A striped racer, also known as the California whipsnake, is a species of non-venomous snake native to the coast and foothills of California. It is a long, slender, and fast-moving snake, identified by its distinctive stripes running down the length of its body. They primarily feed on lizards and small rodents. The scientific name for this species is Masticophis lateralis.
Overview of lysosomal storage disorders
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Lysosomal storage disorders are hereditary metabolic disorders. Hereditary disorders occur when parents pass the defective genes that cause these disorders on to their children. There are different types of inherited disorders. In many hereditary metabolic disorders, both parents of the affected child carry a copy of the abnormal gene. Because usually two copies of the abnormal gene are necessary for the disorder to occur, usually neither parent has the disorder. Some hereditary metabolic disorders are X-linked, which means only one copy of the abnormal gene can cause the disorder in boys.
Lysosomes are tiny components within cells. Lysosomes contain enzymes that break down (metabolize) many types of molecules entering a cell. When the enzymes do not work correctly, the molecules build up, causing damage in many areas in the body.
Types of lysosomal storage disorders includeMucopolysaccharidoses
Sphingolipidoses
LipidosesMucopolysaccharidoses occur when the body lacks enzymes needed to break down and store complex sugar molecules (glycosaminoglycans).
Sphingolipidoses occur when people do not have the enzymes needed to break down sphingolipids, which are compounds that protect the cell surface and serve certain functions in the cells. There are many types of sphingolipidoses. The most common sphingolipidosis isGaucher disease
Others sphingolipidoses include
Fabry disease
Krabbe disease
Metachromatic leukodystrophy
Niemann-Pick disease (types A and
Sandhoff disease
Tay-Sachs diseaseLipidoses occur when the body lacks one of the enzymes that help the body break down and process fats (lipids). This can lead to the buildup of specific fatty substances that normally would have been broken down by the enzymes. Over time, accumulations of these substances can be harmful to many organs of the body. Lipidoses include
Cholesteryl ester storage disease
Wolman diseaseBefore birth, doctors diagnose some lysosomal storage disorders by doing the prenatal screening tests amniocentesis or chorionic villus sampling. After birth, some lysosomal storage disorders are diagnosed by routine newborn screening tests or other tests.
Treatment of lysosomal storage disorders varies depending on the substances that accumulate in the blood and tissues.
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