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@ARTICLE{Rasheed:274007,
author = {Rasheed, Urwah and Khalid, Minahil and Noor, Aneeqa and
Saeed, Umar and Uppal, Rizwan and Zafar, Saima},
title = {{G}enetic assessment of apolipoprotein {E} polymorphism and
{PRNP} genotypes in rapidly progressive dementias in
{P}akistan.},
journal = {Prion},
volume = {18},
number = {1},
issn = {1933-6896},
address = {London [u.a.]},
publisher = {Taylor $\&$ Francis},
reportid = {DZNE-2024-01435},
pages = {1 - 7},
year = {2024},
abstract = {Rapidly progressive dementias (RPDs) are a type of fatal
dementias that cause rapid progression of neuronal
dysfunction. This study aimed to assess the prevalence of
APOE genotypes (ε2, ε3, ε4) and PRNP mutations (E200K,
M129V) in the general population of Pakistan because of
their association with RPDs, including Rapidly Progressive
Alzheimer's Disease (rpAD) and Creutzfeldt-Jakob Disease
(CJD). Blood samples (n = 100) were collected from healthy
Pakistani population and the stated mutations were assessed
using polymerase chain reaction. In the analysis of the APOE
genotype, ε3/ε3 genotype was the most common $(95\%),$
followed by ε3/ε4 $(5\%)$ and ε2 allele was completely
absent. A low frequency of ε4 allele and the absence of a
protective ε2 allele is associated with an increased risk
of rpAD. In the case of PRNP mutations, the most common
genotype was M129-Ε200 $(71\%)$ and V129-Ε200 $(29\%).$
E200K mutation was completely absent from the given
population. It is noteworthy that the MM homozygous genotype
was present in 71 samples, VV genotype was present in 29.
Homozygosity on codon 129, as observed in most of our
samples, has been associated with more efficient production
of PrPSc and disease pathology. This study provides
preliminary data indicating that rpAD and CJD pose a
significant threat to the Pakistani population.},
keywords = {Humans / Pakistan / Prion Proteins: genetics / Genotype /
Apolipoproteins E: genetics / Female / Male / Polymorphism,
Genetic: genetics / Creutzfeldt-Jakob Syndrome: genetics /
Dementia: genetics / Mutation: genetics / Alleles / Gene
Frequency: genetics / Middle Aged / Alzheimer Disease:
genetics / Aged / Alzheimer’s disease (Other) /
Creutzfeldt-Jakob disease (Other) / incidence (Other) /
rapidly progressive Alzheimer’s disease (Other) / rapidly
progressive dementia (Other) / Prion Proteins (NLM
Chemicals) / PRNP protein, human (NLM Chemicals) /
Apolipoproteins E (NLM Chemicals)},
cin = {AG Zerr},
ddc = {570},
cid = {I:(DE-2719)1440011-1},
pnm = {353 - Clinical and Health Care Research (POF4-353)},
pid = {G:(DE-HGF)POF4-353},
typ = {PUB:(DE-HGF)16},
pubmed = {pmid:39654135},
doi = {10.1080/19336896.2024.2439598},
url = {https://pub.dzne.de/record/274007},
}