PDB entry 6i4z

View 6i4z on RCSB PDB site
Description: crystal structure of the disease-causing p453l mutant of the human dihydrolipoamide dehydrogenase
Deposited on 2018-11-12, released 2019-11-20
The last revision was dated 2019-12-11, with a file datestamp of 2019-12-06.
Experiment type: XRAY
Resolution: 2.34 Å
R-factor: N/A
AEROSPACI score: 0.24 (click here for full SPACI score report)

Chains and heterogens:

  • Chain 'A':
    Compound: Dihydrolipoyl dehydrogenase, mitochondrial
    Species: Homo sapiens [TaxId:9606]
    Gene: DLD, GCSL, LAD, PHE3
    Database cross-references and differences (RAF-indexed):
    • Uniprot P09622 (Start-495)
      • engineered mutation (474)
  • Chain 'B':
    Compound: Dihydrolipoyl dehydrogenase, mitochondrial
    Species: Homo sapiens [TaxId:9606]
    Gene: DLD, GCSL, LAD, PHE3
    Database cross-references and differences (RAF-indexed):
    • Uniprot P09622 (Start-495)
      • engineered mutation (474)
  • Chain 'C':
    Compound: Dihydrolipoyl dehydrogenase, mitochondrial
    Species: Homo sapiens [TaxId:9606]
    Gene: DLD, GCSL, LAD, PHE3
    Database cross-references and differences (RAF-indexed):
    • Uniprot P09622 (Start-495)
      • engineered mutation (474)
  • Chain 'D':
    Compound: Dihydrolipoyl dehydrogenase, mitochondrial
    Species: Homo sapiens [TaxId:9606]
    Gene: DLD, GCSL, LAD, PHE3
    Database cross-references and differences (RAF-indexed):
    • Uniprot P09622 (Start-495)
      • engineered mutation (474)
  • Chain 'E':
    Compound: Dihydrolipoyl dehydrogenase, mitochondrial
    Species: Homo sapiens [TaxId:9606]
    Gene: DLD, GCSL, LAD, PHE3
    Database cross-references and differences (RAF-indexed):
    • Uniprot P09622 (Start-495)
      • engineered mutation (474)
  • Chain 'F':
    Compound: Dihydrolipoyl dehydrogenase, mitochondrial
    Species: Homo sapiens [TaxId:9606]
    Gene: DLD, GCSL, LAD, PHE3
    Database cross-references and differences (RAF-indexed):
    • Uniprot P09622 (Start-495)
      • engineered mutation (474)
  • Chain 'G':
    Compound: Dihydrolipoyl dehydrogenase, mitochondrial
    Species: Homo sapiens [TaxId:9606]
    Gene: DLD, GCSL, LAD, PHE3
    Database cross-references and differences (RAF-indexed):
    • Uniprot P09622 (Start-495)
      • engineered mutation (474)
  • Chain 'H':
    Compound: Dihydrolipoyl dehydrogenase, mitochondrial
    Species: Homo sapiens [TaxId:9606]
    Gene: DLD, GCSL, LAD, PHE3
    Database cross-references and differences (RAF-indexed):
    • Uniprot P09622 (Start-495)
      • engineered mutation (474)
  • Heterogens: FAD, SO4, HOH

PDB Chain Sequences:
This PDB entry is not classified in SCOPe 2.08, so the chain sequences below are not included in the ASTRAL sequence sets.

  • Chain 'A':
    Sequence, based on SEQRES records:
    >6i4zA (A:)
maswshpqfekgalevlfqgpgadqpidadvtvigsgpggyvaaikaaqlgfktvciekn
etlggtclnvgcipskallnnshyyhmahgkdfasrgiemsevrlnldkmmeqkstavka
ltggiahlfkqnkvvhvngygkitgknqvtatkadggtqvidtkniliatgsevtpfpgi
tidedtivsstgalslkkvpekmvvigagvigvelgsvwqrlgadvtaveflghvggvgi
dmeisknfqrilqkqgfkfklntkvtgatkksdgkidvsieaasggkaevitcdvllvci
grrpftknlgleelgieldprgripvntrfqtkipniyaigdvvagpmlahkaedegiic
vegmaggavhidyncvpsviythpevawvgkseeqlkeegieykvgkfpfaansraktna
dtdgmvkilgqkstdrvlgahilgpgagemvneaalaleygascediarvchahltlsea
freanlaasfgksinf

