Showing entry for Ornithine carbamoyltransferase, mitochondrial


　 　 　 　 　 　 　 　 　 　 　 　
General Target Information
BXGT IdBXGT005551
Protein NameOrnithine carbamoyltransferase, mitochondrial
Uniport IdP00480
GeneOTC
Gene Id5009
DomainOTCace; OTCace_N
Pfam PF00185   PF02729  
OrganismHomo sapiens (Human)
Target protein  affiliated  biological  pathways
KEGG
Class Subclass KEGG Id Pathway Name
1. Metabolism 1.5 Amino acid metabolism hsa00220 Arginine biosynthesis
1. Metabolism 1.0 Global and overview maps hsa01100 Metabolic pathways
1. Metabolism 1.0 Global and overview maps hsa01230 Biosynthesis of amino acids
Gene Ontology
GO Type GO Id Gene Ontology
Biological Process GO:0097272 ammonia homeostasis
Biological Process GO:0055081 anion homeostasis
Biological Process GO:0042450 arginine biosynthetic process via ornithine
Biological Process GO:0019240 citrulline biosynthetic process
Biological Process GO:0001889 liver development
Biological Process GO:0007494 midgut development
Biological Process GO:0006593 ornithine catabolic process
Biological Process GO:0070781 response to biotin
Biological Process GO:0042493 response to drug
Biological Process GO:0032868 response to insulin
Biological Process GO:0010043 response to zinc ion
Biological Process GO:0000050 urea cycle
molecular function GO:0016597 amino acid binding
molecular function GO:0042802 identical protein binding
molecular function GO:0004585 ornithine carbamoyltransferase activity
molecular function GO:0042301 phosphate ion binding
molecular function GO:0005543 phospholipid binding
cellular component GO:0043231 intracellular membrane-bounded organelle
cellular component GO:0005743 mitochondrial inner membrane
cellular component GO:0005759 mitochondrial matrix
cellular component GO:0005739 mitochondrion
Reactome
Pathway Id Pathway Name
R-HSA-1268020 Mitochondrial protein import
R-HSA-1268020 Mitochondrial protein import
R-HSA-1430728 Metabolism
R-HSA-70635 Urea cycle
R-HSA-71291 Metabolism of amino acids and derivatives
R-HSA-9609507 Protein localization
R-HSA-9609507 Protein localization
Target protein-related  diseases
Disease
CUI Id BXGD Id Disease Name Disease Class
C0001122 BXGD000023 Acidosis Nutritional and Metabolic Diseases
C0002064 BXGD000100 Alkalosis, Respiratory Nutritional and Metabolic Diseases; Respiratory Tract Diseases
C0002395 BXGD000111 Alzheimer's Disease Nervous System Diseases; Mental Disorders
C0004134 BXGD000256 Ataxia Pathological Conditions, Signs and Symptoms; Nervous System Diseases
C0006111 BXGD000369 Brain Diseases Nervous System Diseases
C0006114 BXGD000371 Cerebral Edema Nervous System Diseases
C0006118 BXGD000372 Brain Neoplasms Neoplasms; Nervous System Diseases
C0006826 BXGD000408 Malignant Neoplasms Neoplasms
C0008370 BXGD000534 Cholestasis Digestive System Diseases
C0008626 BXGD000559 Congenital chromosomal disease Congenital, Hereditary, and Neonatal Diseases and Abnormalities
C0009241 BXGD000595 Cognition Disorders Mental Disorders
C0009402 BXGD000605 Colorectal Carcinoma Digestive System Diseases; Neoplasms
C0009421 BXGD000608 Comatose Pathological Conditions, Signs and Symptoms; Nervous System Diseases
C0010054 BXGD000647 Coronary Arteriosclerosis Cardiovascular Diseases
C0010068 BXGD000648 Coronary heart disease Cardiovascular Diseases
C0011853 BXGD000752 Diabetes Mellitus, Experimental Nutritional and Metabolic Diseases; Endocrine System Diseases
C0013264 BXGD000808 Muscular Dystrophy, Duchenne Congenital, Hereditary, and Neonatal Diseases and Abnormalities; Musculoskeletal Diseases; Nervous System Diseases
C0013390 BXGD000828 Dysmenorrhea Pathological Conditions, Signs and Symptoms
C0015544 BXGD001003 Failure to Thrive Pathological Conditions, Signs and Symptoms
C0015695 BXGD001013 Fatty Liver Digestive System Diseases
C0015696 BXGD001014 Fatty Liver, Alcoholic Digestive System Diseases; Chemically-Induced Disorders
