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ACAD9 encodes a member of the acyl-CoA dehydrogenase family. Additionally we are shipping Acyl-CoA Dehydrogenase Family, Member 9 Proteins (2) and and many more products for this protein.
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Mutations in the ND6 (show MT-ND6 Antibodies), NDUFV1 (show NDUFV1 Antibodies) or ACAD9 genes are responsible for the mitochondrial complex I deficiency.
Study identified new mutations in ACAD9 responsible for a wide spectrum of heart diseases in the presence of elevated serum lactate levels.
ACAD9 mutation is the most frequent cause of cardiac hypertrophy and isolated complex I deficiency.
Case Report: neonatal multiorgan failure due to ACAD9 mutation and complex I deficiency with mitochondrial hyperplasia in liver, cardiac myocytes, skeletal muscle, and renal tubules.
In cells where it is strongly expressed, ACAD9 plays a physiological role in fatty acid oxidation.
Our results underscore the importance of the ACAD9 protein in complex I assembly and suggest that the enzymatic activity is a rudiment of the duplication event.
Our data support a new function for ACAD9 in complex I function, making this gene an important new candidate for patients with complex I deficiency, which could be improved by riboflavin treatment.
ACAD9 screening of 120 additional complex I-defective index cases led us to identify two additional unrelated cases and a total of five pathogenic ACAD9 alleles.
Data show that two closely related metabolic enzymes, ACAD9 and VLCAD (show ACADVL Antibodies), diverged at the root of the vertebrate lineage to function in two separate mitochondrial metabolic pathways and have clinical implications for the diagnosis of complex I deficiency.
Very high activity of CPT2 (show CPT2 Antibodies) and VCLAD, involved in the metabolism of long-chain fatty acids. Fatty acid oxidation may play role in energy generation in placenta, and deficiency in may result in placental dysfunction and gestational complications.
We conclude that disturbed mitochondrial bioenergetics in BAT (show BAAT Antibodies) is a critical contributing factor for the cold sensitivity in VLCAD (show ACADVL Antibodies) deficiency
This gene encodes a member of the acyl-CoA dehydrogenase family. Members of this family of proteins localize to the mitochondria and catalyze the rate-limiting step in the beta-oxidation of fatty acyl-CoA. The encoded protein is specifically active toward palmitoyl-CoA and long-chain unsaturated substrates. Mutations in this gene cause acyl-CoA dehydrogenase family member type 9 deficiency. Alternate splicing results in multiple transcript variants.
acyl-CoA dehydrogenase family member 9, mitochondrial
, acyl-Coenzyme A dehydrogenase family, member 9
, very-long-chain acyl-CoA dehydrogenase VLCAD
, very-long-chain acyl-CoA dehydrogenase VLCAD homolog