Nutrition Publication

NNIW50 - Metabolic Disorders Diseases in Infant and Child Nutrition

Editor(s): C. Bachman, B. Koletzko. vol. 50

Related Articles

Genetic Variation of Homocysteine Metabolism and Atherosclerosis

Author(s): J.M. Scott

Homocysteine is an amino acid that is found in mammalian cells and in the plasma.It arises in the first instance from the catabolism of the essential dietary amino acidmethionine, to which it is very similar in structure (Fig. 1).

Minimum Protein Requirements in Infancy and Childhood: Insights from Patients with Protein-Restricted Diets

Author(s): H. Ogier de Baulny, L. François, M.O. Greneche, M. Giraud, E. Depondt

Knowledge about the normal child's requirements for protein and essential aminoacids is still limited. Two approaches have been used to estimate protein requirements.

How to Assess Amino Acid Requirements (With Particular Reference to Inborn Errors of Amino Acid Metabolism)

Author(s): P.B. Pencharz, R.O. Ball

The 20 amino acids for which transfer RNA exists are the components from whichall proteins are synthesized. If they cannot be synthesized endogenously in sufficientamounts then they are needed in the diet (1).

Long-Chain Omega-3 Fatty Acids Are Conditionally Essential Substrates in Children: Effects on Visual Function in Children with Phenylketonuria

Author(s): B. Koletzko, S. Beblo, A. Muntau-Heger, W. Mueller-Felber, H. Reinhardt

Classical phenylketonuria (PKU) is one of the most common inborn errors of metabolismand affects about 1 in 7,000 white neonates. PKU is caused by deficientactivity of hepatic phenylalanine hydroxylase.

Fatty Acid Regulation of Gene Expression: A Genomic Mechanism to Improve the Metabolic Syndrome

Author(s): S.D. Clarke, C.Nelson, D. Gasperikova, J. DeMar, A. Lapillonne, W.C. Heird

The development of obesity and associated insulin resistance involves a multitude ofgene products, including proteins involved in lipid synthesis and oxidation, thermogenesis,and cell differentiation.

Genetic Disorders of Mitochondrial and Peroxisomal Fatty Acid Oxidation and Peroxisome Proliferator-Activated Receptors

Author(s): R.J.A. Wanders

Fatty acids are an important source of energy in humans, especially during fasting.Most tissues are able to degrade fatty acids to carbon dioxide and water, but in addition,some organs—notably the liver—have the capacity to convert the acetyl-CoAunits produced during |3 oxidation into the ketone bodies acetoacetate and 3-hydroxybutyrate.

Oxidative Stress: Antioxidants In Degenerative Neurologic And Ophthalmologic Diseases

Author(s): Helmut Sies, Wilhelm Stahl, and Maria Cristina Polidori

Reactive oxygen species, also called prooxidants, are produced physiologically duringnormal metabolic reactions in the aerobic organism. However, when they are producedin excessive amounts and are not sufficiently detoxified, the steady state balancebetween prooxidants and antioxidants may be disrupted.

Carbohydrate Therapy of Congenital Disorders of Glycosylation

Author(s): T. Marquardt

Inherited disorders caused by defects in the assembly or processing of glycoconjugatesare called congenital disorders of glycosylation (CDG). Until recently, thesediseases were called carbohydrate-deficient glycoprotein syndromes (CDG syndromes,CDGS), but rapidly improving knowledge in recent years has led to the needfor a more general term (1).

Genetic Variability of Human Milk Oligosaccharides: Are There Biologic Consequences?

Author(s): C. Kunz, L. Bode, S. Rudloff

Human milk is a rich source of complex oligosaccharides synthesized within themammary gland. The concentration of this fraction, however, varies widely with lactationalstage, decreasing from large amounts of up to 50 g/1 or more in colostrum toan average of 5 to 10 g/1 in mature milk (1).

From Arginine to Creatine: Regional and Cellular Gene Expression of Enzymes and Transporters Linking Nitrogen and Energy Metabolism in Brain

Author(s): C. Bachmann, H. Henry, O. Braissant

Arginine, a semiessential amino acid, is provided to the organism by protein intake andnewly synthesized from citruUine, in the kidney. In the liver and intestine it is the finaluseful product of the urea cycle.

Current Understanding of the Molecular and Cellular Basis of Celiac Disease

Author(s): L.M. Sollid

Celiac disease has received increased attention in recent years. It has become evidentthat this is a common disorder in Western societies, affecting around 1:130 to 1:300individuals (1).

Genetic Variations of Gliadins: New Approaches for the Treatment of Celiac Disease

Author(s): O.D. Anderson

The ingestion of a wide range of foods by humans is accompanied by an equally widerange of responses to those foods. While there are examples of raw foodstuffs containingmajor allergens or toxins requiring special processing before ingestion, suchsources do not usually form part of the human diet.

Current Uses and Future Perspectives of Recombinant Modifications in Food Production

Author(s): D.M. Bier

Agriculture is the world's oldest and largest industry. For more than 10,000 years,humans have been using biologic technologies to select and improve desired characteristicsin plants and animals for food and other uses.

Diet as a Determinant of Drug Response

Author(s): D.G. Bailey

Therapeutic drugs and food are often taken together. Linking drug administration to aregular event like a meal can improve compliance with a treatment regimen, especiallyin elderly people (1).

Diet, Gene Expression, and Apoptosis: Clues to Cancer Prevention?

Author(s): H. Daniel, U. Wenzel

The development of cancer results from the interplay between genetic factors and theenvironment, and dietary factors have been identified as modulators of almost everystep in the process.

Gene Therapy: A Potential Solution for Nutrition-Dependent Disorders?

Author(s): M.L. Batshaw, J.M. Wilson, X. Ye, S.E. Raper

Over the past few decades there has been significant progress in the treatment of inheritednutrition-dependent disorders, especially the inborn errors of amino acid andorganic acid metabolism (1).