C0015934 BXGD001026 Fetal Growth Retardation Pathological Conditions, Signs and Symptoms; Congenital, Hereditary, and Neonatal Diseases and Abnormalities; Female Urogenital Diseases and Pregnancy Complications
C0015967 BXGD001030 Fever Pathological Conditions, Signs and Symptoms
C0016952 BXGD001085 Galactosemias Congenital, Hereditary, and Neonatal Diseases and Abnormalities; Nutritional and Metabolic Diseases; Nervous System Diseases
C0018203 BXGD001184 Chronic granulomatous disease Congenital, Hereditary, and Neonatal Diseases and Abnormalities; Immune System Diseases; Hemic and Lymphatic Diseases
C0019158 BXGD001293 Hepatitis Digestive System Diseases
C0019188 BXGD001297 Hepatitis, Animal Digestive System Diseases; Infections; Animal Diseases
C0020538 BXGD001422 Hypertensive disease Cardiovascular Diseases
C0020615 BXGD001445 Hypoglycemia Nutritional and Metabolic Diseases
C0020619 BXGD001447 Hypogonadism Endocrine System Diseases
C0022107 BXGD001530 Irritable Mood Behavior and Behavior Mechanisms
C0022735 BXGD001581 Klinefelter Syndrome Congenital, Hereditary, and Neonatal Diseases and Abnormalities; Female Urogenital Diseases and Pregnancy Complications; Male Urogenital Diseases; Endocrine System Diseases
C0023380 BXGD001640 Lethargy Pathological Conditions, Signs and Symptoms; Nervous System Diseases; Behavior and Behavior Mechanisms
C0023449 BXGD001650 Acute lymphocytic leukemia Neoplasms; Immune System Diseases; Hemic and Lymphatic Diseases
C0023452 BXGD001651 Childhood Acute Lymphoblastic Leukemia Neoplasms; Immune System Diseases; Hemic and Lymphatic Diseases
C0023890 BXGD001713 Liver Cirrhosis Pathological Conditions, Signs and Symptoms; Digestive System Diseases
C0023893 BXGD001716 Liver Cirrhosis, Experimental Pathological Conditions, Signs and Symptoms; Digestive System Diseases
C0023895 BXGD001717 Liver diseases Digestive System Diseases
C0023896 BXGD001718 Alcoholic Liver Diseases Digestive System Diseases; Chemically-Induced Disorders
C0025517 BXGD001875 Metabolic Diseases Nutritional and Metabolic Diseases
C0025521 BXGD001876 Inborn Errors of Metabolism Congenital, Hereditary, and Neonatal Diseases and Abnormalities; Nutritional and Metabolic Diseases
C0027019 BXGD001961 Myelomonocytic leukemia Neoplasms
C0027092 BXGD001971 Myopia Eye Diseases
C0027651 BXGD002009 Neoplasms Neoplasms
C0027746 BXGD002032 Nerve Degeneration Pathological Conditions, Signs and Symptoms
C0028077 BXGD002069 Nyctalopia Eye Diseases
C0028754 BXGD002082 Obesity Pathological Conditions, Signs and Symptoms; Nutritional and Metabolic Diseases
C0030193 BXGD002191 Pain Pathological Conditions, Signs and Symptoms
C0030305 BXGD002206 Pancreatitis Digestive System Diseases
C0031090 BXGD002280 Periodontal Diseases Stomatognathic Diseases
C0031350 BXGD002300 Pharyngitis Infections; Respiratory Tract Diseases; Stomatognathic Diseases; Otorhinolaryngologic Diseases
C0031485 BXGD002303 Phenylketonurias Congenital, Hereditary, and Neonatal Diseases and Abnormalities; Nutritional and Metabolic Diseases; Nervous System Diseases
C0034194 BXGD002472 Pyloric Stenosis Digestive System Diseases
C0035126 BXGD002509 Reperfusion Injury Pathological Conditions, Signs and Symptoms; Cardiovascular Diseases
C0035334 BXGD002539 Retinitis Pigmentosa Congenital, Hereditary, and Neonatal Diseases and Abnormalities; Eye Diseases
C0036572 BXGD002625 Seizures Pathological Conditions, Signs and Symptoms; Nervous System Diseases
C0036982 BXGD002651 Shock, Hemorrhagic Pathological Conditions, Signs and Symptoms
C0037090 BXGD002665 Signs and Symptoms, Respiratory Pathological Conditions, Signs and Symptoms
C0038002 BXGD002723 Splenomegaly Pathological Conditions, Signs and Symptoms
C0040264 BXGD002857 Tinnitus Pathological Conditions, Signs and Symptoms; Nervous System Diseases; Otorhinolaryngologic Diseases
C0042963 BXGD003018 Vomiting Pathological Conditions, Signs and Symptoms
C0043194 BXGD003036 Wiskott-Aldrich Syndrome Congenital, Hereditary, and Neonatal Diseases and Abnormalities; Immune System Diseases; Hemic and Lymphatic Diseases
