ABC7, mABC1 and mABC2 mediate heme transport

Stable Identifier
Reaction [transition]
Homo sapiens
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Mitochondrial ABC transporters are thought to play a key role in iron metabolism and heme biosynthesis. All mitochondrial ABC transporters described to date are of the half-transporter type and would probably function as dimers (Ramjeesingh et al. 2003) but their dimerization partners have not yet been identified. ABC7 is the functional human orthologue of yeast Atm1p (Csere et al. 1998), is predicted to dimerize in the same way as Atm1p (Chloupkov√° et al. 2004) and is probably involved in iron homeostasis. Defects in ABCB7 are the cause of X-linked sideroblastic anemia with ataxia (ASAT) [MIM:301310] (Allikmets et al. 1999). Human genes ABCB8 and ABCB10 encode mABC1 and mABC2 respectively (Hogue et al. 1999, Zhang et al 2000 respectively). They would be expected to dimerize, as demonstrated for mABC2 (Graf et al. 2004). Both are believed to have similar functionality to ABC7 although this has not been demonstrated yet.

Literature References
PubMed ID Title Journal Year
15215243 Targeting, import, and dimerization of a mammalian mitochondrial ATP binding cassette (ABC) transporter, ABCB10 (ABC-me)

Graf, SA, Haigh, SE, Shirihai, OS, Corson, ED

J Biol Chem 2004
10922475 M-ABC2, a new human mitochondrial ATP-binding cassette membrane protein

Hogue, DL, Zhang, F, Fisher, CL, Childs, S, Ling, V, Hui, D, Liu, L

FEBS Lett 2000
15225610 The mitochondrial ABC transporter Atm1p functions as a homodimer

LeBard, LM, Reaves, SK, Chloupkov√°, M, Koeller, DM

FEBS Lett 2004
12892562 Stable dimeric assembly of the second membrane-spanning domain of CFTR (cystic fibrosis transmembrane conductance regulator) reconstitutes a chloride-selective pore

Li, C, Ramjeesingh, M, Ugwu, F, Bear, CE, Dhani, S, Wang, Y, Huan, LJ

Biochem J 2003
9878413 Identification and characterization of a mammalian mitochondrial ATP-binding cassette membrane protein

Hogue, DL, Ling, V, Liu, L

J Mol Biol 1999
10196363 Mutation of a putative mitochondrial iron transporter gene (ABC7) in X-linked sideroblastic anemia and ataxia (XLSA/A)

Schueck, ND, Hutchinson, A, Raskind, WH, Allikmets, R, Koeller, DM, Dean, M

Hum Mol Genet 1999
9883897 Identification of a human mitochondrial ABC transporter, the functional orthologue of yeast Atm1p

Kispal, G, Lill, R, Csere, P

FEBS Lett 1998
Catalyst Activity

ABC-type heme transporter activity of Heme transporters [mitochondrial inner membrane]

Orthologous Events
Cross References
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