Guinea Pig Acyl-CoA synthetase family member 3, mitochondrial (ACSF3) ELISA Kit | ACSF3 elisa kit
Guinea pig Acyl-CoA synthetase family member 3, mitochondrial (ACSF3) ELISA Kit
NCBI and Uniprot Product Information
NCBI Description
This gene encodes a member of the acyl-CoA synthetase family of enzymes that activate fatty acids by catalyzing the formation of a thioester linkage between fatty acids and coenzyme A. The encoded protein is localized to mitochondria, has high specificity for malonate and methylmalonate and possesses malonyl-CoA synthetase activity. Mutations in this gene are a cause of combined malonic and methylmalonic aciduria. Alternatively spliced transcript variants have been observed for this gene. [provided by RefSeq, Sep 2013]
Uniprot Description
ACSF3: Catalyzes the initial reaction in intramitochondrial fatty acid synthesis, by activating malonate and methylmalonate, but not acetate, into their respective CoA thioester. May have some preference toward very-long-chain substrates. Defects in ACSF3 are the cause of combined malonic and methylmalonic aciduria (CMAMMA). A metabolic disease characterized by malonic and methylmalonic aciduria, with urinary excretion of much larger amounts of methylmalonic acid than malonic acid, in the presence of normal malonyl-CoA decarboxylase activity. Clinical features include coma, ketoacidosis, hypoglycemia, failure to thrive, microcephaly, dystonia, axial hypotonia and/or developmental delay, and neurologic manifestations including seizures, psychiatric disease and/or cognitive decline. Belongs to the ATP-dependent AMP-binding enzyme family.
Protein type: EC 6.-.-.-; EC 6.2.1.-; Ligase
Chromosomal Location of Human Ortholog: 16q24.3
Cellular Component: mitochondrion
Molecular Function: acid-thiol ligase activity; ATP binding
Biological Process: fatty acid metabolic process; fatty acid biosynthetic process
Disease: Combined Malonic And Methylmalonic Aciduria
Research Articles on ACSF3
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Product Notes
The Guinea Pig ACSF3 acsf3 (Catalog #AAA7225122) is an ELISA Kit and is intended for research purposes only. The product is available for immediate purchase. The AAA7225122 ELISA Kit recognizes Guinea Pig ACSF3. It is sometimes possible for the material contained within the vial of "Acyl-CoA synthetase family member 3, mitochondrial (ACSF3), ELISA Kit" to become dispersed throughout the inside of the vial, particularly around the seal of said vial, during shipment and storage. We always suggest centrifuging these vials to consolidate all of the liquid away from the lid and to the bottom of the vial prior to opening. Please be advised that certain products may require dry ice for shipping and that, if this is the case, an additional dry ice fee may also be required.Precautions
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