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1P1V

Crystal Structure of FALS-associated human Copper-Zinc Superoxide Dismutase (CuZnSOD) Mutant D125H to 1.4A

Functional Information from GO Data
ChainGOidnamespacecontents
A0000302biological_processresponse to reactive oxygen species
A0000303biological_processresponse to superoxide
A0001541biological_processovarian follicle development
A0001819biological_processpositive regulation of cytokine production
A0001890biological_processplacenta development
A0001895biological_processretina homeostasis
A0002262biological_processmyeloid cell homeostasis
A0004784molecular_functionsuperoxide dismutase activity
A0005507molecular_functioncopper ion binding
A0005515molecular_functionprotein binding
A0005576cellular_componentextracellular region
A0005615cellular_componentextracellular space
A0005634cellular_componentnucleus
A0005654cellular_componentnucleoplasm
A0005737cellular_componentcytoplasm
A0005739cellular_componentmitochondrion
A0005758cellular_componentmitochondrial intermembrane space
A0005759cellular_componentmitochondrial matrix
A0005777cellular_componentperoxisome
A0005829cellular_componentcytosol
A0005886cellular_componentplasma membrane
A0006749biological_processglutathione metabolic process
A0006801biological_processsuperoxide metabolic process
A0006879biological_processintracellular iron ion homeostasis
A0006915biological_processapoptotic process
A0006979biological_processresponse to oxidative stress
A0007283biological_processspermatogenesis
A0007566biological_processembryo implantation
A0007605biological_processsensory perception of sound
A0007626biological_processlocomotory behavior
A0008089biological_processanterograde axonal transport
A0008090biological_processretrograde axonal transport
A0008217biological_processregulation of blood pressure
A0008270molecular_functionzinc ion binding
A0008340biological_processdetermination of adult lifespan
A0009408biological_processresponse to heat
A0009410biological_processresponse to xenobiotic stimulus
A0010467biological_processgene expression
A0016209molecular_functionantioxidant activity
A0016491molecular_functionoxidoreductase activity
A0019226biological_processtransmission of nerve impulse
A0019228biological_processneuronal action potential
A0019430biological_processremoval of superoxide radicals
A0030346molecular_functionprotein phosphatase 2B binding
A0031267molecular_functionsmall GTPase binding
A0031410cellular_componentcytoplasmic vesicle
A0032287biological_processperipheral nervous system myelin maintenance
A0032839cellular_componentdendrite cytoplasm
A0032930biological_processpositive regulation of superoxide anion generation
A0032991cellular_componentprotein-containing complex
A0033081biological_processregulation of T cell differentiation in thymus
A0035234biological_processectopic germ cell programmed cell death
A0040014biological_processregulation of multicellular organism growth
A0042542biological_processresponse to hydrogen peroxide
A0042554biological_processsuperoxide anion generation
A0042802molecular_functionidentical protein binding
A0042803molecular_functionprotein homodimerization activity
A0043025cellular_componentneuronal cell body
A0043065biological_processpositive regulation of apoptotic process
A0043066biological_processnegative regulation of apoptotic process
A0043087biological_processregulation of GTPase activity
A0043410biological_processpositive regulation of MAPK cascade
A0043524biological_processnegative regulation of neuron apoptotic process
A0045471biological_processresponse to ethanol
A0046620biological_processregulation of organ growth
A0046716biological_processmuscle cell cellular homeostasis
A0046872molecular_functionmetal ion binding
A0048538biological_processthymus development
A0048678biological_processresponse to axon injury
A0050665biological_processhydrogen peroxide biosynthetic process
A0050728biological_processnegative regulation of inflammatory response
A0050766biological_processpositive regulation of phagocytosis
A0051087molecular_functionprotein-folding chaperone binding
A0051093biological_processnegative regulation of developmental process
A0051881biological_processregulation of mitochondrial membrane potential
A0060047biological_processheart contraction
A0060052biological_processneurofilament cytoskeleton organization
A0060087biological_processrelaxation of vascular associated smooth muscle
A0060088biological_processauditory receptor cell stereocilium organization
A0070062cellular_componentextracellular exosome
A0072593biological_processreactive oxygen species metabolic process
A0099610biological_processaction potential initiation
A1902177biological_processpositive regulation of oxidative stress-induced intrinsic apoptotic signaling pathway
A1904115cellular_componentaxon cytoplasm
A2000242biological_processnegative regulation of reproductive process
B0000302biological_processresponse to reactive oxygen species
B0000303biological_processresponse to superoxide
B0001541biological_processovarian follicle development
B0001819biological_processpositive regulation of cytokine production
B0001890biological_processplacenta development
B0001895biological_processretina homeostasis
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
B0005777cellular_componentperoxisome
B0005829cellular_componentcytosol
B0005886cellular_componentplasma membrane
B0006749biological_processglutathione metabolic process
B0006801biological_processsuperoxide metabolic process
B0006879biological_processintracellular iron ion homeostasis
B0006915biological_processapoptotic process
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
B0008340biological_processdetermination of adult lifespan
B0009408biological_processresponse to heat
B0009410biological_processresponse to xenobiotic stimulus
B0010467biological_processgene expression
B0016209molecular_functionantioxidant activity
B0016491molecular_functionoxidoreductase activity
B0019226biological_processtransmission of nerve impulse
