Table of Contents
Cover
Title page
Copyright
Contributors
Section A
Chapter 1: An overview on metabolic disorders and current therapy
Abstract
1: Introduction
2: Origins of metabolic disease
3: Metabolic disorders
4: Present status and new trends on the treatment of metabolic disorders
5: Pharmacologic enzyme replacement therapy (ERT)
6: Conclusions
References
Chapter 2: Effects of phytonutrients in various metabolic pathways
Abstract
1: Introduction
2: Significance of metabolic pathways in health and diseases
3: Phytonutrients
4: Conclusions
References
Chapter 3: Nanotechnology and phytonutrients
Abstract
1: Introduction
2: Resveratrol
3: Emodin
4: Berberine
5: Curcumin
6: Quercetin
7: Other phytonutrients
8: Conclusions
References
Section B
Chapter 4: Genetic effects of phytonutrients in metabolic disorders
Abstract
1: Introduction
2: Phytonutrient-rich dietary components and genetic interactions
3: Phytonutrients/natural biomolecules targeting metabolic cell signaling pathways
4: Dietary fats involved with gene interactions in metabolic disorders
5: Conclusions References
Chapter 5: Therapeutic role of nutraceuticals in the management of brain disorders
Abstract
Authors’ contribution
1: Introduction
2: Classes of neutraceuticals and their mechanisms of action
3: Current evidence on the use of neutraceuticals in multiple sclerosis
4: Amyotrophic lateral sclerosis and nutritional supplementations
5: Effects of neutraceuticals supplementation in Parkinson’s disease
6: Effects of nutraceuticals in Huntington’s disease
7: The role of nutraceuticals supplementation in brain ataxia
8: Proposed neuroprotective mechanisms of some popular nutraceuticals
9: Conclusions
References
Chapter 6: Phytonutrients in the management of glucose metabolism
Abstract
1: Introduction
2: Diabetes mellitus
3: Phytonutrients and DM
4: Concluding remarks
References
Chapter 7: Phytonutrients in the management of lipids metabolism
Abstract
1: Introduction
2: Disorders of lipid metabolism
3: Hypertriglyceridemia
4: Hypercholesterolemia
5: Low HDL-related abnormalities
6: Atherosclerosis
7: Obesity
8: Cancer
9: The role of phytonutrients in managing lipid-metabolismassociated disorders
10: Concluding remarks
References
Chapter 8: Cancer metabolism regulation by phytonutrients
Abstract
1: Introduction
2: Pentose phosphate pathway (PPP)
3: Serine pathway
4: Targeting enzymes of TCA by phytonutrients
5: Lactic acid fermentation
6: Other mechanisms involved in cancer metabolism and their modulation by phytonutrients
7: Clinical trials
8: Bioavailability of phytonutrients
9: Synergistic effects of phytonutrients
10: Conclusions
References
Chapter 9: Acid-base and electrolyte balance regulations with phytonutrients
Abstract
1: Introduction
2: Acid-base balance regulations
3: Phytonutrients
4: Regulation of acid-base and electrolyte balance with phytonutrients
5: Conclusions
References
Chapter 10: Therapeutic role of nutraceuticals in mitochondrial disorders
Abstract
1: Introduction
2: Mitochondrial disorders
3: Therapeutic nutraceuticals
4: Traditional nutraceuticals
5: Plant-based nutraceuticals
6: Fruit- and vegetable-based nutraceuticals
7: Nutraceutical biocatalyst
8: Nontraditional nutraceuticals
9: Molecular mechanisms/targeting signaling pathway
10: Nutraceuticals interact with protein misfolding and endoplasmic reticulum stress pathway
11: Combined effects of nutraceuticals
12: Interaction of nutraceuticals with medicines
13: Regulatory challenges of nutraceuticals
14: Conclusions References
Chapter 11: Phytonutrients in regulation of malabsorption disorders
Abstract
1: Summary
2: Introduction
3: Pathophysiology of malabsorption
4: The role of phytonutrients in management of malabsorptionrelated disorders
5: Conclusions
References
Chapter 12: Skin metabolic syndrome and phytonutrients
Abstract
Acknowledgment
1: Introduction
2: Metabolic syndrome and skin diseases
3: Phytochemicals in metabolic syndrome
4: Conclusions
References
Chapter 13: Cachexia and phytonutrients
Abstract
1: Introduction
2: Cachexia and molecular mechanisms: Targeted signaling pathways
3: Effect of natural products on cachexia
4: Conclusions
References
Copyright
Academic Press is an imprint of Elsevier
125 London Wall, London EC2Y 5AS, United Kingdom
525 B Street, Suite 1650, San Diego, CA 92101, United States
50 Hampshire Street, 5th Floor, Cambridge, MA 02139, United States
The Boulevard, Langford Lane, Kidlington, Oxford OX5 1GB, United Kingdom
Copyright © 2022 Elsevier Inc. All rights reserved.
