1HZD

CRYSTAL STRUCTURE OF HUMAN AUH PROTEIN, AN RNA-BINDING HOMOLOGUE OF ENOYL-COA HYDRATASE

External Resource: Annotation

Domain Annotation: SCOP/SCOPe Classification
Domain Annotation: SCOP2 Classification
Domain Annotation: ECOD Classification
Domain Annotation: CATH
Protein Family Annotation
Gene Ontology: Gene Product Annotation
InterPro: Protein Family Classification
Pharos: Disease Associations
Structure Motif: Primary M-CSA Annotation

Domain Annotation: SCOP/SCOPe Classification SCOP-e Database Homepage

Chains	Domain Info	Class	Fold	Superfamily	Family	Domain	Species	Provenance Source (Version)
A	d1hzda_	Alpha and beta proteins (a/b)	ClpP/crotonase	ClpP/crotonase	Crotonase-like	AUH protein	human (Homo sapiens ) [TaxId: 9606 ],	SCOPe (2.08)
B	d1hzdb_	Alpha and beta proteins (a/b)	ClpP/crotonase	ClpP/crotonase	Crotonase-like	AUH protein	human (Homo sapiens ) [TaxId: 9606 ],	SCOPe (2.08)
C	d1hzdc_	Alpha and beta proteins (a/b)	ClpP/crotonase	ClpP/crotonase	Crotonase-like	AUH protein	human (Homo sapiens ) [TaxId: 9606 ],	SCOPe (2.08)
D	d1hzdd_	Alpha and beta proteins (a/b)	ClpP/crotonase	ClpP/crotonase	Crotonase-like	AUH protein	human (Homo sapiens ) [TaxId: 9606 ],	SCOPe (2.08)
E	d1hzde_	Alpha and beta proteins (a/b)	ClpP/crotonase	ClpP/crotonase	Crotonase-like	AUH protein	human (Homo sapiens ) [TaxId: 9606 ],	SCOPe (2.08)
F	d1hzdf_	Alpha and beta proteins (a/b)	ClpP/crotonase	ClpP/crotonase	Crotonase-like	AUH protein	human (Homo sapiens ) [TaxId: 9606 ],	SCOPe (2.08)

Domain Annotation: SCOP2 Classification SCOP2 Database Homepage

Chains	Type	Family Name	Domain Identifier	Family Identifier	Provenance Source (Version)
A	SCOP2 Family	Crotonase-like	8058006	4000036	SCOP2 (2022-06-29)
A	SCOP2 Superfamily	ClpP/crotonase	8058007	3000033	SCOP2 (2022-06-29)
B	SCOP2B Superfamily	ClpP/crotonase	8058007	3000033	SCOP2B (2022-06-29)
C	SCOP2B Superfamily	ClpP/crotonase	8058007	3000033	SCOP2B (2022-06-29)
D	SCOP2B Superfamily	ClpP/crotonase	8058007	3000033	SCOP2B (2022-06-29)
E	SCOP2B Superfamily	ClpP/crotonase	8058007	3000033	SCOP2B (2022-06-29)
F	SCOP2B Superfamily	ClpP/crotonase	8058007	3000033	SCOP2B (2022-06-29)

Domain Annotation: ECOD Classification ECOD Database Homepage

Chains	Family Name	Domain Identifier	Architecture	Possible Homology	Homology	Topology	Family	Provenance Source (Version)
A	PF00378	e1hzdA1	A: a/b three-layered sandwiches	X: ClpP/crotonase (From Topology)	H: ClpP/crotonase (From Topology)	T: ClpP/crotonase	F: PF00378	ECOD (1.6)
B	PF00378	e1hzdB1	A: a/b three-layered sandwiches	X: ClpP/crotonase (From Topology)	H: ClpP/crotonase (From Topology)	T: ClpP/crotonase	F: PF00378	ECOD (1.6)
C	PF00378	e1hzdC1	A: a/b three-layered sandwiches	X: ClpP/crotonase (From Topology)	H: ClpP/crotonase (From Topology)	T: ClpP/crotonase	F: PF00378	ECOD (1.6)
D	PF00378	e1hzdD1	A: a/b three-layered sandwiches	X: ClpP/crotonase (From Topology)	H: ClpP/crotonase (From Topology)	T: ClpP/crotonase	F: PF00378	ECOD (1.6)
E	PF00378	e1hzdE1	A: a/b three-layered sandwiches	X: ClpP/crotonase (From Topology)	H: ClpP/crotonase (From Topology)	T: ClpP/crotonase	F: PF00378	ECOD (1.6)
F	PF00378	e1hzdF1	A: a/b three-layered sandwiches	X: ClpP/crotonase (From Topology)	H: ClpP/crotonase (From Topology)	T: ClpP/crotonase	F: PF00378	ECOD (1.6)

