Screening exons 16 and 17 of the amyloid precursor protein gene in sporadic early-onset Alzheimer’s disease

Early-onset Alzheimer’s disease (EOAD) can be familial (FAD) or sporadic (sEOAD); both have a disease onset ≤ 65 years of age. 451 sEOAD samples were screened for known causative mutations in exon 16 and 17 of the Amyloid Precursor Protein gene (APP). Four samples were shown to be heterozygous for o...

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Main Authors: Barber, Imelda S., García-Cárdenas, Jennyfer M., Sakdapanichkul, Chidchanok, Deacon, Christopher, Zapata Erazo, Gabriela, Guerreiro, Rita, Bras, Jose, Hernandez, Dena, Singleton, Andrew, Guetta-Baranes, Tamar, Braae, Anne, Clement, Naomi, Patel, Tulsi, Brookes, Keeley, Medway, Christopher, Chappell, Sally, Mann, David M.
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Published: Elsevier 2016
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Online Access:https://eprints.nottingham.ac.uk/31627/
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author Barber, Imelda S.
García-Cárdenas, Jennyfer M.
Sakdapanichkul, Chidchanok
Deacon, Christopher
Zapata Erazo, Gabriela
Guerreiro, Rita
Bras, Jose
Hernandez, Dena
Singleton, Andrew
Guetta-Baranes, Tamar
Braae, Anne
Clement, Naomi
Patel, Tulsi
Brookes, Keeley
Medway, Christopher
Chappell, Sally
Mann, David M.
author_facet Barber, Imelda S.
García-Cárdenas, Jennyfer M.
Sakdapanichkul, Chidchanok
Deacon, Christopher
Zapata Erazo, Gabriela
Guerreiro, Rita
Bras, Jose
Hernandez, Dena
Singleton, Andrew
Guetta-Baranes, Tamar
Braae, Anne
Clement, Naomi
Patel, Tulsi
Brookes, Keeley
Medway, Christopher
Chappell, Sally
Mann, David M.
author_sort Barber, Imelda S.
building Nottingham Research Data Repository
collection Online Access
description Early-onset Alzheimer’s disease (EOAD) can be familial (FAD) or sporadic (sEOAD); both have a disease onset ≤ 65 years of age. 451 sEOAD samples were screened for known causative mutations in exon 16 and 17 of the Amyloid Precursor Protein gene (APP). Four samples were shown to be heterozygous for one of three known causative mutations: p.A713T, p.V717I and p.V717G, this highlights the importance of screening EOAD patients for causative mutations. Additionally, we document an intronic 6 base pair (bp) deletion located 83 bp downstream of exon 17 (rs367709245, IVS17 83-88delAAGTAT), which has a nonsignificantly increased minor allele frequency in our sEOAD cohort (0.006) compared to LOAD (0.002) and controls (0.002). To assess the effect of the 6 bp deletion on splicing, COS-7 and BE(2)-C cells were transfected with a minigene vector encompassing exon 17. There was no change in splicing of exon 17 from constructs containing either wild type or deletion inserts. Sequencing of cDNA generated from cerebellum and temporal cortex of a patient harbouring the deletion found no evidence of transcripts with exon 17 removed.
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spelling nottingham-316272020-05-04T17:34:01Z https://eprints.nottingham.ac.uk/31627/ Screening exons 16 and 17 of the amyloid precursor protein gene in sporadic early-onset Alzheimer’s disease Barber, Imelda S. García-Cárdenas, Jennyfer M. Sakdapanichkul, Chidchanok Deacon, Christopher Zapata Erazo, Gabriela Guerreiro, Rita Bras, Jose Hernandez, Dena Singleton, Andrew Guetta-Baranes, Tamar Braae, Anne Clement, Naomi Patel, Tulsi Brookes, Keeley Medway, Christopher Chappell, Sally Mann, David M. Early-onset Alzheimer’s disease (EOAD) can be familial (FAD) or sporadic (sEOAD); both have a disease onset ≤ 65 years of age. 451 sEOAD samples were screened for known causative mutations in exon 16 and 17 of the Amyloid Precursor Protein gene (APP). Four samples were shown to be heterozygous for one of three known causative mutations: p.A713T, p.V717I and p.V717G, this highlights the importance of screening EOAD patients for causative mutations. Additionally, we document an intronic 6 base pair (bp) deletion located 83 bp downstream of exon 17 (rs367709245, IVS17 83-88delAAGTAT), which has a nonsignificantly increased minor allele frequency in our sEOAD cohort (0.006) compared to LOAD (0.002) and controls (0.002). To assess the effect of the 6 bp deletion on splicing, COS-7 and BE(2)-C cells were transfected with a minigene vector encompassing exon 17. There was no change in splicing of exon 17 from constructs containing either wild type or deletion inserts. Sequencing of cDNA generated from cerebellum and temporal cortex of a patient harbouring the deletion found no evidence of transcripts with exon 17 removed. Elsevier 2016-03-01 Article PeerReviewed Barber, Imelda S., García-Cárdenas, Jennyfer M., Sakdapanichkul, Chidchanok, Deacon, Christopher, Zapata Erazo, Gabriela, Guerreiro, Rita, Bras, Jose, Hernandez, Dena, Singleton, Andrew, Guetta-Baranes, Tamar, Braae, Anne, Clement, Naomi, Patel, Tulsi, Brookes, Keeley, Medway, Christopher, Chappell, Sally and Mann, David M. (2016) Screening exons 16 and 17 of the amyloid precursor protein gene in sporadic early-onset Alzheimer’s disease. Neurobiology of Aging, 39 . 220.e1-220.e7. ISSN 1558-1497 (In Press) Alzheimer's disease early-onset sporadic screening APP rs367709245 http://www.sciencedirect.com/science/article/pii/S0197458015006156 doi:10.1016/j.neurobiolaging.2015.12.011 doi:10.1016/j.neurobiolaging.2015.12.011
spellingShingle Alzheimer's disease
early-onset
sporadic
screening
APP
rs367709245
Barber, Imelda S.
García-Cárdenas, Jennyfer M.
Sakdapanichkul, Chidchanok
Deacon, Christopher
Zapata Erazo, Gabriela
Guerreiro, Rita
Bras, Jose
Hernandez, Dena
Singleton, Andrew
Guetta-Baranes, Tamar
Braae, Anne
Clement, Naomi
Patel, Tulsi
Brookes, Keeley
Medway, Christopher
Chappell, Sally
Mann, David M.
Screening exons 16 and 17 of the amyloid precursor protein gene in sporadic early-onset Alzheimer’s disease
title Screening exons 16 and 17 of the amyloid precursor protein gene in sporadic early-onset Alzheimer’s disease
title_full Screening exons 16 and 17 of the amyloid precursor protein gene in sporadic early-onset Alzheimer’s disease
title_fullStr Screening exons 16 and 17 of the amyloid precursor protein gene in sporadic early-onset Alzheimer’s disease
title_full_unstemmed Screening exons 16 and 17 of the amyloid precursor protein gene in sporadic early-onset Alzheimer’s disease
title_short Screening exons 16 and 17 of the amyloid precursor protein gene in sporadic early-onset Alzheimer’s disease
title_sort screening exons 16 and 17 of the amyloid precursor protein gene in sporadic early-onset alzheimer’s disease
topic Alzheimer's disease
early-onset
sporadic
screening
APP
rs367709245
url https://eprints.nottingham.ac.uk/31627/
https://eprints.nottingham.ac.uk/31627/
https://eprints.nottingham.ac.uk/31627/