4RHP

Crystal structure of human COQ9 in complex with a phospholipid, Northeast Structural Genomics Consortium Target HR5043

External Resource: Annotation

Domain Annotation: ECOD Classification
Protein Family Annotation
Gene Ontology: Gene Product Annotation
InterPro: Protein Family Classification
Pharos: Disease Associations

Domain Annotation: ECOD Classification ECOD Database Homepage

Chains	Family Name	Domain Identifier	Architecture	Possible Homology	Homology	Topology	Family	Provenance Source (Version)
A	diverge_rpsU	e4rhpA2	A: alpha arrays	X: HTH	H: HTH	T: tri-helical	F: diverge_rpsU	ECOD (1.6)
A	COQ9	e4rhpA1	A: alpha bundles	X: Tetracyclin repressor-like, C-terminal domain (From Topology)	H: Tetracyclin repressor-like, C-terminal domain (From Topology)	T: Tetracyclin repressor-like, C-terminal domain	F: COQ9	ECOD (1.6)
B	diverge_rpsU	e4rhpB2	A: alpha arrays	X: HTH	H: HTH	T: tri-helical	F: diverge_rpsU	ECOD (1.6)
B	COQ9	e4rhpB1	A: alpha bundles	X: Tetracyclin repressor-like, C-terminal domain (From Topology)	H: Tetracyclin repressor-like, C-terminal domain (From Topology)	T: Tetracyclin repressor-like, C-terminal domain	F: COQ9	ECOD (1.6)

Protein Family Annotation Pfam Database Homepage

Chains	Accession	Name	Description	Comments	Source
A, B	PF08511	COQ9 (COQ9)	COQ9	COQ9 is an enzyme that is required for the biosynthesis of coenzyme Q [1]. It may either catalyse a reaction in the coenzyme Q biosynthetic pathway or have a regulatory role.	Domain
A, B	PF21392	Ubiquinone biosynthesis protein COQ9, N-terminal domain (COQ9_N)	Ubiquinone biosynthesis protein COQ9, N-terminal domain	This domain is found at the N-terminal end of Ubiquinone biosynthesis protein COQ9 from humans and similar proteins predominantly from vertebrates. COQ9 is a lipid-binding protein involved in the biosynthesis of coenzyme Q, also named ubiquinone, an ...	This domain is found at the N-terminal end of Ubiquinone biosynthesis protein COQ9 from humans and similar proteins predominantly from vertebrates. COQ9 is a lipid-binding protein involved in the biosynthesis of coenzyme Q, also named ubiquinone, an essential lipid-soluble electron transporter for aerobic cellular respiration. This protein has structural homology to the TFR family of bacterial transcriptional regulators. This domain contains an a helix-turn- helix (HTH) motif that largely lacks the positive charges required for DNA binding [1,2].	Domain

Gene Ontology: Gene Product Annotation Gene Ontology Database Homepage

Chains	Polymer	Molecular Function	Biological Process	Cellular Component
A, B	Ubiquinone biosynthesis protein COQ9, mitochondrial	identical protein binding protein binding binding protein homodimerization activity protein dimerization activity lipid binding	aerobic respiration cellular respiration aerobic electron transport chain oxidative phosphorylation energy derivation by oxidation of organic compounds ATP synthesis coupled electron transport electron transport chain generation of precursor metabolites and energy mitochondrial electron transport, NADH to ubiquinone respiratory electron transport chain metabolic process cellular process mitochondrial ATP synthesis coupled electron transport small molecule biosynthetic process aerobic respiration cellular respiration aerobic electron transport chain oxidative phosphorylation energy derivation by oxidation of organic compounds ATP synthesis coupled electron transport electron transport chain generation of precursor metabolites and energy mitochondrial electron transport, NADH to ubiquinone respiratory electron transport chain metabolic process cellular process mitochondrial ATP synthesis coupled electron transport small molecule biosynthetic process ketone metabolic process ubiquinone biosynthetic process quinone metabolic process biosynthetic process quinone biosynthetic process small molecule metabolic process ubiquinone metabolic process ketone biosynthetic process	membrane-bounded organelle intracellular organelle cellular anatomical structure intracellular anatomical structure mitochondrion organelle cytoplasm intracellular membrane-bounded organelle mitochondrial membrane organelle inner membrane membrane mitochondrial inner membrane organelle envelope mitochondrial envelope membrane-bounded organelle intracellular organelle cellular anatomical structure intracellular anatomical structure mitochondrion organelle cytoplasm intracellular membrane-bounded organelle mitochondrial membrane organelle inner membrane membrane mitochondrial inner membrane organelle envelope mitochondrial envelope organelle membrane protein-containing complex catalytic complex ubiquinone biosynthesis complex

InterPro: Protein Family Classification InterPro Database Homepage

Chains	Accession	Name	Type
A, B	IPR013718	COQ9, C-terminal domain	Domain
A, B	IPR048674	Ubiquinone biosynthesis protein COQ9, HTH domain	Domain
A, B	IPR012762	Ubiquinone biosynthesis protein COQ9	Family

Pharos: Disease Associations Pharos Homepage Annotation

Chains	Drug Target	Associated Disease
A, B	Pharos: O75208	encephalopathy-hypertrophic cardiomyopathy-renal tubular disease syndrome coenzyme Q10 deficiency Epileptic encephalopathy systemic lupus erythematosus drug-induced liver injury intellectual disability osteosarcoma leukodystrophy ovarian cancer

RCSB PDB Core Operations are funded by the U.S. National Science Foundation (DBI-2321666), the US Department of Energy (DE-SC0019749), and the National Cancer Institute, National Institute of Allergy and Infectious Diseases, and National Institute of General Medical Sciences of the National Institutes of Health under grant R01GM157729.