Domain Annotation: CATH CATH Database Homepage

Chain	Domain	Class	Architecture	Topology	Homology	Provenance Source (Version)
A	3.90.226.10	Alpha Beta	Alpha-Beta Complex	2-enoyl-CoA Hydratase	Chain A, domain 1	CATH (4.3.0)
A	1.10.12.10	Mainly Alpha	Orthogonal Bundle	Lyase 2-enoyl-coa Hydratase	Chain A, domain 2	CATH (4.3.0)
B	3.90.226.10	Alpha Beta	Alpha-Beta Complex	2-enoyl-CoA Hydratase	Chain A, domain 1	CATH (4.3.0)
B	1.10.12.10	Mainly Alpha	Orthogonal Bundle	Lyase 2-enoyl-coa Hydratase	Chain A, domain 2	CATH (4.3.0)
C	3.90.226.10	Alpha Beta	Alpha-Beta Complex	2-enoyl-CoA Hydratase	Chain A, domain 1	CATH (4.3.0)
C	1.10.12.10	Mainly Alpha	Orthogonal Bundle	Lyase 2-enoyl-coa Hydratase	Chain A, domain 2	CATH (4.3.0)
D	3.90.226.10	Alpha Beta	Alpha-Beta Complex	2-enoyl-CoA Hydratase	Chain A, domain 1	CATH (4.3.0)
D	1.10.12.10	Mainly Alpha	Orthogonal Bundle	Lyase 2-enoyl-coa Hydratase	Chain A, domain 2	CATH (4.3.0)
E	3.90.226.10	Alpha Beta	Alpha-Beta Complex	2-enoyl-CoA Hydratase	Chain A, domain 1	CATH (4.3.0)
E	1.10.12.10	Mainly Alpha	Orthogonal Bundle	Lyase 2-enoyl-coa Hydratase	Chain A, domain 2	CATH (4.3.0)
F	3.90.226.10	Alpha Beta	Alpha-Beta Complex	2-enoyl-CoA Hydratase	Chain A, domain 1	CATH (4.3.0)
F	1.10.12.10	Mainly Alpha	Orthogonal Bundle	Lyase 2-enoyl-coa Hydratase	Chain A, domain 2	CATH (4.3.0)

Protein Family Annotation Pfam Database Homepage

Chains	Accession	Name	Description	Comments	Source
A, B, C, D, E A, B, C, D, E, F	PF00378	Enoyl-CoA hydratase/isomerase (ECH_1)	Enoyl-CoA hydratase/isomerase	This family contains a diverse set of enzymes including: enoyl-CoA hydratase, napthoate synthase, carnitate racemase, 3-hydroxybutyryl-CoA dehydratase and dodecanoyl-CoA delta-isomerase.	Domain

