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SummaryStructural detailsExperimental detailsFunctional detailsSequence NeighborHistoryDownloads

6FON

Elongated conformer of the human copper chaperone for SOD1 complexed with human SOD1

Functional Information from GO Data
ChainGOidnamespacecontents
A0005507molecular_functioncopper ion binding
A0005515molecular_functionprotein binding
A0005634cellular_componentnucleus
A0005737cellular_componentcytoplasm
A0005739cellular_componentmitochondrion
A0005829cellular_componentcytosol
A0006801biological_processsuperoxide metabolic process
A0015035molecular_functionprotein-disulfide reductase activity
A0016532molecular_functionsuperoxide dismutase copper chaperone activity
A0019430biological_processremoval of superoxide radicals
A0034599biological_processcellular response to oxidative stress
A0045296molecular_functioncadherin binding
A0046872molecular_functionmetal ion binding
A0051604biological_processprotein maturation
B0000303biological_processresponse to superoxide
B0001541biological_processovarian follicle development
B0001819biological_processpositive regulation of cytokine production
B0001890biological_processplacenta development
B0001895biological_processretina homeostasis
B0001975biological_processresponse to amphetamine
B0002262biological_processmyeloid cell homeostasis
B0004784molecular_functionsuperoxide dismutase activity
B0005507molecular_functioncopper ion binding
B0005515molecular_functionprotein binding
B0005576cellular_componentextracellular region
B0005615cellular_componentextracellular space
B0005634cellular_componentnucleus
B0005654cellular_componentnucleoplasm
B0005737cellular_componentcytoplasm
B0005739cellular_componentmitochondrion
B0005758cellular_componentmitochondrial intermembrane space
B0005759cellular_componentmitochondrial matrix
B0005764cellular_componentlysosome
B0005777cellular_componentperoxisome
B0005829cellular_componentcytosol
B0005886cellular_componentplasma membrane
B0006749biological_processglutathione metabolic process
B0006801biological_processsuperoxide metabolic process
B0006879biological_processintracellular iron ion homeostasis
B0006979biological_processresponse to oxidative stress
B0007283biological_processspermatogenesis
B0007566biological_processembryo implantation
B0007605biological_processsensory perception of sound
B0007626biological_processlocomotory behavior
B0008089biological_processanterograde axonal transport
B0008090biological_processretrograde axonal transport
B0008217biological_processregulation of blood pressure
B0008270molecular_functionzinc ion binding
B0009408biological_processresponse to heat
B0016209molecular_functionantioxidant activity
B0016491molecular_functionoxidoreductase activity
B0019226biological_processtransmission of nerve impulse
B0019430biological_processremoval of superoxide radicals
B0019899molecular_functionenzyme binding
B0030141cellular_componentsecretory granule
B0030346molecular_functionprotein phosphatase 2B binding
B0031045cellular_componentdense core granule
B0031267molecular_functionsmall GTPase binding
B0031410cellular_componentcytoplasmic vesicle
B0031667biological_processresponse to nutrient levels
B0032287biological_processperipheral nervous system myelin maintenance
B0032839cellular_componentdendrite cytoplasm
B0032930biological_processpositive regulation of superoxide anion generation
B0032991cellular_componentprotein-containing complex
B0033081biological_processregulation of T cell differentiation in thymus
B0034465biological_processresponse to carbon monoxide
B0034599biological_processcellular response to oxidative stress
B0035865biological_processcellular response to potassium ion
B0040014biological_processregulation of multicellular organism growth
B0042542biological_processresponse to hydrogen peroxide
B0042802molecular_functionidentical protein binding
B0042803molecular_functionprotein homodimerization activity
