O00244 (ATOX1_HUMAN) Reviewed, UniProtKB/Swiss-Prot

Names and origin

Protein names

Recommended name:
Copper transport protein ATOX1

Alternative name(s):
Metal transport protein ATX1

Gene names

Name:	ATOX1
Synonyms:	HAH1

Organism

Homo sapiens (Human) [Reference proteome]

Taxonomic identifier

9606 [NCBI]

Taxonomic lineage

cellular organisms › Eukaryota › Opisthokonta › Metazoa › Eumetazoa › Bilateria › Deuterostomia › Chordata › Craniata › Vertebrata › Gnathostomata › Teleostomi › Euteleostomi › Sarcopterygii › Tetrapoda › Amniota › Mammalia › Theria › Eutheria › Euarchontoglires › Primates › Haplorrhini › Simiiformes › Catarrhini › Hominoidea › Hominidae › Homininae › Homo

Protein attributes

Sequence length	68 AA.
Sequence status	Complete.
Protein existence	Evidence at protein level

General annotation (Comments)

Function	Binds and deliver cytosolic copper to the copper ATPase proteins. May be important in cellular antioxidant defense.
Subunit structure	Interacts with ATP7B. Ref.10
Tissue specificity	Ubiquitous.
Sequence similarities	Belongs to the ATX1 family. Contains 1 HMA domain.

Ontologies

Keywords
Biological process	Copper transport Ion transport Transport
Ligand	Copper Metal-binding
Molecular function	Chaperone
Technical term	3D-structure Complete proteome Reference proteome
Gene Ontology (GO)
Biological_process	cellular copper ion homeostasis Traceable author statement PubMed 9430722. Source: ProtInc copper ion transport Traceable author statement PubMed 15670166. Source: UniProtKB response to oxidative stress Traceable author statement PubMed 9430722. Source: ProtInc
Cellular_component	cytosol Traceable author statement PubMed 12029094. Source: UniProtKB
Molecular_function	copper chaperone activity Inferred from direct assay PubMed 12029094. Source: UniProtKB
Complete GO annotation...

Sequence annotation (Features)

Feature key

Position(s)

Length

Description

Graphical view

Feature identifier

Molecule processing

Chain

1 – 68

68

Copper transport protein ATOX1

PRO_0000212537

Regions

Domain

2 – 68

67

HMA

Sites

Metal binding

12

1

Copper

Metal binding

15

1

Copper

Secondary structure

1

.

.

.

.

.

.

.

.

.

.

.

.

.

.

68

Helix Strand Turn

Details...

Beta strand

3 – 8

6

Helix

13 – 26

14

Beta strand

28 – 34

7

Turn

35 – 38

4

Beta strand

39 – 46

8

Helix

48 – 56

9

Turn

57 – 59

3

Beta strand

62 – 66

5

Sequences

	Sequence		Length	Mass (Da)
	O00244 [UniParc]. Last modified July 1, 1997. Version 1. Checksum: 0B364B1BD1279314	FASTA	68	7,402
	10 20 30 40 50 60 MPKHEFSVDM TCGGCAEAVS RVLNKLGGVK YDIDLPNKKV CIESEHSMDT LLATLKKTGK TVSYLGLE

