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European Journal of Applied Sciences – Vol. 12, No. 1
Publication Date: February 25, 2024
DOI:10.14738/aivp.121.16177
Sanyour, A., & Alhazmi, S. (2024). Giant Platelets as Potential Biomarker for Autism Spectrum Disorder. European Journal of
Applied Sciences, Vol - 12(1). 255-267.
Services for Science and Education – United Kingdom
Giant Platelets as Potential Biomarker for Autism Spectrum
Disorder
Aya Sanyour
Department of Biological Sciences,
King Abdulaziz University, Jeddah, Saudi Arabia
Safiah Alhazmi
Department of Biological Sciences, King Abdulaziz University,
Jeddah, Saudi Arabia and Immunology Unit, King Fahad Medical
Research Centre, King Abdulaziz University, Jeddah, Saudi Arabia
ABSTRACT
Introduction: Platelet hyperserotonemia has been repeatedly observed in
individuals with autism spectrum disorder (ASD). Ras p21 protein activator 3
(RASA3) is essential in forming and differentiating blood cells, especially platelets.
Therefore, this study aims to evaluate the expression of RASA3 and examine the
platelet morphology in addition measuring the plateletcrit and platelet count in all
samples. Methods: RNA was extracted from 12 autistic children and four control
samples then qPCR was used to determine the expression of RASA3. Platelet
morphology was examined under light microscope. In addition, Complete blood
count (CBC) test with automated haematology analysers was done for all children
to determine the platelet count and the plateletcrit (PCT). Results: The qPCR result
showed differential expression patterns of RASA3 between autistic and normal
samples. The platelet morphology revealed large and giant platelets in all autistic
samples and the plateletcrit percentage was increased in 10 out of 12 ASD samples.
Conclusions: There is a correlation between dysregulated RASA3 and the large and
giant platelets in ASD cases which could explain the platelet hyperserotonemia in
this disorder and could be used as an early biomarker for ASD diagnosis or may be
used for early interventions.
Keywords: RASA3, ASD, DNA methylation, Plateletcrit, Hyperserotonemia,
INTRODUCTION
ASD is a persistent impairment in social communication and interaction, as well as restricted
and repetitive patterns of behavior, interests, or activities. Indeed, the prevalence of ASD has
increased worldwide, and it is considered one of the most common disorders in the Gulf
Cooperation Council (GCC) countries (Almandil et al., 2019). However, ASD is considered a
heritable neurodevelopmental disorder due to the large differences in concordance rates
between monozygotic (60–92%) and dizygotic twins (0–10%) where the risk for ASD in
siblings is much higher than that in the general population (Elagoz Yuksel et al., 2016).
On the other hand, the diagnosis of autism still relies on expert judgment, observation, and
assessment of behavior and cognition to detect a significant impairment in the core behavioral
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European Journal of Applied Sciences (EJAS) Vol. 12, Issue 1, February-2024
domains when there is no exact biomarker for ASD is identified (American Psychiatric
Association, 2013). Therefore, ASD is usually appeared in the developmental period, often
during the second year of life, and it is in varying degrees of limitations, but if developmental
delay is severe, it may be seen earlier than 12 months, or later than 24 months if symptoms are
simple (Schaefer and Mendelsohn, 2013). However, earlier detection, intervention,
compensation, and current support for individuals with ASD could lead to a major long-term
positive effect on the symptoms (American Psychiatric Association, 2013).
However, in the 1950s, platelets (PLTs) studies had been an intriguing aspect for studying
neurodevelopmental disorders since the discovery of these tiny fragments of cells as storage
reservoirs for circulating serotonin (Maclean and Schoenwaelder, 2019). Therefore, the
researchers' interest has increased in using the platelets as a model cell for studying
neuropathology and also as a biomarker for the diagnosis of complex neurological disorders
(Padmakumar et al., 2019, Goubau et al., 2014). Platelets or thrombocytes are small blood cells
that stop bleeding by forming clots. In humans, platelets have an average life span of 8-14 days
then eliminated in the spleen and liver, they are originally derived from megakaryocytes in the
bone marrow that originate numerous cellular extensions called proplatelets resulting in the
release of thousands of platelets at once (Maclean and Schoenwaelder, 2019, Sharathkumar and
Shapiro, 2008). In addition, platelets size is 1-2 μm, six to eight times smaller than red cells
under the microscope, or 1.5 to 3 μm in greatest diameter (Handtke and Thiele, 2020, Palmer
et al., 2015). Although the basic function of platelets is to be activated when a blood vessel is
damaged to form a clot, hemostasis and blood coagulation is not the exclusive function of
platelets, they also play many pivotal roles in disease pathophysiology (van der Meijden and
Heemskerk, 2019, Ghoshal and Bhattacharyya, 2014). For instance, A previous study reported
that when platelet contains high amounts of serotonin it leads to dysfunction of the
serotoninergic system and the development of several behavioral disorders (Ehrlich and
Humpel, 2012). In general, dysfunction, abnormal activation, and morphological alteration of
platelets have been implicated in many complex neurological disorders such as schizophrenia,
migraine, Parkinson's disease, Alzheimer's disease, and autism (Goubau et al., 2014).
