8VGY

Crystal structure of human apoptosis-inducing factor (AIF) bound to the fused N-terminal domain of CHCHD4

External Resource: Annotation

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

Protein Family Annotation Pfam Database Homepage

Chains	Accession	Name	Description	Comments	Source
A, B [auth C]	PF00070	Pyridine nucleotide-disulphide oxidoreductase (Pyr_redox)	Pyridine nucleotide-disulphide oxidoreductase	This family includes both class I and class II oxidoreductases and also NADH oxidases and peroxidases. This domain is actually a small NADH binding domain within a larger FAD binding domain.	Domain
A, B [auth C]	PF14721	Apoptosis-inducing factor, mitochondrion-associated, C-term (AIF_C)	Apoptosis-inducing factor, mitochondrion-associated, C-term	This C-terminal domain appears to be a dimerisation domain of the mitochondrial apoptosis-inducing factor 1. protein. The domain also appears at the C-terminus of FAD-dependent pyridine nucleotide-disulfide oxidoreductases. Apoptosis inducing factor ...	This C-terminal domain appears to be a dimerisation domain of the mitochondrial apoptosis-inducing factor 1. protein. The domain also appears at the C-terminus of FAD-dependent pyridine nucleotide-disulfide oxidoreductases. Apoptosis inducing factor (AIF) is a bifunctional mitochondrial flavoprotein critical for energy metabolism and induction of caspase-independent apoptosis. On reduction with NADH, AIF undergoes dimerisation and forms tight, long-lived FADH2-NAD charge-transfer complexes proposed to be functionally important.	Domain

Gene Ontology: Gene Product Annotation Gene Ontology Database Homepage

Chains	Polymer	Molecular Function	Biological Process	Cellular Component
A, B [auth C]	Apoptosis-inducing factor 1, mitochondrial,Mitochondrial intermembrane space import and assembly protein 40	FAD binding flavin adenine dinucleotide binding binding nucleoside phosphate binding ion binding organic cyclic compound binding anion binding nucleotide binding heterocyclic compound binding small molecule binding NAD(P)H oxidase H2O2-forming activity oxidoreductase activity oxidoreductase activity, acting on NAD(P)H catalytic activity FAD binding flavin adenine dinucleotide binding binding nucleoside phosphate binding ion binding organic cyclic compound binding anion binding nucleotide binding heterocyclic compound binding small molecule binding NAD(P)H oxidase H2O2-forming activity oxidoreductase activity oxidoreductase activity, acting on NAD(P)H catalytic activity oxidoreductase activity, acting on NAD(P)H, oxygen as acceptor DNA binding nucleic acid binding NADH dehydrogenase activity poly-ADP-D-ribose binding carbohydrate derivative binding protein binding protein dimerization activity oxidoreductase activity, acting on a sulfur group of donors disulfide oxidoreductase activity catalytic activity, acting on a protein protein-disulfide reductase activity	response to stimulus response to stress response to ischemia response to decreased oxygen levels cellular response to stimulus cellular response to oxygen levels cellular response to hypoxia cellular response to decreased oxygen levels cellular response to chemical stimulus cellular response to stress response to hypoxia response to chemical response to oxygen levels response to abiotic stimulus response to stimulus response to stress response to ischemia response to decreased oxygen levels cellular response to stimulus cellular response to oxygen levels cellular response to hypoxia cellular response to decreased oxygen levels cellular response to chemical stimulus cellular response to stress response to hypoxia response to chemical response to oxygen levels response to abiotic stimulus cellular process cellular component organization organelle organization chromosome condensation chromosome organization cellular component organization or biogenesis apoptotic process cell death programmed cell death response to ketone response to lipid cellular response to mineralocorticoid stimulus response to mineralocorticoid cellular response to endogenous stimulus cellular response to aldehyde response to hormone response to steroid hormone cellular response to ketone response to organic cyclic compound cellular response to steroid hormone stimulus cellular response to hormone stimulus cellular response to aldosterone response to aldosterone response to endogenous stimulus response to alcohol cellular response to organic cyclic compound response to oxygen-containing compound cellular response to oxygen-containing compound cellular response to corticosteroid stimulus response to corticosteroid cellular response to alcohol cellular response to lipid regulation of biological process positive regulation of programmed cell death regulation of cellular process regulation of apoptotic process biological regulation positive regulation of biological process regulation of programmed cell death positive regulation of apoptotic process positive regulation of cellular process protein-containing complex organization mitochondrial respirasome assembly NADH dehydrogenase complex assembly cellular component biogenesis cellular component assembly mitochondrial respiratory chain complex assembly protein-containing complex assembly mitochondrial respiratory chain complex I assembly mitochondrion organization neuron apoptotic process positive regulation of neuron apoptotic process regulation of neuron apoptotic process developmental process multicellular organismal process cellular developmental process nervous system development generation of neurons neurogenesis multicellular organism development neuron differentiation cell differentiation anatomical structure development system development response to toxic substance cellular response to oxidative stress cellular response to chemical stress cellular response to hydrogen peroxide response to reactive oxygen species response to hydrogen peroxide response to oxidative stress cellular response to reactive oxygen species catabolic process macromolecule catabolic process regulation of DNA catabolic process regulation of primary metabolic process nucleobase-containing compound metabolic process execution phase of apoptosis DNA catabolic process apoptotic nuclear changes regulation of nucleobase-containing compound metabolic process regulation of apoptotic DNA fragmentation cellular component disassembly primary metabolic process DNA metabolic process regulation of macromolecule metabolic process regulation of catabolic process nucleic acid metabolic process nucleobase-containing compound catabolic process apoptotic DNA fragmentation cellular component disassembly involved in execution phase of apoptosis nucleic acid catabolic process metabolic process macromolecule metabolic process regulation of metabolic process regulation of DNA metabolic process signaling intrinsic apoptotic signaling pathway in response to endoplasmic reticulum stress intracellular signal transduction cell communication apoptotic signaling pathway signal transduction intrinsic apoptotic signaling pathway response to endoplasmic reticulum stress response to estradiol cellular response to estradiol stimulus positive regulation of necroptotic process necroptotic process programmed necrotic cell death regulation of necroptotic process regulation of programmed necrotic cell death positive regulation of programmed necrotic cell death response to acid chemical response to nitrogen compound response to L-glutamate response to amino acid protein localization establishment of protein localization cellular localization nitrogen compound transport protein transmembrane import into intracellular organelle transport protein targeting localization protein transmembrane transport cellular macromolecule localization mitochondrial transport protein import into mitochondrial intermembrane space establishment of localization mitochondrial transmembrane transport transmembrane transport establishment of protein localization to organelle protein transport macromolecule localization protein localization to organelle protein localization to mitochondrion protein targeting to mitochondrion establishment of protein localization to mitochondrion cellular response to nitrogen compound cellular response to nitric oxide response to nitric oxide cellular response to reactive nitrogen species gene expression macromolecule biosynthetic process protein maturation protein folding biosynthetic process protein metabolic process 'de novo' protein folding 'de novo' post-translational protein folding protein maturation by protein folding mitochondrial genome maintenance mitochondrial DNA metabolic process DNA damage response mitochondrial DNA repair DNA repair peptidyl-cysteine modification protein oxidation macromolecule modification peptidyl-amino acid modification peptidyl-cysteine oxidation protein modification process regulation of localization regulation of protein localization nucleocytoplasmic transport nuclear transport establishment of localization in cell regulation of protein export from nucleus nuclear export regulation of intracellular transport regulation of transport regulation of establishment of protein localization intracellular transport regulation of nucleocytoplasmic transport regulation of protein transport protein export from nucleus regulation of cellular localization regulation of intracellular protein transport intracellular protein transport cellular respiration energy derivation by oxidation of organic compounds regulation of generation of precursor metabolites and energy generation of precursor metabolites and energy regulation of cellular respiration positive regulation of cellular respiration positive regulation of metabolic process response to peptide response to cytokine response to leukemia inhibitory factor cellular response to cytokine stimulus cellular response to leukemia inhibitory factor	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 cytosol perinuclear region of cytoplasm mitochondrial intermembrane space membrane-enclosed lumen intracellular organelle lumen organelle envelope lumen organelle lumen nucleus