    Sequence, based on observed residues (ATOM records):
    >6i4zA (A:)
qpidadvtvigsgpggyvaaikaaqlgfktvcieknetlggtclnvgcipskallnnshy
yhmahgkdfasrgiemsevrlnldkmmeqkstavkaltggiahlfkqnkvvhvngygkit
gknqvtatkadggtqvidtkniliatgsevtpfpgitidedtivsstgalslkkvpekmv
vigagvigvelgsvwqrlgadvtaveflghvggvgidmeisknfqrilqkqgfkfklntk
vtgatkksdgkidvsieaasggkaevitcdvllvcigrrpftknlgleelgieldprgri
pvntrfqtkipniyaigdvvagpmlahkaedegiicvegmaggavhidyncvpsviythp
evawvgkseeqlkeegieykvgkfpfaansraktnadtdgmvkilgqkstdrvlgahilg
pgagemvneaalaleygascediarvchahltlseafreanlaasfgksinf

  • Chain 'B':
    Sequence, based on SEQRES records:
    >6i4zB (B:)
maswshpqfekgalevlfqgpgadqpidadvtvigsgpggyvaaikaaqlgfktvciekn
etlggtclnvgcipskallnnshyyhmahgkdfasrgiemsevrlnldkmmeqkstavka
ltggiahlfkqnkvvhvngygkitgknqvtatkadggtqvidtkniliatgsevtpfpgi
tidedtivsstgalslkkvpekmvvigagvigvelgsvwqrlgadvtaveflghvggvgi
dmeisknfqrilqkqgfkfklntkvtgatkksdgkidvsieaasggkaevitcdvllvci
grrpftknlgleelgieldprgripvntrfqtkipniyaigdvvagpmlahkaedegiic
vegmaggavhidyncvpsviythpevawvgkseeqlkeegieykvgkfpfaansraktna
dtdgmvkilgqkstdrvlgahilgpgagemvneaalaleygascediarvchahltlsea
freanlaasfgksinf

    Sequence, based on observed residues (ATOM records):
    >6i4zB (B:)
qpidadvtvigsgpggyvaaikaaqlgfktvcieknetlggtclnvgcipskallnnshy
yhmahgkdfasrgiemsevrlnldkmmeqkstavkaltggiahlfkqnkvvhvngygkit
gknqvtatkadggtqvidtkniliatgsevtpfpgitidedtivsstgalslkkvpekmv
vigagvigvelgsvwqrlgadvtaveflghvggvgidmeisknfqrilqkqgfkfklntk
vtgatkksdgkidvsieaasggkaevitcdvllvcigrrpftknlgleelgieldprgri
pvntrfqtkipniyaigdvvagpmlahkaedegiicvegmaggavhidyncvpsviythp
evawvgkseeqlkeegieykvgkfpfaansraktnadtdgmvkilgqkstdrvlgahilg
pgagemvneaalaleygascediarvchahltlseafreanlaasfgksinf

  • Chain 'C':
    Sequence, based on SEQRES records:
    >6i4zC (C:)
maswshpqfekgalevlfqgpgadqpidadvtvigsgpggyvaaikaaqlgfktvciekn
etlggtclnvgcipskallnnshyyhmahgkdfasrgiemsevrlnldkmmeqkstavka
ltggiahlfkqnkvvhvngygkitgknqvtatkadggtqvidtkniliatgsevtpfpgi
tidedtivsstgalslkkvpekmvvigagvigvelgsvwqrlgadvtaveflghvggvgi
dmeisknfqrilqkqgfkfklntkvtgatkksdgkidvsieaasggkaevitcdvllvci
grrpftknlgleelgieldprgripvntrfqtkipniyaigdvvagpmlahkaedegiic
vegmaggavhidyncvpsviythpevawvgkseeqlkeegieykvgkfpfaansraktna
dtdgmvkilgqkstdrvlgahilgpgagemvneaalaleygascediarvchahltlsea
freanlaasfgksinf

    Sequence, based on observed residues (ATOM records):
    >6i4zC (C:)
qpidadvtvigsgpggyvaaikaaqlgfktvcieknetlggtclnvgcipskallnnshy
yhmahgkdfasrgiemsevrlnldkmmeqkstavkaltggiahlfkqnkvvhvngygkit
gknqvtatkadggtqvidtkniliatgsevtpfpgitidedtivsstgalslkkvpekmv
vigagvigvelgsvwqrlgadvtaveflghvggvgidmeisknfqrilqkqgfkfklntk
vtgatkksdgkidvsieaasggkaevitcdvllvcigrrpftknlgleelgieldprgri
pvntrfqtkipniyaigdvvagpmlahkaedegiicvegmaggavhidyncvpsviythp
evawvgkseeqlkeegieykvgkfpfaansraktnadtdgmvkilgqkstdrvlgahilg
pgagemvneaalaleygascediarvchahltlseafreanlaasfgksinf