References

	« Hide 'large scale' referencesShow 'large scale' references »
[1]	"Identification and functional expression of HAH1, a novel human gene involved in copper homeostasis." Klomp L.W.J., Lin S.-J., Yuan D.S., Klausner R.D., Culotta V.C., Gitlin J.D. J. Biol. Chem. 272:9221-9226(1997) [PubMed] [Europe PMC] [Abstract] Cited for: NUCLEOTIDE SEQUENCE [MRNA]. Tissue: Liver.
[2]	"Genomic organization of ATOX1, a human copper chaperone." Liu P.-C., Koeller D.M., Kaler S.G. BMC Genet. 4:4-4(2003) [PubMed] [Europe PMC] [Abstract] Cited for: NUCLEOTIDE SEQUENCE [GENOMIC DNA].
[3]	"Cloning of human full-length CDSs in BD Creator(TM) system donor vector." Kalnine N., Chen X., Rolfs A., Halleck A., Hines L., Eisenstein S., Koundinya M., Raphael J., Moreira D., Kelley T., LaBaer J., Lin Y., Phelan M., Farmer A. Submitted (AUG-2003) to the EMBL/GenBank/DDBJ databases Cited for: NUCLEOTIDE SEQUENCE [LARGE SCALE MRNA].
[4]	"Complete sequencing and characterization of 21,243 full-length human cDNAs." Ota T., Suzuki Y., Nishikawa T., Otsuki T., Sugiyama T., Irie R., Wakamatsu A., Hayashi K., Sato H., Nagai K., Kimura K., Makita H., Sekine M., Obayashi M., Nishi T., Shibahara T., Tanaka T., Ishii S. , Yamamoto J., Saito K., Kawai Y., Isono Y., Nakamura Y., Nagahari K., Murakami K., Yasuda T., Iwayanagi T., Wagatsuma M., Shiratori A., Sudo H., Hosoiri T., Kaku Y., Kodaira H., Kondo H., Sugawara M., Takahashi M., Kanda K., Yokoi T., Furuya T., Kikkawa E., Omura Y., Abe K., Kamihara K., Katsuta N., Sato K., Tanikawa M., Yamazaki M., Ninomiya K., Ishibashi T., Yamashita H., Murakawa K., Fujimori K., Tanai H., Kimata M., Watanabe M., Hiraoka S., Chiba Y., Ishida S., Ono Y., Takiguchi S., Watanabe S., Yosida M., Hotuta T., Kusano J., Kanehori K., Takahashi-Fujii A., Hara H., Tanase T.-O., Nomura Y., Togiya S., Komai F., Hara R., Takeuchi K., Arita M., Imose N., Musashino K., Yuuki H., Oshima A., Sasaki N., Aotsuka S., Yoshikawa Y., Matsunawa H., Ichihara T., Shiohata N., Sano S., Moriya S., Momiyama H., Satoh N., Takami S., Terashima Y., Suzuki O., Nakagawa S., Senoh A., Mizoguchi H., Goto Y., Shimizu F., Wakebe H., Hishigaki H., Watanabe T., Sugiyama A., Takemoto M., Kawakami B., Yamazaki M., Watanabe K., Kumagai A., Itakura S., Fukuzumi Y., Fujimori Y., Komiyama M., Tashiro H., Tanigami A., Fujiwara T., Ono T., Yamada K., Fujii Y., Ozaki K., Hirao M., Ohmori Y., Kawabata A., Hikiji T., Kobatake N., Inagaki H., Ikema Y., Okamoto S., Okitani R., Kawakami T., Noguchi S., Itoh T., Shigeta K., Senba T., Matsumura K., Nakajima Y., Mizuno T., Morinaga M., Sasaki M., Togashi T., Oyama M., Hata H., Watanabe M., Komatsu T., Mizushima-Sugano J., Satoh T., Shirai Y., Takahashi Y., Nakagawa K., Okumura K., Nagase T., Nomura N., Kikuchi H., Masuho Y., Yamashita R., Nakai K., Yada T., Nakamura Y., Ohara O., Isogai T., Sugano S. Nat. Genet. 36:40-45(2004) [PubMed] [Europe PMC] [Abstract] Cited for: NUCLEOTIDE SEQUENCE [LARGE SCALE MRNA]. Tissue: Uterus.
[5]	NIEHS SNPs program Submitted (MAR-2005) to the EMBL/GenBank/DDBJ databases Cited for: NUCLEOTIDE SEQUENCE [GENOMIC DNA].
[6]	Mural R.J., Istrail S., Sutton G.G., Florea L., Halpern A.L., Mobarry C.M., Lippert R., Walenz B., Shatkay H., Dew I., Miller J.R., Flanigan M.J., Edwards N.J., Bolanos R., Fasulo D., Halldorsson B.V., Hannenhalli S., Turner R. , Yooseph S., Lu F., Nusskern D.R., Shue B.C., Zheng X.H., Zhong F., Delcher A.L., Huson D.H., Kravitz S.A., Mouchard L., Reinert K., Remington K.A., Clark A.G., Waterman M.S., Eichler E.E., Adams M.D., Hunkapiller M.W., Myers E.W., Venter J.C. Submitted (SEP-2005) to the EMBL/GenBank/DDBJ databases Cited for: NUCLEOTIDE SEQUENCE [LARGE SCALE GENOMIC DNA].
[7]	"The status, quality, and expansion of the NIH full-length cDNA project: the Mammalian Gene Collection (MGC)." The MGC Project Team Genome Res. 14:2121-2127(2004) [PubMed] [Europe PMC] [Abstract] Cited for: NUCLEOTIDE SEQUENCE [LARGE SCALE MRNA].
[8]	"Lysine acetylation targets protein complexes and co-regulates major cellular functions." Choudhary C., Kumar C., Gnad F., Nielsen M.L., Rehman M., Walther T.C., Olsen J.V., Mann M. Science 325:834-840(2009) [PubMed] [Europe PMC] [Abstract] Cited for: IDENTIFICATION BY MASS SPECTROMETRY [LARGE SCALE ANALYSIS].
[9]	"Initial characterization of the human central proteome." Burkard T.R., Planyavsky M., Kaupe I., Breitwieser F.P., Buerckstuemmer T., Bennett K.L., Superti-Furga G., Colinge J. BMC Syst. Biol. 5:17-17(2011) [PubMed] [Europe PMC] [Abstract] Cited for: IDENTIFICATION BY MASS SPECTROMETRY [LARGE SCALE ANALYSIS].
[10]	"Structural basis for copper transfer by the metallochaperone for the Menkes/Wilson disease proteins." Wernimont A.K., Huffman D.L., Lamb A.L., O'Halloran T.V., Rosenzweig A.C. Nat. Struct. Biol. 7:766-771(2000) [PubMed] [Europe PMC] [Abstract] Cited for: X-RAY CRYSTALLOGRAPHY (1.75 ANGSTROMS), COPPER-BINDING SITES, INTERACTION WITH ATP7B.
+	Additional computationally mapped references.

