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3.A.1.210.4
ABC7 or ABCB7 iron transporter (X-linked sideroblastis anemia protein, XLSA/A (Fujiwara and Harigae 2013)).  Glutathione-complexed [2Fe-2S] stimulates the ATPase activity in both solution and proteoliposome-bound forms (Kd ∼ 68 μM). This cluster is a likely natural substrate for this transporter, which has been implicated in cytosolic Fe-S cluster protein maturation (Qi et al. 2014).

Accession Number:O75027
Protein Name:ABC7 aka ABCB7
Length:752
Molecular Weight:82641.00
Species:Homo sapiens (Human) [9606]
Number of TMSs:5
Location1 / Topology2 / Orientation3: Mitochondrion inner membrane1 / Multi-pass membrane protein2
Substrate Fe2+, FeS-glutathione, FeS-proteoliposome

Cross database links:

Genevestigator: O75027
eggNOG: prNOG18169
RefSeq: NP_004290.2   
Entrez Gene ID: 22   
Pfam: PF00664    PF00005   
OMIM: 300135  gene
301310  phenotype
KEGG: hsa:22   

Gene Ontology

GO:0016021 C:integral to membrane
GO:0005743 C:mitochondrial inner membrane
GO:0005524 F:ATP binding
GO:0042626 F:ATPase activity, coupled to transmembrane m...
GO:0015232 F:heme transporter activity
GO:0055085 P:transmembrane transport

References (9)

[1] “Cloning and chromosomal mapping of a novel ABC transporter gene (hABC7), a candidate for X-linked sideroblastic anemia with spinocerebellar ataxia.”  Shimada Y.et.al.   9621516
[2] “Mutation of a putative mitochondrial iron transporter gene (ABC7) in X-linked sideroblastic anemia and ataxia (XLSA/A).”  Allikmets R.et.al.   10196363
[3] “Human ABC7 transporter: gene structure and mutation causing X-linked sideroblastic anemia with ataxia with disruption of cytosolic iron-sulfur protein maturation.”  Bekri S.et.al.   11050011
[4] “Identification of genes expressed in human CD34(+) hematopoietic stem/progenitor cells by expressed sequence tags and efficient full-length cDNA cloning.”  Mao M.et.al.   9653160
[5] “The DNA sequence of the human X chromosome.”  Ross M.T.et.al.   15772651
[6] “The status, quality, and expansion of the NIH full-length cDNA project: the Mammalian Gene Collection (MGC).”  The MGC Project Teamet.al.   15489334
[7] “Identification of a human mitochondrial ABC transporter, the functional orthologue of yeast Atm1p.”  Csere P.et.al.   9883897
[8] “Lysine acetylation targets protein complexes and co-regulates major cellular functions.”  Choudhary C.et.al.   19608861
[9] “X-linked cerebellar ataxia and sideroblastic anaemia associated with a missense mutation in the ABC7 gene predicting V411L.”  Maguire A.et.al.   11843825

External Searches:

  • Search: DB with
  • BLAST ExPASy (Swiss Institute of Bioinformatics (SIB) BLAST)
  • CDD Search (Conserved Domain Database)
  • Search COGs (Clusters of Orthologous Groups of proteins)
  • 2° Structure (Network Protein Sequence Analysis)

Analyze:

Predict TMSs (Predict number of transmembrane segments)
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FASTA formatted sequence
1:	MALLAMHSWR WAAAAAAFEK RRHSAILIRP LVSVSGSGPQ WRPHQLGALG TARAYQIPES 
61:	LKSITWQRLG KGNSGQFLDA AKALQVWPLI EKRTCWHGHA GGGLHTDPKE GLKDVDTRKI 
121:	IKAMLSYVWP KDRPDLRARV AISLGFLGGA KAMNIVVPFM FKYAVDSLNQ MSGNMLNLSD 
181:	APNTVATMAT AVLIGYGVSR AGAAFFNEVR NAVFGKVAQN SIRRIAKNVF LHLHNLDLGF 
241:	HLSRQTGALS KAIDRGTRGI SFVLSALVFN LLPIMFEVML VSGVLYYKCG AQFALVTLGT 
301:	LGTYTAFTVA VTRWRTRFRI EMNKADNDAG NAAIDSLLNY ETVKYFNNER YEAQRYDGFL 
361:	KTYETASLKS TSTLAMLNFG QSAIFSVGLT AIMVLASQGI VAGTLTVGDL VMVNGLLFQL 
421:	SLPLNFLGTV YRETRQALID MNTLFTLLKV DTQIKDKVMA SPLQITPQTA TVAFDNVHFE 
481:	YIEGQKVLSG ISFEVPAGKK VAIVGGSGSG KSTIVRLLFR FYEPQKGSIY LAGQNIQDVS 
541:	LESLRRAVGV VPQDAVLFHN TIYYNLLYGN ISASPEEVYA VAKLAGLHDA ILRMPHGYDT 
601:	QVGERGLKLS GGEKQRVAIA RAILKDPPVI LYDEATSSLD SITEETILGA MKDVVKHRTS 
661:	IFIAHRLSTV VDADEIIVLD QGKVAERGTH HGLLANPHSI YSEMWHTQSS RVQNHDNPKW 
721:	EAKKENISKE EERKKLQEEI VNSVKGCGNC SC