No part of this publication may be reproduced or transmi ed in any form or by any means, electronic or mechanical, including photocopying, recording, or any information storage and retrieval system, without permission in writing from the publisher. Details on how to seek permission, further information about the Publisher’s permissions policies and our arrangements with organizations such as the Copyright Clearance Center and the Copyright Licensing Agency, can be found at our website: www.elsevier.com/permissions.
This book and the individual contributions contained in it are protected under copyright by the Publisher (other than as may be noted herein).
Notices
Knowledge and best practice in this field are constantly changing. As new research and experience broaden our understanding, changes in research methods, professional practices, or medical treatment may become necessary.
Practitioners and researchers must always rely on their own experience and knowledge in evaluating and using any information, methods, compounds, or experiments described herein. In using such information or methods they should be mindful of their own safety and the safety of others, including parties for whom they have a professional responsibility.
To the fullest extent of the law, neither the Publisher nor the authors, contributors, or editors, assume any liability for any injury and/or damage to persons or property as a ma er of products liability, negligence or otherwise, or from any use or operation of any methods, products, instructions, or ideas contained in the material herein.
ISBN 978-0-12-824356-5
For information on all Academic Press publications visit our website at h ps://www.elsevier.com/books-and-journals
Publisher: Andre G. Wolff
Editorial Project Manager: Samantha Allard
Production Project Manager: Maria Bernard
Cover Designer: Mark Rogers
Typeset by STRAIVE, India
Contributors
Imad Ahmad
Department of Pharmacy, Abdul Wali Khan University Mardan, Mardan
Department of Pharmacy, Abasyn University Peshawar, Peshawar, Pakistan
Esra Küpeli Akkol Department of Pharmacognosy, Faculty of Pharmacy, Gazi University, Ankara, Turkey
Waqas Alam Department of Pharmacy, Abdul Wali Khan University Mardan, Mardan, Pakistan
Ahmed Al-Harrasi Natural and Medical Sciences Research Centre, University of Nizwa, Nizwa, Oman
Reem Hasaballah Alhasani
Department of Biology, Jamoum University College
Department of Biology, Faculty of Applied Science, Umm Al-Qura University, Makkah, Saudi Arabia
Ifat Alsharif
Department of Biology, Faculty of Applied Science
Department of Biology, Jamoum University College, Umm Al-Qura University, Makkah, Saudi Arabia
Norah A. Althobaiti Department of Biology, College of Science and Humanities, Shaqra University, Al-Quwaiiyah, Saudi Arabia
Giuseppe Annunziata Department of Pharmacy, University of Naples Federico II, Naples, Italy
Michael Aschner Department of Molecular Pharmacognosy, Albert Einstein College of Medicine, Bronx, NY, United States
Amira Yasmine Benmelouka Faculty of Medicine, University of Algiers, Algiers, Algeria
Shabana Bibi
Department of Biosciences, Shifa Tameer-e-Millat University, Islamabad, Pakistan
Yunnan Herbal Laboratory, School of Ecology and Environmental Sciences
Research Center for Sustainable Utilization of Cordyceps
Bioresources in China and Southeast Asia, Yunnan University, Kunming, China
Partha Biswas Department of Genetic Engineering and Biotechnology, Faculty of Biological Science and Technology, Jashore University of Science and Technology (JUST), Jashore, Bangladesh
Shazia Anwer Bukhari Department of Biochemistry, Government College University, Faisalabad, Pakistan
Gul Bushra Department of Bioinformatics and Biotechnology, Faculty of Life Sciences, Government College University, Faisalabad, Pakistan
