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Rabbit Anti-ACADM/FITC Conjugated antibody
background:
ACADM protein is a medium chain specific (C4 to C12 straight chain) acyl Coenzyme A dehydrogenase. The enzyme catalyzes the initial step of the mitochondrial fatty acid beta oxidation pathway. ACADM expression is induced during periods of fasting, when reliance on fatty acids for energy is increased. Clinical phenotypes are associated with ACADM hereditary deficiency.
Function:
This enzyme is specific for acyl chain lengths of 4 to 16.
Subunit:
Homotetramer. Interacts with the heterodimeric electron transfer flavoprotein ETF.
Subcellular Location:
Mitochondrion matrix.
DISEASE:
Acyl-CoA dehydrogenase medium-chain deficiency (ACADMD) [MIM:201450]: An inborn error of mitochondrial fatty acid beta-oxidation which causes fasting hypoglycemia, hepatic dysfunction and encephalopathy, often resulting in death in infancy. Note=The disease is caused by mutations affecting the gene represented in this entry.
Similarity:
Belongs to the acyl-CoA dehydrogenase family.
Database links:
Entrez Gene: 505968 Cow
Entrez Gene: 34 Human
Entrez Gene: 11364 Mouse
Entrez Gene: 24158 Rat
Omim: 607008 Human
SwissProt: Q3SZB4 Cow
SwissProt: P11310 Human
SwissProt: P45952 Mouse
SwissProt: P08503 Rat
Unigene: 445040 Human
Unigene: 10530 Mouse
Unigene: 6302 Rat
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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