ABCB6 transports porphyrin from cytosol to mitchondrial matrix

Stable Identifier
R-HSA-1369065
Type
Reaction [transition]
Species
Homo sapiens
Compartment
Synonyms
MTABC3 transports porphyrin into mitochondria
ReviewStatus
5/5
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The human gene ABCB6 encodes a mitochondrial half-type ATP-binding cassette (ABC) protein MTABC3 which is uniquely located on the outer mitochondrial membrane and is functional as a homodimer (Krishnamurthy et al. 2006). It plays a crucial role in heme synthesis by mediating porphyrin uptake into mitochondria (Mitsuhashi et al. 2000, Krishnamurthy et al. 2006).
Literature References
PubMed ID Title Journal Year
10837493 MTABC3, a novel mitochondrial ATP-binding cassette protein involved in iron homeostasis

Nakajima, N, Yokoi, N, Mitsuhashi, N, Seino, S, Yano, H, Iwanaga, T, Shibata, T, Yokoyama, Y, Miki, T, Miyazaki, M, Senbongi, H

J Biol Chem 2000
17006453 Identification of a mammalian mitochondrial porphyrin transporter

Sosa-Pineda, B, Sampath, J, Fukuda, Y, Sun, D, Murti, KG, Krishnamurthy, PC, Du, G, Schuetz, JD, Wang, J, Mercer, KE

Nature 2006
Participants
Participates
Catalyst Activity

ABC-type heme transporter activity of MTABC3 dimer [mitochondrial outer membrane]

Orthologous Events
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