C0085110 BXGD003126 Severe Combined Immunodeficiency Congenital, Hereditary, and Neonatal Diseases and Abnormalities; Nutritional and Metabolic Diseases; Immune System Diseases
C0085584 BXGD003195 Encephalopathies Nervous System Diseases
C0085605 BXGD003200 Liver Failure Digestive System Diseases
C0149925 BXGD003387 Small cell carcinoma of lung Neoplasms; Respiratory Tract Diseases
C0154246 BXGD003709 Urea Cycle Disorders, Inborn Congenital, Hereditary, and Neonatal Diseases and Abnormalities; Nutritional and Metabolic Diseases; Nervous System Diseases
C0162311 BXGD003935 Androgenetic Alopecia Pathological Conditions, Signs and Symptoms; Skin and Connective Tissue Diseases
C0162429 BXGD003942 Malnutrition Nutritional and Metabolic Diseases
C0162557 BXGD003956 Liver Failure, Acute Digestive System Diseases
C0175683 BXGD003998 Citrullinemia Congenital, Hereditary, and Neonatal Diseases and Abnormalities; Nutritional and Metabolic Diseases; Nervous System Diseases
C0206624 BXGD004190 Hepatoblastoma Neoplasms
C0220756 BXGD004338 Niemann-Pick Disease, Type C Congenital, Hereditary, and Neonatal Diseases and Abnormalities; Nutritional and Metabolic Diseases; Nervous System Diseases; Hemic and Lymphatic Diseases
C0220766 BXGD004341 Congenital hypoplasia of adrenal gland Congenital, Hereditary, and Neonatal Diseases and Abnormalities; Endocrine System Diseases
C0220994 BXGD004357 Hyperammonemia Pathological Conditions, Signs and Symptoms
C0221228 BXGD004412 Comedone Skin and Connective Tissue Diseases
C0238621 BXGD004947 Aminoaciduria Congenital, Hereditary, and Neonatal Diseases and Abnormalities; Nutritional and Metabolic Diseases
C0239946 BXGD005010 Fibrosis, Liver Pathological Conditions, Signs and Symptoms; Digestive System Diseases
C0242429 BXGD005166 Sore Throat Infections; Respiratory Tract Diseases; Stomatognathic Diseases; Otorhinolaryngologic Diseases
C0242510 BXGD005173 Drug usage Chemically-Induced Disorders; Mental Disorders
C0242966 BXGD005201 Systemic Inflammatory Response Syndrome Pathological Conditions, Signs and Symptoms
C0243026 BXGD005210 Sepsis Pathological Conditions, Signs and Symptoms; Infections
C0262405 BXGD005237 Cerebral dysfunction Nervous System Diseases
C0265216 BXGD005466 X-linked hydrocephalus syndrome Congenital, Hereditary, and Neonatal Diseases and Abnormalities; Nervous System Diseases
C0268130 BXGD005829 Hereditary orotic aciduria, type 1 Congenital, Hereditary, and Neonatal Diseases and Abnormalities; Nutritional and Metabolic Diseases
C0268151 BXGD005838 Classical galactosemia Congenital, Hereditary, and Neonatal Diseases and Abnormalities; Nutritional and Metabolic Diseases; Nervous System Diseases
C0268490 BXGD005962 Tyrosinemia, Type I Congenital, Hereditary, and Neonatal Diseases and Abnormalities; Nutritional and Metabolic Diseases; Nervous System Diseases
C0268505 BXGD005968 Ocular albinism, type II Congenital, Hereditary, and Neonatal Diseases and Abnormalities; Nutritional and Metabolic Diseases; Eye Diseases; Skin and Connective Tissue Diseases
C0268542 BXGD005977 Ornithine carbamoyltransferase deficiency Congenital, Hereditary, and Neonatal Diseases and Abnormalities; Nutritional and Metabolic Diseases; Nervous System Diseases
C0268548 BXGD005980 Hyperargininemia Congenital, Hereditary, and Neonatal Diseases and Abnormalities; Nutritional and Metabolic Diseases; Nervous System Diseases
C0268601 BXGD006002 HMG CoA lyase deficiency Congenital, Hereditary, and Neonatal Diseases and Abnormalities; Nutritional and Metabolic Diseases
C0339528 BXGD007261 X-linked retinitis pigmentosa Congenital, Hereditary, and Neonatal Diseases and Abnormalities; Eye Diseases
C0342482 BXGD007486 X-linked Adrenal Hypoplasia Endocrine System Diseases
C0376618 BXGD008003 Endotoxemia Pathological Conditions, Signs and Symptoms; Infections
C0398762 BXGD008226 Properdin deficiency disease
C0424605 BXGD008535 Developmental delay (disorder) Mental Disorders