  • Chain 'D':
    Sequence, based on SEQRES records:
    >6i4zD (D:)
maswshpqfekgalevlfqgpgadqpidadvtvigsgpggyvaaikaaqlgfktvciekn
etlggtclnvgcipskallnnshyyhmahgkdfasrgiemsevrlnldkmmeqkstavka
ltggiahlfkqnkvvhvngygkitgknqvtatkadggtqvidtkniliatgsevtpfpgi
tidedtivsstgalslkkvpekmvvigagvigvelgsvwqrlgadvtaveflghvggvgi
dmeisknfqrilqkqgfkfklntkvtgatkksdgkidvsieaasggkaevitcdvllvci
grrpftknlgleelgieldprgripvntrfqtkipniyaigdvvagpmlahkaedegiic
vegmaggavhidyncvpsviythpevawvgkseeqlkeegieykvgkfpfaansraktna
dtdgmvkilgqkstdrvlgahilgpgagemvneaalaleygascediarvchahltlsea
freanlaasfgksinf

    Sequence, based on observed residues (ATOM records):
    >6i4zD (D:)
qpidadvtvigsgpggyvaaikaaqlgfktvcieknetlggtclnvgcipskallnnshy
yhmahgkdfasrgiemsevrlnldkmmeqkstavkaltggiahlfkqnkvvhvngygkit
gknqvtatkadggtqvidtkniliatgsevtpfpgitidedtivsstgalslkkvpekmv
vigagvigvelgsvwqrlgadvtaveflghvggvgidmeisknfqrilqkqgfkfklntk
vtgatkksdgkidvsieaasggkaevitcdvllvcigrrpftknlgleelgieldprgri
pvntrfqtkipniyaigdvvagpmlahkaedegiicvegmaggavhidyncvpsviythp
evawvgkseeqlkeegieykvgkfpfaansraktnadtdgmvkilgqkstdrvlgahilg
pgagemvneaalaleygascediarvchahltlseafreanlaasfgksinf

  • Chain 'E':
    Sequence, based on SEQRES records:
    >6i4zE (E:)
maswshpqfekgalevlfqgpgadqpidadvtvigsgpggyvaaikaaqlgfktvciekn
etlggtclnvgcipskallnnshyyhmahgkdfasrgiemsevrlnldkmmeqkstavka
ltggiahlfkqnkvvhvngygkitgknqvtatkadggtqvidtkniliatgsevtpfpgi
tidedtivsstgalslkkvpekmvvigagvigvelgsvwqrlgadvtaveflghvggvgi
dmeisknfqrilqkqgfkfklntkvtgatkksdgkidvsieaasggkaevitcdvllvci
grrpftknlgleelgieldprgripvntrfqtkipniyaigdvvagpmlahkaedegiic
vegmaggavhidyncvpsviythpevawvgkseeqlkeegieykvgkfpfaansraktna
dtdgmvkilgqkstdrvlgahilgpgagemvneaalaleygascediarvchahltlsea
freanlaasfgksinf

    Sequence, based on observed residues (ATOM records):
    >6i4zE (E:)
qpidadvtvigsgpggyvaaikaaqlgfktvcieknetlggtclnvgcipskallnnshy
yhmahgkdfasrgiemsevrlnldkmmeqkstavkaltggiahlfkqnkvvhvngygkit
gknqvtatkadggtqvidtkniliatgsevtpfpgitidedtivsstgalslkkvpekmv
vigagvigvelgsvwqrlgadvtaveflghvggvgidmeisknfqrilqkqgfkfklntk
vtgatkksdgkidvsieaasggkaevitcdvllvcigrrpftknlgleelgieldprgri
pvntrfqtkipniyaigdvvagpmlahkaedegiicvegmaggavhidyncvpsviythp
evawvgkseeqlkeegieykvgkfpfaansraktnadtdgmvkilgqkstdrvlgahilg
pgagemvneaalaleygascediarvchahltlseafreanlaasfgksinf

  • Chain 'F':
    Sequence, based on SEQRES records:
    >6i4zF (F:)
maswshpqfekgalevlfqgpgadqpidadvtvigsgpggyvaaikaaqlgfktvciekn
etlggtclnvgcipskallnnshyyhmahgkdfasrgiemsevrlnldkmmeqkstavka
ltggiahlfkqnkvvhvngygkitgknqvtatkadggtqvidtkniliatgsevtpfpgi
tidedtivsstgalslkkvpekmvvigagvigvelgsvwqrlgadvtaveflghvggvgi
dmeisknfqrilqkqgfkfklntkvtgatkksdgkidvsieaasggkaevitcdvllvci
grrpftknlgleelgieldprgripvntrfqtkipniyaigdvvagpmlahkaedegiic
vegmaggavhidyncvpsviythpevawvgkseeqlkeegieykvgkfpfaansraktna
dtdgmvkilgqkstdrvlgahilgpgagemvneaalaleygascediarvchahltlsea
freanlaasfgksinf