B0019228biological_processneuronal action potential
B0019430biological_processremoval of superoxide radicals
B0030346molecular_functionprotein phosphatase 2B binding
B0031267molecular_functionsmall GTPase binding
B0031410cellular_componentcytoplasmic vesicle
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
B0035234biological_processectopic germ cell programmed cell death
B0040014biological_processregulation of multicellular organism growth
B0042542biological_processresponse to hydrogen peroxide
B0042554biological_processsuperoxide anion generation
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
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
B0051093biological_processnegative regulation of developmental process
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
B0072593biological_processreactive oxygen species metabolic process
B0099610biological_processaction potential initiation
B1902177biological_processpositive regulation of oxidative stress-induced intrinsic apoptotic signaling pathway
B1904115cellular_componentaxon cytoplasm
B2000242biological_processnegative regulation of reproductive process
C0000302biological_processresponse to reactive oxygen species
C0000303biological_processresponse to superoxide
C0001541biological_processovarian follicle development
C0001819biological_processpositive regulation of cytokine production
C0001890biological_processplacenta development
C0001895biological_processretina homeostasis
C0002262biological_processmyeloid cell homeostasis
C0004784molecular_functionsuperoxide dismutase activity
C0005507molecular_functioncopper ion binding
C0005515molecular_functionprotein binding
C0005576cellular_componentextracellular region
C0005615cellular_componentextracellular space
C0005634cellular_componentnucleus
C0005654cellular_componentnucleoplasm
C0005737cellular_componentcytoplasm
C0005739cellular_componentmitochondrion
C0005758cellular_componentmitochondrial intermembrane space
C0005759cellular_componentmitochondrial matrix
C0005777cellular_componentperoxisome
C0005829cellular_componentcytosol
C0005886cellular_componentplasma membrane
C0006749biological_processglutathione metabolic process
C0006801biological_processsuperoxide metabolic process
C0006879biological_processintracellular iron ion homeostasis
C0006915biological_processapoptotic process
C0006979biological_processresponse to oxidative stress
C0007283biological_processspermatogenesis
C0007566biological_processembryo implantation
C0007605biological_processsensory perception of sound
C0007626biological_processlocomotory behavior
C0008089biological_processanterograde axonal transport
C0008090biological_processretrograde axonal transport
C0008217biological_processregulation of blood pressure
C0008270molecular_functionzinc ion binding
C0008340biological_processdetermination of adult lifespan
C0009408biological_processresponse to heat
C0009410biological_processresponse to xenobiotic stimulus
C0010467biological_processgene expression
C0016209molecular_functionantioxidant activity
C0016491molecular_functionoxidoreductase activity
C0019226biological_processtransmission of nerve impulse
C0019228biological_processneuronal action potential
C0019430biological_processremoval of superoxide radicals
C0030346molecular_functionprotein phosphatase 2B binding
C0031267molecular_functionsmall GTPase binding
C0031410cellular_componentcytoplasmic vesicle
C0032287biological_processperipheral nervous system myelin maintenance
C0032839cellular_componentdendrite cytoplasm
C0032930biological_processpositive regulation of superoxide anion generation
C0032991cellular_componentprotein-containing complex
C0033081biological_processregulation of T cell differentiation in thymus
C0035234biological_processectopic germ cell programmed cell death
C0040014biological_processregulation of multicellular organism growth
C0042542biological_processresponse to hydrogen peroxide
C0042554biological_processsuperoxide anion generation
C0042802molecular_functionidentical protein binding
C0042803molecular_functionprotein homodimerization activity
C0043025cellular_componentneuronal cell body
C0043065biological_processpositive regulation of apoptotic process
C0043066biological_processnegative regulation of apoptotic process
C0043087biological_processregulation of GTPase activity
C0043410biological_processpositive regulation of MAPK cascade
C0043524biological_processnegative regulation of neuron apoptotic process
C0045471biological_processresponse to ethanol
C0046620biological_processregulation of organ growth
C0046716biological_processmuscle cell cellular homeostasis
C0046872molecular_functionmetal ion binding
C0048538biological_processthymus development
C0048678biological_processresponse to axon injury
C0050665biological_processhydrogen peroxide biosynthetic process
C0050728biological_processnegative regulation of inflammatory response
C0050766biological_processpositive regulation of phagocytosis
C0051087molecular_functionprotein-folding chaperone binding
C0051093biological_processnegative regulation of developmental process
C0051881biological_processregulation of mitochondrial membrane potential
C0060047biological_processheart contraction
C0060052biological_processneurofilament cytoskeleton organization
C0060087biological_processrelaxation of vascular associated smooth muscle
C0060088biological_processauditory receptor cell stereocilium organization
C0070062cellular_componentextracellular exosome
C0072593biological_processreactive oxygen species metabolic process
C0099610biological_processaction potential initiation
C1902177biological_processpositive regulation of oxidative stress-induced intrinsic apoptotic signaling pathway
C1904115cellular_componentaxon cytoplasm
C2000242biological_processnegative regulation of reproductive process
Functional Information from PDB Data
site_idAC1
Number of Residues12
DetailsBINDING SITE FOR RESIDUE SO4 A 801
ChainResidue
AHIS46
AHOH868
AHOH877
CASN26
AHIS48
AHIS63
AHIS120
ATHR137
AARG143
AZN201
AHOH823
AHOH852