Maria Daglia
Department of Pharmacy, School of Medicine and Surgery, University of Naples Federico II, Naples, Italy
International Research Center for Food Nutrition and Safety, Jiangsu University, Zhenjiang, China
Alaa Ahmed Elshanbary Faculty of Medicine, Alexandria University, Alexandria, Egypt
Sajad Fakhri Pharmaceutical Sciences Research Center, Health Institute, Kermanshah University of Medical Sciences, Kermanshah, Iran
Mohammad Mehedi Hasan Department of Biochemistry and Molecular Biology, Mawlana Bhashani Science and Technology
University, Tangail, Bangladesh
Yaseen Hussain
Laboratory of Controlled Release and Drug Delivery System, College of Pharmaceutical Sciences, Soochow University, Suzhou, China
Department of Pharmacy, Bashir Institute of Health Sciences, Islamabad, Pakistan
Shabnoor Iqbal Department of Zoology, Faculty of Life Sciences, Government College University, Faisalabad, Pakistan
Muhammad Irfan Department of Pharmaceutics, Government College University, Faisalabad, Pakistan
Nazia Kanwal Department of Life Sciences, Khwaja Fareed University of Engineering and Information Technology, Rahim Yar Khan, Pakistan
Abdul Haleem Khan Department of Pharmacy, Forman Christian College (A Chartered University), Lahore, Pakistan
Ajmal Khan Natural and Medical Sciences Research Centre, University of Nizwa, Nizwa, Oman
Haroon Khan Department of Pharmacy, Abdul Wali Khan University Mardan, Mardan, Pakistan
Mostafa Meshref Department of Neurology, Al-Azhar University, Cairo, Egypt
Sana Piri Pharmaceutical Sciences Research Center, Health Institute, Kermanshah University of Medical Sciences, Kermanshah, Iran
Shafiq Ur Rahman Department of Pharmacy, Shaheed Benazir Bhu o University, Sheringal, Dir Upper, Pakistan
Azhar Rasul
Department of Life Sciences, Khwaja Fareed University of Engineering and Information Technology, Rahim Yar Khan
Department of Zoology, Faculty of Life Sciences, Government College University, Faisalabad, Pakistan
Ammara Riaz Department of Life Sciences, Khwaja Fareed University of Engineering and Information Technology, Rahim Yar Khan, Pakistan
Ayesha Sadiqa Department of Life Sciences, Khwaja Fareed University of Engineering and Information Technology, Rahim Yar Khan, Pakistan
Uzma Saleem Department of Pharmacology, Faculty of Pharmaceutical Sciences, Government College University, Faisalabad, Pakistan
Iqra Sarfraz Department of Life Sciences, Khwaja Fareed University of Engineering and Information Technology, Rahim Yar Khan, Pakistan
Gökçe Şeker Karatoprak Department of Pharmacognosy, Faculty of Pharmacy, Erciyes University, Kayseri, Turkey
Ghulam Mujtaba Shah Department of Pharmacy Department of Botany, Hazara University, Mansehra, Pakistan
Muhammad Ajmal Shah Department of Pharmacy, Hazara University, Mansehra Department of Pharmacognosy, Faculty of Pharmaceutical Sciences, Government College University, Faisalabad, Pakistan
Shahid Shah Department of Pharmacy Practice, Faculty of Pharmaceutical Sciences, Government College University, Faisalabad, Pakistan
Farzana Shareef Department of Life Sciences, Khwaja Fareed University of Engineering and Information Technology, Rahim Yar Khan, Pakistan
Anastasiia Shkodina Poltava State Medical University, Poltava, Ukraine
Antoni Sureda Research Group on Community Nutrition and Oxidative Stress (NUCOX), Department of Fundamental Biology
and Health Sciences, Health Research Institute of the Balearic Islands (IdISBa), and CIBEROBN (Physiopathology of Obesity and Nutrition
CB12/03/30038), University of the Balearic Islands, Palma, Spain
Silvia Tejada Laboratory of Neurophysiology, Department of Biology, Health Research Institute of the Balearic Islands (IdISBa), and CIBEROBN (Physiopathology of Obesity and Nutrition
CB12/03/30038), University of the Balearic Islands, Palma, Spain
