Reactome | ABCB6 transports porphyrin from cytosol to mitchondrial matrix

ABCB6 transports porphyrin from cytosol to mitchondrial matrix

Stable Identifier

R-HSA-1369065

Type

Reaction [transition]

Species

Homo sapiens

Compartment

mitochondrial outer membrane, cytosol, mitochondrial matrix

Synonyms

MTABC3 transports porphyrin into mitochondria

ReviewStatus

5/5

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ABCB6 transports porphyrin from cytosol to mitchondrial matrix

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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 Mitsuhashi, N, Miki, T, Senbongi, H, Yokoi, N, Yano, H, Miyazaki, M, Nakajima, N, Iwanaga, T, Yokoyama, Y, Shibata, T, Seino, S	J Biol Chem	2000
17006453	Identification of a mammalian mitochondrial porphyrin transporter Krishnamurthy, PC, Du, G, Fukuda, Y, Sun, D, Sampath, J, Mercer, KE, Wang, J, Sosa-Pineda, B, Murti, KG, Schuetz, JD	Nature	2006