site_idAC2
Number of Residues10
DetailsBINDING SITE FOR RESIDUE SO4 B 802
ChainResidue
BHIS46
BHIS48
BHIS63
BHIS120
BTHR137
BARG143
BZN203
BHOH881
BHOH897
BHOH899

site_idAC3
Number of Residues12
DetailsBINDING SITE FOR RESIDUE SO4 C 803
ChainResidue
AASN26
CHIS46
CHIS48
CHIS63
CHIS120
CTHR137
CARG143
CZN205
CHOH819
CHOH826
CHOH838
CHOH862

site_idAC4
Number of Residues5
DetailsBINDING SITE FOR RESIDUE ZN A 201
ChainResidue
AHIS46
AHIS48
AHIS63
AHIS120
ASO4801

site_idAC5
Number of Residues4
DetailsBINDING SITE FOR RESIDUE ZN A 202
ChainResidue
AHIS63
AHIS71
AHIS80
AASP83

site_idAC6
Number of Residues5
DetailsBINDING SITE FOR RESIDUE ZN B 203
ChainResidue
BHIS46
BHIS48
BHIS63
BHIS120
BSO4802

site_idAC7
Number of Residues4
DetailsBINDING SITE FOR RESIDUE ZN B 204
ChainResidue
BHIS63
BHIS71
BHIS80
BASP83

site_idAC8
Number of Residues5
DetailsBINDING SITE FOR RESIDUE ZN C 205
ChainResidue
CHIS46
CHIS48
CHIS63
CHIS120
CSO4803

site_idAC9
Number of Residues4
DetailsBINDING SITE FOR RESIDUE ZN C 206
ChainResidue
CHIS63
CHIS71
CHIS80
CASP83

Functional Information from PROSITE/UniProt
site_idPS00087
Number of Residues11
DetailsSOD_CU_ZN_1 Copper/Zinc superoxide dismutase signature 1. GFHVHEfGDnT
ChainResidueDetails
AGLY44-THR54

site_idPS00332
Number of Residues12
DetailsSOD_CU_ZN_2 Copper/Zinc superoxide dismutase signature 2. GNAGsRlACgvI
ChainResidueDetails
AGLY138-ILE149