Web resources

NIEHS-SNPs

Cross-references

Sequence databases

EMBL
GenBank
DDBJ

U70660 mRNA. Translation: AAC51227.1.
AY165037 Genomic DNA. Translation: AAN84554.1.
BT009786 mRNA. Translation: AAP88788.1.
AK293063 mRNA. Translation: BAF85752.1.
AY986502 Genomic DNA. Translation: AAX81411.1.
CH471062 Genomic DNA. Translation: EAW61663.1.
CH471062 Genomic DNA. Translation: EAW61664.1.
BC112248 mRNA. Translation: AAI12249.1.
BC112250 mRNA. Translation: AAI12251.1.

IPI

IPI00010863.

RefSeq

NP_004036.1. NM_004045.3.

UniGene

Hs.125213.

3D structure databases

PDBe
RCSB PDB
PDBj

Entry	Method	Resolution (Å)	Chain	Positions	PDBsum
1FE0	X-ray	1.75	A/B	1-68	[»]
1FE4	X-ray	1.75	A/B	1-68	[»]
1FEE	X-ray	1.80	A/B	1-68	[»]
1TL4	NMR	-	A	1-68	[»]
1TL5	NMR	-	A	1-68	[»]
2K1R	NMR	-	B	1-68	[»]
3CJK	X-ray	1.80	A	2-68	[»]
3IWL	X-ray	1.60	A	1-68	[»]
3IWX	X-ray	2.14	A/B	1-68	[»]

ProteinModelPortal

O00244.

ModBase

Search...

Protein-protein interaction databases

STRING

9606.ENSP00000316854.

PTM databases

PhosphoSite

O00244.

Proteomic databases

PaxDb

O00244.

PeptideAtlas

O00244.

PRIDE

O00244.

Protocols and materials databases

DNASU

475.

StructuralBiologyKnowledgebase

Search...

Genome annotation databases

Ensembl

ENST00000313115; ENSP00000316854; ENSG00000177556.
ENST00000522710; ENSP00000429814; ENSG00000177556.
ENST00000524142; ENSP00000430598; ENSG00000177556.

GeneID

475.

KEGG

hsa:475.

UCSC

uc003luk.3. human.

Organism-specific databases

CTD

475.

GeneCards

GC05M151102.

HGNC

HGNC:798. ATOX1.

HPA

HPA055036.

MIM

602270. gene.

neXtProt

NX_O00244.

PharmGKB

PA25096.

GenAtlas

Search...

Phylogenomic databases

eggNOG

NOG238748.

HOGENOM

HOG000038877.

HOVERGEN

HBG050610.

InParanoid

O00244.

KO

K07213.

OrthoDB

EOG45TCPV.

PhylomeDB

O00244.

Gene expression databases

ArrayExpress

O00244.

Bgee

O00244.

CleanEx

HS_ATOX1.

Genevestigator

O00244.

GermOnline

ENSG00000177556. Homo sapiens.

Family and domain databases

InterPro

IPR017969. Heavy-metal-associated_CS.
IPR006121. HeavyMe-assoc_HMA.
[Graphical view]

Pfam

PF00403. HMA. 1 hit.
[Graphical view]

SUPFAM

SSF55008. HeavyMe_transpt. 1 hit.

PROSITE

PS01047. HMA_1. 1 hit.
PS50846. HMA_2. 1 hit.
[Graphical view]

ProtoNet

Search...

Other

ChiTaRS

ATOX1. human.

EvolutionaryTrace

O00244.

GenomeRNAi

475.

NextBio

1967.

SOURCE

Search...

Entry information

Entry name

ATOX1_HUMAN

Accession

Primary (citable) accession number: O00244
Secondary accession number(s): A8KAJ8 , D3DQI2, Q2M1R6, Q56AP3

Entry history

Integrated into UniProtKB/Swiss-Prot:	May 30, 2000
Last sequence update:	July 1, 1997
Last modified:	May 1, 2013
This is version 115 of the entry and version 1 of the sequence. [Complete history]

Entry status

Reviewed (UniProtKB/Swiss-Prot)

Annotation program

Chordata Protein Annotation Program

Disclaimer

Any medical or genetic information present in this entry is provided for research, educational and informational purposes only. It is not in any way intended to be used as a substitute for professional medical advice, diagnosis, treatment or care.