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5I35

Structure of the Human mitochondrial kinase COQ8A R611K with AMPPNP (Cerebellar Ataxia and Ubiquinone Deficiency Through Loss of Unorthodox Kinase Activity)

Functional Information from GO Data
ChainGOidnamespacecontents
A0005739cellular_componentmitochondrion
A0006744biological_processubiquinone biosynthetic process
Functional Information from PDB Data
site_idAC1
Number of Residues12
Detailsbinding site for residue ANP A 700
ChainResidue
APHE336
AASN493
APHE495
AASP507
AALA337
AALA339
ASER340
AVAL344
ALYS358
ATHR445
AGLU446
AVAL448

Functional Information from SwissProt/UniProt
site_idSWS_FT_FI1
Number of Residues3
DetailsMotif: {"description":"KxGQ motif","evidences":[{"source":"PubMed","id":"25498144","evidenceCode":"ECO:0000269"}]}
ChainResidueDetails

site_idSWS_FT_FI2
Number of Residues3
DetailsMotif: {"description":"AAAS motif","evidences":[{"source":"PubMed","id":"25498144","evidenceCode":"ECO:0000269"}]}
ChainResidueDetails

site_idSWS_FT_FI3
Number of Residues1
DetailsActive site: {"description":"Proton acceptor","evidences":[{"source":"PubMed","id":"27499294","evidenceCode":"ECO:0000305"}]}
ChainResidueDetails

site_idSWS_FT_FI4
Number of Residues1
DetailsBinding site: {"evidences":[{"source":"PubMed","id":"27499294","evidenceCode":"ECO:0000269"},{"source":"PubMed","id":"25498144","evidenceCode":"ECO:0000305"},{"source":"PDB","id":"5I35","evidenceCode":"ECO:0000312"}]}
ChainResidueDetails

site_idSWS_FT_FI5
Number of Residues6
DetailsBinding site: {"evidences":[{"source":"PubMed","id":"27499294","evidenceCode":"ECO:0000269"},{"source":"PDB","id":"5I35","evidenceCode":"ECO:0000312"}]}
ChainResidueDetails

238582

PDB entries from 2025-07-09

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