Reactome | ABC7, mABC1 and mABC2 mediate heme transport

ABC7, mABC1 and mABC2 mediate heme transport

Stable Identifier

R-HSA-382560

Type

Reaction [transition]

Species

Homo sapiens

Compartment

mitochondrial inner membrane, cytosol, mitochondrial matrix

ReviewStatus

5/5

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ABC7, mABC1 and mABC2 mediate heme transport

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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 (Chloupková 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, Corson, ED, Shirihai, OS	J Biol Chem	2004
10922475	M-ABC2, a new human mitochondrial ATP-binding cassette membrane protein Zhang, F, Hogue, DL, Liu, L, Fisher, CL, Hui, D, Childs, S, Ling, V	FEBS Lett	2000
15225610	The mitochondrial ABC transporter Atm1p functions as a homodimer Chloupková, M, Reaves, SK, LeBard, LM, 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 Ramjeesingh, M, Ugwu, F, Li, C, Dhani, S, Huan, LJ, Wang, Y, Bear, CE	Biochem J	2003
9878413	Identification and characterization of a mammalian mitochondrial ATP-binding cassette membrane protein Hogue, DL, Liu, L, Ling, V	J Mol Biol	1999
10196363	Mutation of a putative mitochondrial iron transporter gene (ABC7) in X-linked sideroblastic anemia and ataxia (XLSA/A) Allikmets, R, Raskind, WH, Hutchinson, A, Schueck, ND, Dean, M, Koeller, DM	Hum Mol Genet	1999
9883897	Identification of a human mitochondrial ABC transporter, the functional orthologue of yeast Atm1p Csere, P, Lill, R, Kispal, G	FEBS Lett	1998