C0432474 BXGD008793 Klinefelter's syndrome - male with more than two X chromosomes Congenital, Hereditary, and Neonatal Diseases and Abnormalities; Female Urogenital Diseases and Pregnancy Complications; Male Urogenital Diseases; Endocrine System Diseases
C0522254 BXGD009199 Analgesic Overuse Headache Nervous System Diseases
C0524910 BXGD009248 Hepatitis C, Chronic Digestive System Diseases; Infections
C0557874 BXGD009444 Global developmental delay
C0747845 BXGD010184 early pregnancy
C0750325 BXGD010233 Vomiting, recurrent
C0751606 BXGD010523 Adult Acute Lymphocytic Leukemia
C0751651 BXGD010539 Mitochondrial Diseases Nutritional and Metabolic Diseases
C0751753 BXGD010576 Carbamoyl-Phosphate Synthase I Deficiency Disease Congenital, Hereditary, and Neonatal Diseases and Abnormalities; Nutritional and Metabolic Diseases; Nervous System Diseases
C1306459 BXGD012361 Primary malignant neoplasm Neoplasms
C1318485 BXGD012394 Liver regeneration disorder Digestive System Diseases
C1456246 BXGD013110 Bacteroides fragilis infection in conditions classified elsewhere and of unspecified site
C1623038 BXGD013475 Cirrhosis Pathological Conditions, Signs and Symptoms
C1720189 BXGD013668 Episodic Ataxia Pathological Conditions, Signs and Symptoms; Nervous System Diseases
C1839531 BXGD014381 Protein avoidance
C1839532 BXGD014382 Low plasma citrulline
C1839533 BXGD014383 Hyperglutaminemia
C1839541 BXGD014384 Episodic ammonia intoxication
C1845667 BXGD014756 RETINITIS PIGMENTOSA 3 Congenital, Hereditary, and Neonatal Diseases and Abnormalities; Eye Diseases
C1845977 BXGD014769 X- linked recessive
C1846265 BXGD014795 Microphthalmia, syndromic 2 Congenital, Hereditary, and Neonatal Diseases and Abnormalities; Eye Diseases; Cardiovascular Diseases
C1848172 BXGD014908 NIGHT BLINDNESS, CONGENITAL STATIONARY, TYPE 2A Congenital, Hereditary, and Neonatal Diseases and Abnormalities; Eye Diseases
C1857276 BXGD015688 Trichohepatoenteric Syndrome Pathological Conditions, Signs and Symptoms; Congenital, Hereditary, and Neonatal Diseases and Abnormalities; Female Urogenital Diseases and Pregnancy Complications; Skin and Connective Tissue Diseases
C1865145 BXGD016294 Congenital disorder of glycosylation type 1B Congenital, Hereditary, and Neonatal Diseases and Abnormalities; Nutritional and Metabolic Diseases
C1956346 BXGD016627 Coronary Artery Disease Cardiovascular Diseases
C2239176 BXGD016965 Liver carcinoma Digestive System Diseases; Neoplasms
C2700617 BXGD017474 Irritation - emotion Behavior and Behavior Mechanisms
C2711227 BXGD017478 Steatohepatitis Digestive System Diseases
C2828000 BXGD017801 X-Linked Inherited Disorder Congenital, Hereditary, and Neonatal Diseases and Abnormalities
C2830004 BXGD017804 Somnolence Pathological Conditions, Signs and Symptoms
C3160897 BXGD018494 Opioid-Induced Constipation Pathological Conditions, Signs and Symptoms
C3542021 BXGD019101 Duchenne and Becker Muscular Dystrophy Congenital, Hereditary, and Neonatal Diseases and Abnormalities; Musculoskeletal Diseases; Nervous System Diseases
C3711543 BXGD019396 X-Linked Csnb Congenital, Hereditary, and Neonatal Diseases and Abnormalities; Eye Diseases
C3714756 BXGD019429 Intellectual Disability Pathological Conditions, Signs and Symptoms; Nervous System Diseases; Mental Disorders; Behavior and Behavior Mechanisms
C4023070 BXGD021095 Abnormality of ornithine metabolism
C4087436 BXGD022148 HIV viraemia Infections
C4529962 BXGD023178 Fatty Liver Disease
C4552011 BXGD023477 Gonadotropin deficiency
C4552810 BXGD023525 Irritability, CTCAE
Target protein-related  compounds
Compounds
BXGC Id Compound Name Strcuture Molecular Weight
BXGC0006696 Phosphoric acid 98
BXGC0014919 Citrulline 175.1
BXGC0052927 Ornithine 132.09
The  target protein-related  foods
Foods
BXGF Id Food Name Scientific Name Food Group Number of compound? Number of compound in food, higher numbers indicate the food are more associated with the protein