What is the general abnormality in lipid storage diseases?

The GM1 gangliosidoses are caused by a deficiency of the enzyme beta-galactosidase, resulting in abnormal storage of acidic lipid materials particularly in the nerve cells in the central and peripheral nervous systems.

What are the common disorders associated with lipids?

The main disorders of lipid metabolism are LDL-hypercholesterolemia, hypertriglyceridemia, mixed hyperlipoproteinemia, and low HDL cholesterol. The lipoprotein(a) level can also be elevated either in isolation or in combination with other disorders of lipid metabolism.

What are the disease condition of sphingolipid storage disease?

Sphingolipid storage diseases are caused by defective catabolic activities in the endolysosomal system of the cells. Lysosomal accumulation occurs predominantly in cells and organs that have the highest rates of biosynthesis or uptake of the undegradeable sphingolipids and their precursors.

What is the deficiency of lipids is called?

Specialty. Endocrinology. A lipid storage disorder (or lipidosis) is any one of a group of inherited metabolic disorders in which harmful amounts of fats or lipids accumulate in some body cells and tissues.

What is a storage lipid?

Lipids are stored in the body in different forms such as, triglycerides, fat cells, cell membranes and lipoproteins. Any excess energy consumed is converted to triglycerides which together with globules make up 90% of the fat cells. These fat cells contain enough energy storage to keep the body functioning for 30 days.

What are lysosomal storage disorders?

Lysosomal storage diseases are inherited metabolic diseases that are characterized by an abnormal build-up of various toxic materials in the body’s cells as a result of enzyme deficiencies.

Which is involved in storage of lipids?

Lipids such as cholesterol, cholesteryl esters and triglycerides are stored in your body primarily in specialized fat cells called adipocytes, which comprise a specialized fatty tissue called adipose tissue.

What happens when lipids are imbalanced?

They cause a harmful amount of lipids to build up in your body. Over time, that can damage your cells and tissues, especially in the brain, peripheral nervous system, liver, spleen, and bone marrow. Many of these disorders can be very serious, or sometimes even fatal.

What are the abnormalities in digestion and absorption of lipids?

Lipid metabolism disorders, such as Gaucher disease and Tay-Sachs disease, involve lipids. Lipids are fats or fat-like substances. They include oils, fatty acids, waxes, and cholesterol. If you have one of these disorders, you may not have enough enzymes to break down lipids.

What causes abnormal lipid metabolism?

Disorders that affect lipid metabolism may be caused by defects in the structural proteins of lipoprotein particles, in the cell receptors that recognize the various types of lipoproteins, or in the enzymes that break down fats.

What are the disorders associated with lipids and lipoproteins?

Obesity, the metabolic syndrome and diabetes are commonly associated with disorders of lipid and lipoprotein metabolism.

What are the general characteristics of lipids?

Lipids are fatty, waxy, or oily compounds that are soluble in organic solvents and insoluble in polar solvents such as water. Lipids include: Fats and oils (triglycerides)