Recombinant Human ATP-binding cassette sub-family B member 8, mitochondrial (ABCB8), Truncated

Uniprot Id

Q9NUT2

Target Species

Human

Target Name

ABCB8

Target Full Name

Mitochondrial potassium channel ATP-binding subunit

Target Function

ATP-binding subunit of the mitochondrial potassium channel located in the mitochondrial inner membrane. Together with CCDC51/MITOK, forms a protein complex localized in the mitochondria that mediates ATP-dependent potassium currents across the inner membrane (that is, mitoK(ATP) channel). Plays a role in mitochondrial iron transport. Required for maintenance of normal cardiac function, possibly by influencing mitochondrial iron export and regulating the maturation of cytosolic iron sulfur cluster-containing enzymes.

Target Subcellular Location

Mitochondrion inner membrane; Multi-pass membrane protein.

Target Protein Families

ABC transporter superfamily, ABCB family, Multidrug resistance exporter (TC 3.A.1.201) subfamily

Target Tissue Specificity

Ubiquitous.

Target Synonyms

ABCB8; MABC1; MITOSUR; Mitochondrial potassium channel ATP-binding subunit; ATP-binding cassette sub-family B member 8, mitochondrial; ABCB8; Mitochondrial ATP-binding cassette 1; M-ABC1; Mitochondrial sulfonylurea-receptor; MITOSUR

Target Background

This nuclear gene encodes a multi-pass membrane protein that is targeted to the mitochondrial inner membrane. The encoded protein is an ATP-dependent transporter that may mediate the passage of organic and inorganic molecules out of the mitochondria. Loss of function of the related gene in mouse results in a disruption of iron homeostasis between the mitochondria and cytosol. Alternative splicing results in multiple transcript variants.