B0043025cellular_componentneuronal cell body
B0043065biological_processpositive regulation of apoptotic process
B0043066biological_processnegative regulation of apoptotic process
B0043087biological_processregulation of GTPase activity
B0043410biological_processpositive regulation of MAPK cascade
B0043524biological_processnegative regulation of neuron apoptotic process
B0045471biological_processresponse to ethanol
B0046620biological_processregulation of organ growth
B0046688biological_processresponse to copper ion
B0046716biological_processmuscle cell cellular homeostasis
B0046872molecular_functionmetal ion binding
B0048538biological_processthymus development
B0048678biological_processresponse to axon injury
B0050665biological_processhydrogen peroxide biosynthetic process
B0050728biological_processnegative regulation of inflammatory response
B0050766biological_processpositive regulation of phagocytosis
B0051087molecular_functionprotein-folding chaperone binding
B0051881biological_processregulation of mitochondrial membrane potential
B0060047biological_processheart contraction
B0060052biological_processneurofilament cytoskeleton organization
B0060087biological_processrelaxation of vascular associated smooth muscle
B0060088biological_processauditory receptor cell stereocilium organization
B0070062cellular_componentextracellular exosome
B0071276biological_processcellular response to cadmium ion
B0071318biological_processcellular response to ATP
B0072593biological_processreactive oxygen species metabolic process
B0097332biological_processresponse to antipsychotic drug
B1902177biological_processpositive regulation of oxidative stress-induced intrinsic apoptotic signaling pathway
B1904115cellular_componentaxon cytoplasm
C0005507molecular_functioncopper ion binding
C0005515molecular_functionprotein binding
C0005634cellular_componentnucleus
C0005737cellular_componentcytoplasm
C0005739cellular_componentmitochondrion
C0005829cellular_componentcytosol
C0006801biological_processsuperoxide metabolic process
C0015035molecular_functionprotein-disulfide reductase activity
C0016532molecular_functionsuperoxide dismutase copper chaperone activity
C0019430biological_processremoval of superoxide radicals
C0034599biological_processcellular response to oxidative stress
C0045296molecular_functioncadherin binding
C0046872molecular_functionmetal ion binding
C0051604biological_processprotein maturation
D0000303biological_processresponse to superoxide
D0001541biological_processovarian follicle development
D0001819biological_processpositive regulation of cytokine production
D0001890biological_processplacenta development
D0001895biological_processretina homeostasis
D0001975biological_processresponse to amphetamine
D0002262biological_processmyeloid cell homeostasis
D0004784molecular_functionsuperoxide dismutase activity
D0005507molecular_functioncopper ion binding
D0005515molecular_functionprotein binding
D0005576cellular_componentextracellular region
D0005615cellular_componentextracellular space
D0005634cellular_componentnucleus
D0005654cellular_componentnucleoplasm
D0005737cellular_componentcytoplasm
D0005739cellular_componentmitochondrion
D0005758cellular_componentmitochondrial intermembrane space
D0005759cellular_componentmitochondrial matrix
D0005764cellular_componentlysosome
D0005777cellular_componentperoxisome
D0005829cellular_componentcytosol
D0005886cellular_componentplasma membrane
D0006749biological_processglutathione metabolic process
D0006801biological_processsuperoxide metabolic process
D0006879biological_processintracellular iron ion homeostasis
D0006979biological_processresponse to oxidative stress
D0007283biological_processspermatogenesis
D0007566biological_processembryo implantation
D0007605biological_processsensory perception of sound
D0007626biological_processlocomotory behavior
D0008089biological_processanterograde axonal transport
D0008090biological_processretrograde axonal transport
