LP15698-1
Sphingomyelin
Active
Description
Sphingomyelin (SPH) is a type of sphingolipid found in animal cell membranes, especially in the membranous myelin sheath that surrounds some nerve cell axons. It usually consists of phosphorylcholine and ceramide. In humans SPH represents ~85% of all sphingolipids.
Sphingomyelin can accumulate in a rare hereditary disease called Niemann-Pick Disease, types A and B. It is a genetically-inherited disease caused by a deficiency in the enzyme Sphingomyelinase, which causes the accumulation of Sphingomyelin in spleen, liver, lungs, bone marrow, and the brain, causing irreversible neurological damage. Of the two types involving Sphingomyelinase, type A occurs in infants. It is characterized by jaundice, an enlarged liver, and profound brain damage. Children with this type rarely live beyond 18 months. Type B involves an enlarged liver and spleen, which usually occurs in the pre-teen years. The brain is not affected. Most patients present with <1% normal levels of the enzyme in comparison to normal levels.
An excess of spingomyelin in the red blood cell membrane (as in abetalipoproteinemia) causes excess lipid accumulation in the outer leaflet of the red blood cell plasma membrane. This results in abnormally shaped red cells called acanthocytes.
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Source: Wikipedia, Wikipedia
Basic Part Properties
- Part Display Name
- Sphingomyelin
- Part Type
- Component (Describes the core component or analyte measured)
- Created On
- 2000-05-04
- Construct for LOINC Short Name
- Sphingomyelin
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Language Variants Get Info
Tag | Language | Translation |
---|---|---|
zh-CN | Chinese (China) | 神经鞘磷脂 Synonyms: 神经磷脂; |
fr-CA | French (Canada) | Sphingomyéline |
et-EE | Estonian (Estonia) | Sfingomüeliin |
es-ES | Spanish (Spain) | Esfingomielina |
it-IT | Italian (Italy) | Sfingomielina |
tr-TR | Turkish (Turkey) | Sfingomiyelin |
ru-RU | Russian (Russian Federation) | Сфингомиелин |
nl-NL | Dutch (Netherlands) | sfingomyeline |
fr-BE | French (Belgium) | Sphingomyéline |
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