Gian Carlo Tenore Department of Pharmacy, University of Naples Federico II, Naples, Italy
Ilknur Ucak Nigde Omer Halisdemir University, Nigde, Turkey
Hammad Ullah Department of Pharmacy, School of Medicine and Surgery, University of Naples Federico II, Naples, Italy
Çiğdem Yücel Department of Pharmaceutical Technology, Faculty of Pharmacy, Erciyes University, Kayseri, Turkey
Rabia Zara Department of Life Sciences, Khwaja Fareed University of Engineering and Information Technology, Rahim Yar Khan, Pakistan
Chapter 1: An overview on metabolic disorders and current therapy
Esra Küpeli Akkola; Michael Aschnerb a Department of Pharmacognosy, Faculty of Pharmacy, Gazi University, Ankara, Turkey b Department of Molecular Pharmacognosy, Albert Einstein College of Medicine, Bronx, NY, United States
Abstract
Metabolic disorders occur when the breakdown of food to its components becomes disrupted. Disorders in metabolism can be inherited, in which case they are known as inborn errors of metabolism, or they may be acquired during the lifetime. Metabolic disorders can be inherent to severe diseases or conditions, including respiratory or liver failure, chronic obstructive pulmonary disease, cancer, and HIV/AIDS. Occasionally highly complex pathways mediate metabolic disorders. At other times, one basepair of the DNA may be solely responsible. These discoveries have led scientists to develop extraordinary treatments for affected individuals, and the pace of discovery continues to accelerate. The symptoms of metabolic disorders vary among individuals and by the type of the disorder. Some metabolic disorders result in mild symptoms that can be managed with treatment and lifestyle changes, whereas others can cause severe and life-threatening symptoms, such as seizures, breathing problems, and organ failure. Some
inherited metabolic disorders can require long-term nutritional supplementation and treatment, however, metabolic disorders that arise as a result of another disease or disorder frequently resolve once the underlying condition is treated.
Keywords
Metabolism; Metabolic disease; Inheritance; Organic acidemias; Disorders; Symptoms
1: Introduction
Metabolism is a process involving the transformation of metabolites, the biochemical pathways in which this transformation takes place, and the mechanisms that regulate metabolite flow in the pathways. Any disease or disorder that disrupts normal metabolism, the process of converting food into energy at the cellular level, is referred to as a metabolic disease. Thousands of enzymes that participate in numerous interconnected metabolic pathways play a role in this process [1]. Metabolic diseases affect the cell's ability to perform critical biochemical reactions involving the processing or transport of carbohydrates (sugars and starches), proteins (amino acids), or lipids (fa y acids).
While metabolic diseases are typically inherited, most individuals affected by them may appear healthy for days, months, or even years. Symptoms usually occur when the body's metabolism undergoes extensive stress, such as prolonged starvation or a febrile illness [2]. Detection of some metabolic disorders is possible with prenatal diagnostic screening [3]. This type of analysis is often offered to families who have a previous child with a metabolic disease or who belong to a defined ethnic group. If a metabolic disorder is detected in a baby soon after birth, appropriate treatment can be initiated early, resulting in an improved prognosis. Early initiation of treatment responds very well in some metabolic disorders [4]. However, others have no effective therapy and cause
severe problems, despite the early diagnosis. Symptoms of inherited and acquired metabolic disorders are presented in Table 1.