In normal conditions, serotonin or 5-hydroxytryptamine (5-HT) modulates a wide range of
neuropsychological processes, and it has important roles in controlling behavior and sociality.
In addition, alterations of serotonin metabolism have been linked to several neurological
disorders such as schizophrenia, migraine, Alzheimer’s disease, and ASD (Bijl et al., 2015). For
example, increased platelet activity has been described during migraine attacks and is related
to releasing serotonin (Goubau et al., 2014). Furthermore, several studies found that platelet
serotonin concentrations were significantly elevated in patients with chronic schizophrenia
(Asor and Ben-Shachar, 2012). Indeed, increased the level of serotonin in platelet of patients
with autism (platelet hyperserotonemia) was repeatedly observed, it seems to be the most
biochemical aberration that persistently appears in patients with ASD (Hranilovic and Blazevic,
2014, Janušonis, 2008). A previous study reported increased levels of serotonin in platelet-rich
plasma of ASD patients and their first-degree relatives (unaffected siblings, mothers, and
fathers), also a decrease in the secretion of adenosine triphosphate (ATP) from platelet dense
granules after stimulation. Moreover, the study has been reported an increase in the platelet
counts in ASD patients and their unaffected siblings compared to the normal controls (Bijl et
al., 2015). Nevertheless, platelets themselves are unable to produce serotonin, they take up
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Sanyour, A., & Alhazmi, S. (2024). Giant Platelets as Potential Biomarker for Autism Spectrum Disorder. European Journal of Applied Sciences, Vol -
12(1). 255-267.
URL: http://dx.doi.org/10.14738/aivp.121.16177
serotonin from the plasma into their dense granules via the serotonin transporter where it is
released upon activation. Consequently, it has been speculated that platelets’ function is to keep
the concentration of circulating serotonin at the normal level (Maclean and Schoenwaelder,
2019).
On the other hand, the blood-brain barrier (BBB), which tightly controls the passage of ions,
molecules, and cells between the blood and the brain tissue, is virtually impermeable to 5-HT.
In early studies on the role of serotonin in ASD, it has been assumed that biological factors that
lead to platelet hyperserotonemia may influence the autistic brain in the early development
stages when the blood-brain barrier is not yet fully formed (Janušonis, 2008, Karande, 2006).
A recent study suggested that platelets may facilitate communication between the blood and
the brain (Leiter and Walker, 2019). To date, there is no direct explanation to elucidate the
association between altered serotonin levels and a variant gene that regulated serotonin
metabolism in autism cases (Padmakumar et al., 2019, Goubau et al., 2014).
DNA methylation is the major and well-studied epigenetic mechanism that regulates gene
expression and has a critical role in normal brain development and stem cell differentiation
(Moore et al., 2013, Berdasco and Esteller, 2011). DNA methylation is essential for the
maintenance of hematopoietic stem cell (HSC) homeostasis. Additionally, aberrant DNA
methylation has been associated with several diseases such as leukemia, autoimmune disease,
and neurological diseases (Jiang et al., 2019, Jin and Liu, 2018). Moreover, aberrant methylation
of RASA3 is correlated with its differentially expressed and is implicated in neurological
disorders such as Schizophrenia and Alzheimer (Broce et al., 2019, Pidsley et al., 2014).
RASA3 is a member of the GAP1 subfamily and is also known as (Ras GTPase-Activating Protein
3, GAP1IP4 Binding Protein, Ins (1,3,4,5) P4-Binding Protein, and R-Ras GAP or GAP III). The
GAP1 family of RasGAPs comprises RASA2, RASA3, RASA4, RASAL1 (King et al., 2013). RASA3
functions as an important negative regulator of the small guanosine triphosphatases (GTPase)
Ras and Rap signaling pathways. Moreover, membrane localization of RASA3 is required for
GAP activity to downregulate RAS and RAP which plays roles in cell differentiation and
development (Chang, 2014).
Small GTPases also known as small G-proteins are molecular switches that cycle between
inactive (GDP)-bound and active (GTP)-bound states, they are deactivated by GTPase- activating proteins (GAPs), where they are activated by guanine-nucleotide exchange factors
(GEFs) (Toma-Fukai and Shimizu, 2019). Ras GTPase superfamily has obtained medical
attention due to its involvement in several diseases such as cancer, cardiovascular disorders,
aging, neurodegeneration, and developmental syndromes (Goitre et al., 2014). However, it’s
well-known that all blood cells are derived from hematopoietic stem cells that are
differentiated into progenitor cells that develop into mature circulating cells. Interestingly,
studies on mouse models have demonstrated the crucial role of RASA3 in hematopoiesis.
Moreover, RASA3 was originally purified from the plasma membrane of human platelets in
1994 and the study found that RASA3 has a pivotal role in platelet activation (Robledo et al.,
2020;Stefanini and Bergmeier, 2016). According to the above, understanding the role of RASA3
in the platelets of individuals with autism is important for enhancing our understanding of the
etiology underlying ASD.