Recombinant Human Acyl-CoA dehydrogenase family member 9, mitochondrial (ACAD9), Truncated

Uniprot Id

Q9H845

Target Species

Human

Target Name

ACAD9

Target Full Name

Complex I assembly factor ACAD9, mitochondrial

Target Function

As part of the MCIA complex, primarily participates in the assembly of the mitochondrial complex I and therefore plays a role in oxidative phosphorylation. This moonlighting protein has also a dehydrogenase activity toward a broad range of substrates with greater specificity for long-chain unsaturated acyl-CoAs. However, in vivo, it does not seem to play a primary role in fatty acid oxidation. In addition, the function in complex I assembly is independent of the dehydrogenase activity of the protein.

Target Involvement

Acyl-CoA dehydrogenase family, member 9, deficiency (ACAD9 deficiency)

Target Subcellular Location

Mitochondrion inner membrane; Peripheral membrane protein; Matrix side.

Target Protein Families

Acyl-CoA dehydrogenase family

Target Tissue Specificity

Ubiquitously expressed in most normal human tissues and cancer cell lines with high level of expression in heart, skeletal muscles, brain, kidney and liver. In the cerebellum uniquely expressed in the granular layer (at protein level).

Target Synonyms

ACAD9Complex I assembly factor ACAD9; mitochondrial; Acyl-CoA dehydrogenase family member 9; ACAD-9; EC 1.3.8.-

Target Background

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.