D0008217biological_processregulation of blood pressure
D0008270molecular_functionzinc ion binding
D0009408biological_processresponse to heat
D0016209molecular_functionantioxidant activity
D0016491molecular_functionoxidoreductase activity
D0019226biological_processtransmission of nerve impulse
D0019430biological_processremoval of superoxide radicals
D0019899molecular_functionenzyme binding
D0030141cellular_componentsecretory granule
D0030346molecular_functionprotein phosphatase 2B binding
D0031045cellular_componentdense core granule
D0031267molecular_functionsmall GTPase binding
D0031410cellular_componentcytoplasmic vesicle
D0031667biological_processresponse to nutrient levels
D0032287biological_processperipheral nervous system myelin maintenance
D0032839cellular_componentdendrite cytoplasm
D0032930biological_processpositive regulation of superoxide anion generation
D0032991cellular_componentprotein-containing complex
D0033081biological_processregulation of T cell differentiation in thymus
D0034465biological_processresponse to carbon monoxide
D0034599biological_processcellular response to oxidative stress
D0035865biological_processcellular response to potassium ion
D0040014biological_processregulation of multicellular organism growth
D0042542biological_processresponse to hydrogen peroxide
D0042802molecular_functionidentical protein binding
D0042803molecular_functionprotein homodimerization activity
D0043025cellular_componentneuronal cell body
D0043065biological_processpositive regulation of apoptotic process
D0043066biological_processnegative regulation of apoptotic process
D0043087biological_processregulation of GTPase activity
D0043410biological_processpositive regulation of MAPK cascade
D0043524biological_processnegative regulation of neuron apoptotic process
D0045471biological_processresponse to ethanol
D0046620biological_processregulation of organ growth
D0046688biological_processresponse to copper ion
D0046716biological_processmuscle cell cellular homeostasis
D0046872molecular_functionmetal ion binding
D0048538biological_processthymus development
D0048678biological_processresponse to axon injury
D0050665biological_processhydrogen peroxide biosynthetic process
D0050728biological_processnegative regulation of inflammatory response
D0050766biological_processpositive regulation of phagocytosis
D0051087molecular_functionprotein-folding chaperone binding
D0051881biological_processregulation of mitochondrial membrane potential
D0060047biological_processheart contraction
D0060052biological_processneurofilament cytoskeleton organization
D0060087biological_processrelaxation of vascular associated smooth muscle
D0060088biological_processauditory receptor cell stereocilium organization
D0070062cellular_componentextracellular exosome
D0071276biological_processcellular response to cadmium ion
D0071318biological_processcellular response to ATP
D0072593biological_processreactive oxygen species metabolic process
D0097332biological_processresponse to antipsychotic drug
D1902177biological_processpositive regulation of oxidative stress-induced intrinsic apoptotic signaling pathway
D1904115cellular_componentaxon cytoplasm
Functional Information from PDB Data
site_idAC1
Number of Residues4
Detailsbinding site for residue ZN A 501
ChainResidue
AHIS147
AHIS155
AHIS164
AASP167
site_idAC2
Number of Residues4
Detailsbinding site for residue ZN B 201
ChainResidue
BHIS63
BHIS71
BHIS80
BASP83
site_idAC3
Number of Residues4
Detailsbinding site for residue ZN C 301
ChainResidue
CHIS155
CHIS164
CASP167
CHIS147
site_idAC4
Number of Residues5
Detailsbinding site for residue ZN D 201
ChainResidue
DHIS63
DHIS71
DHIS80
DASP83
DLYS136
Functional Information from PROSITE/UniProt
site_idPS00087
Number of Residues11
DetailsSOD_CU_ZN_1 Copper/Zinc superoxide dismutase signature 1. GFHVHEfGDnT
ChainResidueDetails
BGLY44-THR54
site_idPS00332
Number of Residues12
DetailsSOD_CU_ZN_2 Copper/Zinc superoxide dismutase signature 2. GNSGeRlACgiI