Gene Ontology: Gene Product Annotation Gene Ontology Database Homepage

Chains	Polymer	Molecular Function	Biological Process	Cellular Component
A, B, C, D, E A, B, C, D, E, F	AU-BINDING PROTEIN/ENOYL-COA HYDRATASE	enoyl-CoA hydratase activity hydro-lyase activity lyase activity catalytic activity carbon-oxygen lyase activity mRNA 3'-UTR binding RNA binding binding organic cyclic compound binding nucleic acid binding mRNA binding methylglutaconyl-CoA hydratase activity itaconyl-CoA hydratase activity	pyruvate family amino acid metabolic process catabolic process oxoacid metabolic process amino acid metabolic process organic acid catabolic process organic acid metabolic process branched-chain amino acid catabolic process alpha-amino acid metabolic process pyruvate family amino acid catabolic process cellular catabolic process organic substance metabolic process carboxylic acid catabolic process small molecule metabolic process branched-chain amino acid metabolic process pyruvate family amino acid metabolic process catabolic process oxoacid metabolic process amino acid metabolic process organic acid catabolic process organic acid metabolic process branched-chain amino acid catabolic process alpha-amino acid metabolic process pyruvate family amino acid catabolic process cellular catabolic process organic substance metabolic process carboxylic acid catabolic process small molecule metabolic process branched-chain amino acid metabolic process amino acid catabolic process L-leucine metabolic process organonitrogen compound catabolic process L-leucine catabolic process organonitrogen compound metabolic process cellular metabolic process carboxylic acid metabolic process L-amino acid catabolic process primary metabolic process L-amino acid metabolic process proteinogenic amino acid metabolic process alpha-amino acid catabolic process proteinogenic amino acid catabolic process metabolic process cellular process small molecule catabolic process organic substance catabolic process cellular lipid catabolic process monocarboxylic acid catabolic process fatty acid oxidation fatty acid beta-oxidation lipid oxidation fatty acid metabolic process lipid catabolic process lipid modification fatty acid catabolic process cellular lipid metabolic process monocarboxylic acid metabolic process lipid metabolic process	membrane-bounded organelle intracellular organelle intracellular anatomical structure mitochondrion organelle cytoplasm cellular anatomical entity intracellular membrane-bounded organelle membrane-enclosed lumen mitochondrial matrix intracellular organelle lumen organelle lumen

InterPro: Protein Family Classification InterPro Database Homepage

Chains	Accession	Name	Type
A, B, C, D, E A, B, C, D, E, F	IPR001753	Enoyl-CoA hydratase/isomerase	Family
A, B, C, D, E A, B, C, D, E, F	IPR029045	ClpP/crotonase-like domain superfamily	Homologous Superfamily
A, B, C, D, E A, B, C, D, E, F	IPR018376	Enoyl-CoA hydratase/isomerase, conserved site	Conserved Site
A, B, C, D, E A, B, C, D, E, F	IPR014748	Enoyl-CoA hydratase, C-terminal	Homologous Superfamily

Pharos: Disease Associations Pharos Homepage Annotation

Chains	Drug Target	Associated Disease
A, B, C, D, E A, B, C, D, E, F	Pharos: Q13825	3-methylglutaconic aciduria type 1 ulcerative colitis gastric carcinoma inflammatory bowel disease malignant ependymoma oligodendroglioma Pick disease tuberculosis astrocytic tumor glioblastoma intellectual disability malignant glioma ovarian cancer pilocytic astrocytoma atypical teratoid rhabdoid tumor 3-methylglutaconic aciduria type 1 ulcerative colitis gastric carcinoma inflammatory bowel disease malignant ependymoma oligodendroglioma Pick disease tuberculosis astrocytic tumor glioblastoma intellectual disability malignant glioma ovarian cancer pilocytic astrocytoma atypical teratoid rhabdoid tumor Hyperammonemia large cell medulloblastoma medulloblastoma pancreatic ductal adenocarcinoma transitional cell carcinoma

Structure Motif Annotation: Mechanism and Catalytic Site Atlas M-CSA Database Homepage

Chains	Enzyme Name	Description	Catalytic Residues
A	methylglutaconyl-CoA hydratase M-CSA #343	AU binding protein/enoyl-coA hydratase (AUH) is from Homo sapiens. It binds to AUUU repeats in RNA and also weakly catalyses the hydration of 2-trans-enoyl-coenzyme A (enoyl-coA). The hydration of enoyl-coA is necessary for the break-down of fatty acids. [PMID:12655555]. This entry represents the 3-hydroxy-3-methylglutaconyl- CoA dehydratase activity. It is a member of the crotonase superfamily.	Defined by 4 residues: ALA:A-74 [auth A-141]GLY:A-119 [auth A-186]GLU:A-122 [auth A-189]GLU:A-142 [auth A-209] \| Explore in 3D: M-CSA Motif Definition Search M-CSA Motif EC: 4.2.1.17 (PDB Primary Data) Search M-CSA Motif + EC 4.2.1.17

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 R01GM133198.