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Fatal familial insomnia
Fatal familial insomnia Abbreviated FFI. An hereditary prion disease characterized by disrupted sleep (insomnia), motor abnormalities (myoclonus, ataxia, dysarthria, dysphagia, and pyramidal signs), and hyperactivation of the autonomic nervous system. Due to a missense mutation at codon 178 of the prion protein gene on chromosome 20. A sporadic form of fatal insomnia is also known.
RELATED TERMS--------------------------------------
Hereditary
Transmitted from parent to offspring; derived from ancestry.
Disease
Illness or sickness often characterized by typical patient problems (symptoms) and physical findings (signs). Disruption sequence: The events that occur when a fetus that is developing normally is subjected to a destructive agent such as the rubella (German measles) virus.
Ataxia
Ataxia is unsteady and clumsy motion of the limbs or trunk due to a failure of the fine coordination of muscle movements. The cerebellum is the center of the fine coordination of muscle movements, while the information emerging from it is carried to the muscles by the spinal cord and peripheral nerves, respectively. Therefore a disorder in either the spinal cord or in the peripheral nerves can cause ataxia.
Dysarthria
Imperfect articulation of speech due to disturbances of muscular control or incoordination.
Dysphagia
Problems in swallowing food or liquid, usually caused by blockage or injury to the esophagus.
Mutation
A change in DNA that alters a gene and thus the gene's product, leading in some cases to deformity or disease. Mutations can occur spontaneously during cell division or can be triggered by environmental stresses, such as sunlight, radiation, and chemicals.
Codon
A set of any three adjacent bases in the DNA or RNA. There are 64 different codons of which 61 specify the incorporation of an amino acid into a polypeptide chain while the remaining three are stop codons that signal the end of a polypeptide.
Protein
Any of a group of complex organic compounds which contain carbon, hydrogen, oxygen, nitrogen, and usually sulfur, the characteristic element being nitrogen. Proteins, the principal constituents of the protoplasm of all cells, are of high molecular weight and consist essentially of combinations of a-amino acids in peptide linkages. Twenty different amino acids are commonly found in proteins, and each protein has a unique genetically defined amino acid sequence which determines its specific shape and function. Their roles include enzymatic catalysis, transport and storage, coordinated motion, nerve impulse generation and many others.
Gene
1. A unit of DNA that carries information for the biosynthesis of a specific product in the cell. 2. Ultimate unit by which inheritable characteristics are transmitted to succeeding generations in all living organisms. Genes are contained by, and arranged along the length of, the chromosome. The gene is composed of deoxyribonucleic acid (DNA). Each chromosome of each species has a definite number and arrangement of genes, which govern both the structure and metabolic functions of the cells and thus of the entire organism.
Chromosome
A structural unit within a eukaryotic nucleus that carries genes. A chromosome consists of a long, continuous strand of DNA and associated proteins.
Insomnia
Disorders characterized by impairment of the ability to initiate or maintain sleep. This may occur as a primary disorder or in association with another medical or psychiatric condition.
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PREVIOUS AND NEXT TERMS--------------------------------------
Fat cell
A cell containing fat. Also called an adipocyte. A fat cell, or adipocyte, is a connective tissue cell that has differentiated and become specialized in the synthesis (manufacture) and storage of fat. The fat cell (adipocyte) is important to the body in maintaining proper energy balance, storing calories in the form of lipids, mobilizing energy sources in response to hormonal stimulation, and commanding changes by signal secretions.
Fat requirements, infant
Fat in human milk provides 30%-35% of the total daily caloric needs for a growing infant. Manufacturers of infant formulas utilize many different vegetable oils for fat including corn, soy, safflower, and coconut oils. Some formulas contain "predigested" fats known as medium chain triglycerides (MCT). These are analogous to the "predigested proteins." Because of their unique application, formulas containing MCT are not routinely recommended for healthy infants and children.
Fat, saturated
A fat that is solid at room temperature and comes chiefly from animal food products. Some examples are butter, lard, meat fat, solid shortening, palm oil, and coconut oil. These fats tend to raise the level of cholesterol in the blood.
Fat, trans
An unhealthy substance, also known as trans fatty acid, made through the chemical process of hydrogenation of oils. Hydrogenation solidifies liquid oils and increases the shelf life and the flavor stability of oils and foods that contain them. Trans fat is found in vegetable shortenings and in some margarines, crackers, cookies, snack foods and other foods.
Fat, unsaturated
A fat that is liquid at room temperature and comes from a plant such as olive, peanut, corn, cottonseed, sunflower, safflower, or soybean. Unsaturated fats tend to lower the level of cholesterol in the blood.
Fatal familial insomnia
Father
(1) The male parent. (2) One who originates. For example, "Wilhelm Roentgen is considered the father of radiology." (3) To produce offspring as a male. (4) To provide paternal protection and guidance to children.
Fatty acid
A condition characterized by a lessened capacity for work and reduced efficiency of accomplishment, usually accompanied by a feeling of weariness and tiredness. Fatigue can be acute and come on suddenly or chronic and persist.
Fatty acid, trans
An unhealthy substance, also known as trans fat, made through the chemical process of hydrogenation of oils. Hydrogenation solidifies liquid oils and increases the shelf life and the flavor stability of oils and foods that contain them. Trans fat is found in vegetable shortenings and in some margarines, crackers, cookies, snack foods and other foods.
Fatty acids
Molecules that are long chains of lipid-carboxylic acid found in fats and oils and in cell membranes as a component of phospholipids and glycolipids.
Fatty acids,omega-3
A class of fatty acids found in fish oils, especially from salmon and other cold-water fish, that acts to lower the levels of cholesterol and LDL (low-density lipoproteins) in the blood.
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