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Fetal Hemoglobin - The Panacea for Major -Hemoglobinopathies (Hardcover)
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Fetal Hemoglobin - The Panacea for Major -Hemoglobinopathies (Hardcover)
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Intricate processes involved in perpetuating the multitude of
physiological phenomena in the human body often encounter
aberrations and omissions in the genetic code of life. While such
errors often lead to lethal diseases, at other times they provide
distinctive survival advantages and thus unscramble cues to
unconventional therapeutic strategies for life-threatening
conditions. Hereditary Persistence of Fetal Hemoglobin (HPFH) is
one such condition wherein the typically inactivated fetal form of
hemoglobin (HbF) remains overexpressed even in adult stages of the
bearer's life. Strikingly, this condition is known to ameliorate
pathological manifestations in patients with aberrant adult
hemoglobin synthesis (e.g. I(2)-hemoglobinopathies like
I(2)-thalassemia, sickle cell disease etc.). Early researchers in
the field expected such patients to suffer from clinical challenges
owing to HbF's high affinity to oxygen and consequent difficulty in
its release to cells and tissues. Surprisingly, these patients are
known to lead a physiologically normal life. Modern-day
hematologists and clinical researchers have looked-up to the
concept of "HbF reactivation" as a potential curative strategy for
patients suffering from I(2)-hemoglobinopathies like
I(2)-thalassemia and sickle cell disease. As a result, several
drugs like hydroxyurea, 5-azacytidine, cytosine arabinoside,
natural products etc. have been tried in clinics to elevate HbF
levels in such patients with limited success and poor understanding
on the mechanisms of their action. Associated side-effects and
complications of using cytotoxic agents like these restrict their
use in most instances. Fortunately, with the advent of newer
molecular tools and techniques, researchers are focusing their
attention to reengineer the molecular machinery and thus reactivate
the gamma-globin gene. This book brings together a selection of
chapters dedicated to fetal hemoglobin a its physiological role,
regulation, methodologies to manipulate and future strategies.
Researchers and scientists interested in the topic will have a
comprehensive understanding of the current concepts on fetal
hemoglobin modulation and therefore will serve as a launching pad
for their research ideas.
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