    Sequence, based on observed residues (ATOM records):
    >6i4zF (F:)
qpidadvtvigsgpggyvaaikaaqlgfktvcieknetlggtclnvgcipskallnnshy
yhmahgkdfasrgiemsevrlnldkmmeqkstavkaltggiahlfkqnkvvhvngygkit
gknqvtatkadggtqvidtkniliatgsevtpfpgitidedtivsstgalslkkvpekmv
vigagvigvelgsvwqrlgadvtaveflghvggvgidmeisknfqrilqkqgfkfklntk
vtgatkksdgkidvsieaasggkaevitcdvllvcigrrpftknlgleelgieldprgri
pvntrfqtkipniyaigdvvagpmlahkaedegiicvegmaggavhidyncvpsviythp
evawvgkseeqlkeegieykvgkfpfaansraktnadtdgmvkilgqkstdrvlgahilg
pgagemvneaalaleygascediarvchahltlseafreanlaasfgksinf

  • Chain 'G':
    Sequence, based on SEQRES records:
    >6i4zG (G:)
maswshpqfekgalevlfqgpgadqpidadvtvigsgpggyvaaikaaqlgfktvciekn
etlggtclnvgcipskallnnshyyhmahgkdfasrgiemsevrlnldkmmeqkstavka
ltggiahlfkqnkvvhvngygkitgknqvtatkadggtqvidtkniliatgsevtpfpgi
tidedtivsstgalslkkvpekmvvigagvigvelgsvwqrlgadvtaveflghvggvgi
dmeisknfqrilqkqgfkfklntkvtgatkksdgkidvsieaasggkaevitcdvllvci
grrpftknlgleelgieldprgripvntrfqtkipniyaigdvvagpmlahkaedegiic
vegmaggavhidyncvpsviythpevawvgkseeqlkeegieykvgkfpfaansraktna
dtdgmvkilgqkstdrvlgahilgpgagemvneaalaleygascediarvchahltlsea
freanlaasfgksinf

    Sequence, based on observed residues (ATOM records):
    >6i4zG (G:)
qpidadvtvigsgpggyvaaikaaqlgfktvcieknetlggtclnvgcipskallnnshy
yhmahgkdfasrgiemsevrlnldkmmeqkstavkaltggiahlfkqnkvvhvngygkit
gknqvtatkadggtqvidtkniliatgsevtpfpgitidedtivsstgalslkkvpekmv
vigagvigvelgsvwqrlgadvtaveflghvggvgidmeisknfqrilqkqgfkfklntk
vtgatkksdgkidvsieaasggkaevitcdvllvcigrrpftknlgleelgieldprgri
pvntrfqtkipniyaigdvvagpmlahkaedegiicvegmaggavhidyncvpsviythp
evawvgkseeqlkeegieykvgkfpfaansraktnadtdgmvkilgqkstdrvlgahilg
pgagemvneaalaleygascediarvchahltlseafreanlaasfgksinf

  • Chain 'H':
    Sequence, based on SEQRES records:
    >6i4zH (H:)
maswshpqfekgalevlfqgpgadqpidadvtvigsgpggyvaaikaaqlgfktvciekn
etlggtclnvgcipskallnnshyyhmahgkdfasrgiemsevrlnldkmmeqkstavka
ltggiahlfkqnkvvhvngygkitgknqvtatkadggtqvidtkniliatgsevtpfpgi
tidedtivsstgalslkkvpekmvvigagvigvelgsvwqrlgadvtaveflghvggvgi
dmeisknfqrilqkqgfkfklntkvtgatkksdgkidvsieaasggkaevitcdvllvci
grrpftknlgleelgieldprgripvntrfqtkipniyaigdvvagpmlahkaedegiic
vegmaggavhidyncvpsviythpevawvgkseeqlkeegieykvgkfpfaansraktna
dtdgmvkilgqkstdrvlgahilgpgagemvneaalaleygascediarvchahltlsea
freanlaasfgksinf

    Sequence, based on observed residues (ATOM records):
    >6i4zH (H:)
qpidadvtvigsgpggyvaaikaaqlgfktvcieknetlggtclnvgcipskallnnshy
yhmahgkdfasrgiemsevrlnldkmmeqkstavkaltggiahlfkqnkvvhvngygkit
gknqvtatkadggtqvidtkniliatgsevtpfpgitidedtivsstgalslkkvpekmv
vigagvigvelgsvwqrlgadvtaveflghvggvgidmeisknfqrilqkqgfkfklntk
vtgatkksdgkidvsieaasggkaevitcdvllvcigrrpftknlgleelgieldprgri
pvntrfqtkipniyaigdvvagpmlahkaedegiicvegmaggavhidyncvpsviythp
evawvgkseeqlkeegieykvgkfpfaansraktnadtdgmvkilgqkstdrvlgahilg
pgagemvneaalaleygascediarvchahltlseafreanlaasfgksinf