InterPro: Protein Family Classification InterPro Database Homepage

Chains	Accession	Name	Type
A, B [auth C]	IPR029324	Mitochondrial apoptosis-inducing factor, C-terminal domain	Domain
A, B [auth C]	IPR036188	FAD/NAD(P)-binding domain superfamily	Homologous Superfamily
A, B [auth C]	IPR016156	FAD/NAD-linked reductase, dimerisation domain superfamily	Homologous Superfamily
A, B [auth C]	IPR050446	FAD-dependent Oxidoreductases and Apoptosis Regulators	Family
A, B [auth C]	IPR023753	FAD/NAD(P)-binding domain	Domain
A, B [auth C]	IPR010625	CHCH	Domain
A, B [auth C]	IPR039289	Mitochondrial intermembrane space import and assembly protein 40	Family

Pharos: Disease Associations Pharos Homepage Annotation

Chains	Drug Target	Associated Disease
A, B [auth C]	Pharos: Q8N4Q1	Charcot-Marie-Tooth disease X-linked hereditary sensory and autonomic neuropathy with deafness Huntington disease severe X-linked mitochondrial encephalomyopathy peripheral neuropathy retinal detachment Cowchock syndrome intellectual disability juvenile Huntington disease lung adenocarcinoma neurodegenerative disease Charcot-Marie-Tooth disease X-linked recessive 4 type 1 diabetes mellitus combined oxidative phosphorylation deficiency Leigh syndrome Charcot-Marie-Tooth disease X-linked hereditary sensory and autonomic neuropathy with deafness Huntington disease severe X-linked mitochondrial encephalomyopathy peripheral neuropathy retinal detachment Cowchock syndrome intellectual disability juvenile Huntington disease lung adenocarcinoma neurodegenerative disease Charcot-Marie-Tooth disease X-linked recessive 4 type 1 diabetes mellitus combined oxidative phosphorylation deficiency Leigh syndrome lung cancer motor peripheral neuropathy pancreatic ductal adenocarcinoma spondyloepimetaphyseal dysplasia, Bieganski type leukodystrophy malignant mesothelioma peripheral nervous system disease Charcot-Marie-Tooth disease type X drug-induced liver injury astrocytic tumor dilated cardiomyopathy malignant ependymoma

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.