Basic Cooking Terms Defined. 1  Al dente: Italian term to describe pasta and rice that are cooked until tender but still firm to the bite. 2  Bain-marie: A pan of water that is used to help mixtures, such as custards, bake evenly and to protect them from the direct heat of the oven or stove.
Gaucher Disease and Heredity. Gaucher disease is an inherited, genetic disorder. People inherit two copies of every geneâone from each parent. Genes contain information about our genetic makeup, including physical characteristics such as eye color and height.All genes that an individual inherits are organized on 23 pairs of chromosomes.he genes for production of the enzyme glucocerebrosidase are passed from parent to child. In patients with Gaucher disease, two copies of the glucocerebrosidase gene that the individual inherited from his or her parents are both defective; thus, the glucocerebrosidase is unable to function normally.
Gaucher's Disease. Gaucher's disease is the most common type of lipid storage disease. It is by caused by a deficiency of an enzyme called glucocerebrosidase. In this inherited disease fat collects in the brain, lungs, liver, spleen, kidneys and bone marrow.ipid or fatty acid oxidation disorders are caused by a deficiency or lack of the enzymes needed to break down fats to produce energy. This decreases energy in the body and leads to delayed physical and mental development, according to the National Institute of Neurological Disorders and Stroke.