Functional Information from SwissProt/UniProt
site_idSWS_FT_FI1
Number of Residues9
DetailsBINDING: BINDING => ECO:0000269|PubMed:12963370, ECO:0000269|PubMed:17548825
ChainResidueDetails
AVAL47
AGLU49
AGLU121
BVAL47
BGLU49
BGLU121
CVAL47
CGLU49
CGLU121

site_idSWS_FT_FI2
Number of Residues12
DetailsBINDING: BINDING => ECO:0000269|PubMed:20727846
ChainResidueDetails
APHE64
CGLY72
CVAL81
CLEU84
AGLY72
AVAL81
ALEU84
BPHE64
BGLY72
BVAL81
BLEU84
CPHE64

site_idSWS_FT_FI3
Number of Residues3
DetailsMOD_RES: N-acetylalanine => ECO:0000269|PubMed:1463506, ECO:0000269|PubMed:7002610, ECO:0007744|PubMed:25944712
ChainResidueDetails
ATHR2
BTHR2
CTHR2

site_idSWS_FT_FI4
Number of Residues9
DetailsMOD_RES: N6-succinyllysine => ECO:0000250|UniProtKB:P08228
ChainResidueDetails
AALA4
AGLY10
AASP92
BALA4
BGLY10
BASP92
CALA4
CGLY10
CASP92

site_idSWS_FT_FI5
Number of Residues3
DetailsMOD_RES: Phosphoserine => ECO:0007744|PubMed:18669648, ECO:0007744|PubMed:20068231, ECO:0007744|PubMed:24275569
ChainResidueDetails
AILE99
BILE99
CILE99

site_idSWS_FT_FI6
Number of Residues3
DetailsMOD_RES: Phosphoserine => ECO:0007744|PubMed:24275569
ChainResidueDetails
AVAL103
BVAL103
CVAL103

site_idSWS_FT_FI7
Number of Residues3
DetailsMOD_RES: Phosphoserine => ECO:0000250|UniProtKB:P07632
ChainResidueDetails
ALEU106
BLEU106
CLEU106

site_idSWS_FT_FI8
Number of Residues3
DetailsMOD_RES: Phosphoserine => ECO:0000250|UniProtKB:P08228
ChainResidueDetails
AGLY108
BGLY108
CGLY108

site_idSWS_FT_FI9
Number of Residues3
DetailsMOD_RES: N6-succinyllysine; alternate => ECO:0000269|PubMed:24140062
ChainResidueDetails
AALA123
BALA123
CALA123

site_idSWS_FT_FI10
Number of Residues3
DetailsMOD_RES: N6-succinyllysine; alternate => ECO:0000250|UniProtKB:P08228
ChainResidueDetails
ATHR137
BTHR137
CTHR137

site_idSWS_FT_FI11
Number of Residues3
DetailsLIPID: S-palmitoyl cysteine => ECO:0000269|PubMed:22496122
ChainResidueDetails
AVAL7
BVAL7
CVAL7

site_idSWS_FT_FI12
Number of Residues6
DetailsCROSSLNK: 1-(tryptophan-3-yl)-tryptophan (Trp-Trp) (interchain with W-33) => ECO:0000269|PubMed:20600836
ChainResidueDetails
AGLY33
BGLY33
CGLY33

Catalytic Information from CSA
site_idCSA1
Number of Residues2
DetailsAnnotated By Reference To The Literature 2jcw
ChainResidueDetails
AARG143
AHIS63

site_idCSA2
Number of Residues2
DetailsAnnotated By Reference To The Literature 2jcw
ChainResidueDetails
BARG143
BHIS63

site_idCSA3
Number of Residues2
DetailsAnnotated By Reference To The Literature 2jcw
ChainResidueDetails
CARG143
CHIS63

site_idCSA4
Number of Residues1
DetailsAnnotated By Reference To The Literature 2jcw
ChainResidueDetails
AHIS63

site_idCSA5
Number of Residues1
DetailsAnnotated By Reference To The Literature 2jcw
ChainResidueDetails
BHIS63

site_idCSA6
Number of Residues1
DetailsAnnotated By Reference To The Literature 2jcw
ChainResidueDetails
CHIS63

226707

PDB entries from 2024-10-30

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