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Rabbit Anti-CRLS1/FITC Conjugated antibody
background:
Cardiolipin synthetase is a mitochondrial protein that belongs to the CDP-alcohol phosphatidyltransferase class-I family. CLS1 is a multi-pass membrane protein localized to the inner membrane of mitochondria. CLS1 is responsible for the catalyzing the reversible transfer of a phosphatidyl group from one phosphatidyl glycerol molecule to another. This process results in the formation of cardiolipin (CL, or diphosphatidyl glycerol) and glycerol. Diphosphatidyl glycerol is a major component of the mitochondrial membrane and constitutes roughly 20% of total mitochondrial lipids. Having four fatty acid tails rather than the usual two, CL is a double phospholipid that is synthesized in the mitochondrion itself. Defects in the CRLS1 gene are likely to effect metabolism, sustained production of ATP and could contribute to diseases such as Barth syndrome.
Function:
Catalyzes the reversible phosphatidyl group transfer from one phosphatidylglycerol molecule to another to form cardiolipin (CL) (diphosphatidylglycerol) and glycerol.
Subcellular Location:
Mitochondrion inner membrane.
Tissue Specificity:
Highly expressed in tissues such as heart, skeletal muscle and liver.
Similarity:
Belongs to the CDP-alcohol phosphatidyltransferase class-I family.
Database links:
Entrez Gene: 54675 Human
Omim: 608188 Human
SwissProt: Q9UJA2 Human
Unigene: 224764 Human
Important Note:
This product as supplied is intended for research use only, not for use in human, therapeutic or diagnostic applications.
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