Table 1
Symptoms of inherited and acquired metabolic disorders.
Symptoms of inherited metabolic disorders
Body fluids that have a maple smell
Bone abnormalities such as osteoporosis (thinning and weakening of the bones)
Difficulty with memory, thinking, talking, comprehension, writing or reading
Enlarged liver, heart, kidney, or spleen
Failure to thrive in infants and children
Frequent infections
Hypoglycemia (low blood sugar)
Symptoms of acquired metabolic disorders
Chronic or persistent diarrhea
Fatigue
Headache
Irritability and mood changes
Muscle cramping
Nausea with or without vomiting
Rapid breathing (tachypnea) or shortness of breath
Loss of vision or changes in vision Bluish coloration of the lips or fingernails
Muscle twitching, spasms, or seizures
Change in mental status or sudden behavior change, such as confusion, delirium, lethargy, hallucinations, and delusions
Symptoms of inherited metabolic disorders
Symptoms of acquired metabolic disorders
Muscle weakness Respiratory or breathing problems, such as shortness of breath, difficulty breathing, labored breathing, wheezing, not breathing, choking
Paralysis
Seizure
Metabolic diseases are very rare when considered individually, but relatively common when considered as a group. Specific metabolic disorders have incidences ranging from approximately 1 in 500 to less than 1 in 1,000,000. When metabolic disorders are considered as a whole, they are estimated to affect approximately 1 in 1000 people [5]. Certain chronic medical conditions, such as lung or kidney disease (includes any type of kidney problem, such as kidney stones, kidney failure, and kidney anomalies), family history of genetic metabolic disorder and HIV/AIDS are the risk factors for metabolic disorders [6].
The treatment approach for metabolic disorders depends on the specific disorder. Inherited metabolic disorders are often treated with nutritional counseling and support, periodic assessment, physical therapy, and other supportive care options. Acquired metabolic disorder treatment includes normalizing the metabolic balance by both reversing the cause and administering medications [7,8].
In this chapter, origins of metabolic diseases, types of metabolic disorders, and present status and new trends on the treatment of metabolic disorders are presented.
2: Origins of metabolic disease
2.1: Metabolic pathways
Foods are broken down into products of different biochemical structures in a series of steps by means of cellular enzymes. Then
these products become a substrate for the next enzyme in a metabolic pathway [9]. In this process, if there is an enzyme deficiency or a decrease in enzyme activity, the pathway becomes blocked and the formation of the final product is insufficient, causing disease [10,11]. Low activity of an enzyme may cause subsequent accumulation of the enzyme substrate, which can be toxic at high levels. Furthermore, minor metabolic pathways that generally lie dormant may be activated when a substrate accumulates, probably forming atypical, potentially toxic, products. Each cell contains many metabolic pathways, all of which are interlinked to some extent, so that a single blockage may affect a plethora of biochemical processes [12,13].
Serious problems can arise as a result of metabolic imbalance. Depending on which enzyme is dysfunctional, blindness, deafness, seizures, mental disability, decreased muscle tone and organ failure may occur [14]. In recent years, it has been determined that even some conditions associated with multiple congenital anomalies such as Smith-Lemli-Opi syndrome are caused by an underlying metabolic cause.
2.2: Inheritance
The inheritance of inborn errors of metabolism is most frequently autosomal recessive, the importance that two mutant genes are essential to produce the symptoms and signs of disease [15]. The parents of an affected child are most often asymptomatic carriers since 50% of normal enzyme activity is adequate to maintain necessary health[16]. When two carriers of a deleterious trait produce offspring, there is a 25% chance of having an affected child, a 25% chance of having a child without the mutant allele, and a 50% chance of having a child who is similarly a carrier (Fig. 1). In genetic terms, the carrier of an autosomal recessive situation has only one mutant gene (heterozygous), whereas an affected individual has two mutant genes (homozygous) [17]. All human beings have about six recessive mutant alleles in their genomes, however, it is relatively rare for an individual to mate with someone who carries a mutation in the same
