neurobiol Aging. 2005]
Molecular misreading: the frequency of dinucleotide deletions in neuronal mRNAs for beta-amyloid precursor protein and ubiquitin B.
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Frameshift mutants of beta amyloid precursor protein and ubiquitin-B in Alzheimer's and Down patients. [Science. 1998]
Frameshift mutants of beta amyloid precursor protein and ubiquitin-B in Alzheimer's and Down patients.
van Leeuwen FW, de Kleijn DP, van den Hurk HH, Neubauer A, Sonnemans MA, Sluijs JA, Köycü S, Ramdjielal RD, Salehi A, Martens GJ, et al. Science. 1998 Jan 9; 279(5348):242-7.
Review Molecular abnormalities of the brain in Down syndrome: relevance to Alzheimer's neurodegeneration. [J Neural Transm Suppl. 1999]
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de la Monte SM. J Neural Transm Suppl. 1999; 57:1-19.

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Cited by 1 PubMed Central article

Beta-amyloid precursor protein transgenic mice that harbor diffuse A beta deposits but do not form plaques show increased ischemic vulnerability: role of inflammation. [Proc Natl Acad Sci U S A. 2002]
Beta-amyloid precursor protein transgenic mice that harbor diffuse A beta deposits but do not form plaques show increased ischemic vulnerability: role of inflammation.
Koistinaho M, Kettunen MI, Goldsteins G, Keinänen R, Salminen A, Ort M, Bures J, Liu D, Kauppinen RA, Higgins LS, et al. Proc Natl Acad Sci U S A. 2002 Feb 5; 99(3):1610-5. Epub 2002 Jan 29.

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