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
Locations in the PathwayBrowser
General
SVG |   | PPTX  | SBGN
Click the image above or here to open this reaction in the Pathway Browser
The layout of this reaction may differ from that in the pathway view due to the constraints in pathway layout

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
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
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
Participants
Participant Of
Catalyst Activity
Catalyst Activity
Title
heme transporter activity of MTABC3 dimer [mitochondrial outer membrane]
Physical Entity
Activity
Orthologous Events
Authored
Reviewed
Created