1JDW

CRYSTAL STRUCTURE AND MECHANISM OF L-ARGININE: GLYCINE AMIDINOTRANSFERASE: A MITOCHONDRIAL ENZYME INVOLVED IN CREATINE BIOSYNTHESIS

External Resource: Annotation

Domain Annotation: SCOP/SCOPe Classification
Domain Annotation: SCOP2 Classification
Domain Annotation: ECOD Classification
Domain Annotation: CATH
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	d1jdwa_	Alpha and beta proteins (a+b)	Pentein, beta/alpha-propeller	Pentein	Amidinotransferase	L-arginine: glycine amidinotransferase	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	Amidinotransferase	8023851	4001986	SCOP2 (2022-06-29)
A	SCOP2 Superfamily	Pentein	8036231	3000640	SCOP2 (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	F_UNCLASSIFIED	e1jdwA1	A: a+b duplicates or obligate multimers	X: Pentein (From Topology)	H: Pentein (From Topology)	T: Pentein	F: F_UNCLASSIFIED	ECOD (1.6)

Domain Annotation: CATH CATH Database Homepage

Chain	Domain	Class	Architecture	Topology	Homology	Provenance Source (Version)
A	3.75.10.10	Alpha Beta	5-stranded Propeller	L-arginine/glycine Amidinotransferase	Chain A	CATH (4.3.0)

Gene Ontology: Gene Product Annotation Gene Ontology Database Homepage

Chains	Polymer	Molecular Function	Biological Process	Cellular Component
A	L-ARGININE\:GLYCINE AMIDINOTRANSFERASE	amidinotransferase activity transferase activity, transferring one-carbon groups glycine amidinotransferase activity transferase activity catalytic activity	multicellular organismal process regulation of biological process homeostatic process biological regulation positive regulation of biological process adaptive thermogenesis positive regulation of metabolic process multicellular organismal-level homeostasis regulation of cold-induced thermogenesis cold-induced thermogenesis temperature homeostasis positive regulation of cold-induced thermogenesis positive regulation of multicellular organismal process metabolic process multicellular organismal process regulation of biological process homeostatic process biological regulation positive regulation of biological process adaptive thermogenesis positive regulation of metabolic process multicellular organismal-level homeostasis regulation of cold-induced thermogenesis cold-induced thermogenesis temperature homeostasis positive regulation of cold-induced thermogenesis positive regulation of multicellular organismal process metabolic process regulation of multicellular organismal process regulation of metabolic process organonitrogen compound metabolic process creatine metabolic process oxoacid metabolic process cellular metabolic process carboxylic acid metabolic process organic acid metabolic process cellular modified amino acid metabolic process organic substance metabolic process cellular process small molecule metabolic process monocarboxylic acid metabolic process muscle atrophy system process muscle adaptation response to stimulus muscle system process monocarboxylic acid biosynthetic process small molecule biosynthetic process cellular biosynthetic process creatine biosynthetic process organonitrogen compound biosynthetic process organic acid biosynthetic process carboxylic acid biosynthetic process biosynthetic process cellular modified amino acid biosynthetic process organic substance biosynthetic process behavior learning or memory nervous system process cognition	membrane-bounded organelle intracellular organelle intracellular anatomical structure mitochondrion organelle cytoplasm cellular anatomical entity intracellular membrane-bounded organelle mitochondrial membrane organelle inner membrane membrane mitochondrial inner membrane organelle envelope mitochondrial envelope membrane-bounded organelle intracellular organelle intracellular anatomical structure mitochondrion organelle cytoplasm cellular anatomical entity intracellular membrane-bounded organelle mitochondrial membrane organelle inner membrane membrane mitochondrial inner membrane organelle envelope mitochondrial envelope organelle membrane extracellular exosome extracellular vesicle extracellular organelle extracellular region extracellular space vesicle extracellular membrane-bounded organelle mitochondrial intermembrane space membrane-enclosed lumen intracellular organelle lumen organelle envelope lumen organelle lumen

InterPro: Protein Family Classification InterPro Database Homepage

Chains	Accession	Name	Type
A	IPR033195	Glycine/inosamine-phosphate amidinotransferase	Family

Pharos: Disease Associations Pharos Homepage Annotation

Chains	Drug Target	Associated Disease
A	Pharos: P50440	AGAT deficiency carcinoma congestive heart failure sarcomatoid carcinoma Necrosis kidney disease intellectual disability large cell medulloblastoma lung cancer adenoma medulloblastoma myocardial ischemia osteosarcoma pancreatic intraductal papillary-mucinous carcinoma pilocytic astrocytoma AGAT deficiency carcinoma congestive heart failure sarcomatoid carcinoma Necrosis kidney disease intellectual disability large cell medulloblastoma lung cancer adenoma medulloblastoma myocardial ischemia osteosarcoma pancreatic intraductal papillary-mucinous carcinoma pilocytic astrocytoma cutaneous lupus erythematosus guanidinoacetate methyltransferase deficiency interstitial cystitis malignant glioma mammary neoplasms, animal atypical teratoid rhabdoid tumor ovarian cancer coronary artery disease pancreatic intraductal papillary-mucinous neoplasm psoriasis Down syndrome subependymal giant cell astrocytoma inborn errors of metabolism lung adenocarcinoma malignant pancreatic neoplasm breast cancer nephrosclerosis chronic kidney disease pancreatic ductal adenocarcinoma creatine transporter deficiency pancreatic intraductal papillary-mucinous neoplasm with low grade dysplasia sarcoidosis undifferentiated carcinoma heart failure

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

Chains	Enzyme Name	Description	Catalytic Residues
A	glycine amidinotransferase M-CSA #18	Glycine amidinotransferase, also known as L-arginine:glycine amidinotransferase (AT) catalyses the committed step in creatine biosynthesis. The enzyme does this by forming guanidinoacetic acid, the immediate precursor of creatine. Both creatine and its phosphorylated form play an essential role in the energy metabolism of muscle and nerve tissues, acting as a dynamic reservoir of high-energy phosphate which buffers the rapid fluctuations of the ATP/ADP ratio during muscle and nerve action.	Defined by 5 residues: ASP:A-170ASP:A-254HIS:A-303ASP:A-305CYS:A-407 \| Explore in 3D: M-CSA Motif Definition Search M-CSA Motif EC: 2.1.4.1 (PDB Primary Data) Search M-CSA Motif + EC 2.1.4.1

RCSB PDB Core Operations are funded by the National Science Foundation (DBI-1832184), 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.