ChainResidueDetails
AGLY219-ILE230
Functional Information from SwissProt/UniProt
site_idSWS_FT_FI1
Number of Residues126
DetailsDomain: {"description":"HMA","evidences":[{"source":"PROSITE-ProRule","id":"PRU00280","evidenceCode":"ECO:0000255"}]}
ChainResidueDetails
site_idSWS_FT_FI2
Number of Residues292
DetailsRegion: {"description":"Superoxide dismutase-like"}
ChainResidueDetails
site_idSWS_FT_FI3
Number of Residues4
DetailsBinding site: {"evidences":[{"source":"PROSITE-ProRule","id":"PRU00280","evidenceCode":"ECO:0000255"},{"source":"PDB","id":"2RSQ","evidenceCode":"ECO:0007744"}]}
ChainResidueDetails
site_idSWS_FT_FI4
Number of Residues8
DetailsBinding site: {"evidences":[{"source":"PubMed","id":"10677207","evidenceCode":"ECO:0000269"},{"source":"PDB","id":"1DO5","evidenceCode":"ECO:0007744"}]}
ChainResidueDetails
site_idSWS_FT_FI5
Number of Residues4
DetailsBinding site: {}
ChainResidueDetails
site_idSWS_FT_FI6
Number of Residues10
DetailsCross-link: {"description":"Glycyl lysine isopeptide (Lys-Gly) (interchain with G-Cter in ubiquitin)","evidences":[{"source":"PubMed","id":"20154138","evidenceCode":"ECO:0000269"}]}
ChainResidueDetails
site_idSWS_FT_FI7
Number of Residues6
DetailsBinding site: {"evidences":[{"source":"PubMed","id":"12963370","evidenceCode":"ECO:0000269"},{"source":"PubMed","id":"17548825","evidenceCode":"ECO:0000269"}]}
ChainResidueDetails
site_idSWS_FT_FI8
Number of Residues8
DetailsBinding site: {"evidences":[{"source":"PubMed","id":"20727846","evidenceCode":"ECO:0000269"}]}
ChainResidueDetails
site_idSWS_FT_FI9
Number of Residues2
DetailsModified residue: {"description":"N-acetylalanine","evidences":[{"source":"PubMed","id":"1463506","evidenceCode":"ECO:0000269"},{"source":"PubMed","id":"7002610","evidenceCode":"ECO:0000269"},{"source":"PubMed","id":"25944712","evidenceCode":"ECO:0007744"}]}
ChainResidueDetails
site_idSWS_FT_FI10
Number of Residues6
DetailsModified residue: {"description":"N6-succinyllysine","evidences":[{"source":"UniProtKB","id":"P08228","evidenceCode":"ECO:0000250"}]}
ChainResidueDetails
site_idSWS_FT_FI11
Number of Residues2
DetailsModified residue: {"description":"Phosphoserine","evidences":[{"source":"PubMed","id":"18669648","evidenceCode":"ECO:0007744"},{"source":"PubMed","id":"20068231","evidenceCode":"ECO:0007744"},{"source":"PubMed","id":"24275569","evidenceCode":"ECO:0007744"}]}
ChainResidueDetails
site_idSWS_FT_FI12
Number of Residues2
DetailsModified residue: {"description":"Phosphoserine","evidences":[{"source":"PubMed","id":"24275569","evidenceCode":"ECO:0007744"}]}
ChainResidueDetails
site_idSWS_FT_FI13
Number of Residues2
DetailsModified residue: {"description":"Phosphoserine","evidences":[{"source":"UniProtKB","id":"P07632","evidenceCode":"ECO:0000250"}]}
ChainResidueDetails
site_idSWS_FT_FI14
Number of Residues2
DetailsModified residue: {"description":"Phosphoserine","evidences":[{"source":"UniProtKB","id":"P08228","evidenceCode":"ECO:0000250"}]}
ChainResidueDetails
site_idSWS_FT_FI15
Number of Residues2
DetailsModified residue: {"description":"N6-succinyllysine; alternate","evidences":[{"source":"PubMed","id":"24140062","evidenceCode":"ECO:0000269"}]}
ChainResidueDetails
site_idSWS_FT_FI16
Number of Residues2
DetailsModified residue: {"description":"N6-succinyllysine; alternate","evidences":[{"source":"UniProtKB","id":"P08228","evidenceCode":"ECO:0000250"}]}
ChainResidueDetails
site_idSWS_FT_FI17
Number of Residues2
DetailsLipidation: {"description":"S-palmitoyl cysteine","evidences":[{"source":"PubMed","id":"22496122","evidenceCode":"ECO:0000269"}]}
ChainResidueDetails
site_idSWS_FT_FI18
Number of Residues4
DetailsCross-link: {"description":"1-(tryptophan-3-yl)-tryptophan (Trp-Trp) (interchain with W-33)","evidences":[{"source":"PubMed","id":"20600836","evidenceCode":"ECO:0000269"}]}
ChainResidueDetails

250059

PDB entries from 2026-03-04

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