Showing entry for Propionyl-CoA carboxylase alpha chain, mitochondrial


　 　 　 　 　 　 　 　 　 　 　 　
General Target Information
BXGT IdBXGT006334
Protein NamePropionyl-CoA carboxylase alpha chain, mitochondrial
Uniport IdP05165
GenePCCA
Gene Id5095
DomainBiotin_carb_C; Biotin_carb_N; Biotin_lipoyl; CPSase_L_D2; PCC_BT
Pfam PF02785   PF00289   PF00364   PF02786   PF18140  
OrganismHomo sapiens (Human)
Target protein  affiliated  biological  pathways
KEGG
Class Subclass KEGG Id Pathway Name
1. Metabolism 1.5 Amino acid metabolism hsa00280 Valine, leucine and isoleucine degradation
1. Metabolism 1.1 Carbohydrate metabolism hsa00630 Glyoxylate and dicarboxylate metabolism
1. Metabolism 1.1 Carbohydrate metabolism hsa00640 Propanoate metabolism
1. Metabolism 1.0 Global and overview maps hsa01100 Metabolic pathways
1. Metabolism 1.0 Global and overview maps hsa01200 Carbon metabolism
Gene Ontology
GO Type GO Id Gene Ontology
Biological Process GO:0006768 biotin metabolic process
Biological Process GO:0019626 short-chain fatty acid catabolic process
molecular function GO:0005524 ATP binding
molecular function GO:0009374 biotin binding
molecular function GO:0016421 CoA carboxylase activity
molecular function GO:0019899 enzyme binding
molecular function GO:0046872 metal ion binding
molecular function GO:0004658 propionyl-CoA carboxylase activity
cellular component GO:0005829 cytosol
cellular component GO:0005759 mitochondrial matrix
cellular component GO:0005739 mitochondrion
Reactome
Pathway Id Pathway Name
R-HSA-1430728 Metabolism
R-HSA-1643685 Disease
R-HSA-196780 Biotin transport and metabolism
R-HSA-196849 Metabolism of water-soluble vitamins and cofactors
R-HSA-196854 Metabolism of vitamins and cofactors
R-HSA-3296482 Defects in vitamin and cofactor metabolism
R-HSA-3323169 Defects in biotin (Btn) metabolism
R-HSA-3371599 Defective HLCS causes multiple carboxylase deficiency
R-HSA-556833 Metabolism of lipids
R-HSA-5668914 Diseases of metabolism
R-HSA-71032 Propionyl-CoA catabolism
R-HSA-77289 Mitochondrial Fatty Acid Beta-Oxidation
R-HSA-8978868 Fatty acid metabolism
Target protein-related  diseases
Disease
CUI Id BXGD Id Disease Name Disease Class
C0001125 BXGD000024 Acidosis, Lactic Nutritional and Metabolic Diseases
C0001418 BXGD000050 Adenocarcinoma Neoplasms
C0002871 BXGD000132 Anemia Hemic and Lymphatic Diseases
C0003578 BXGD000211 Apnea Pathological Conditions, Signs and Symptoms; Respiratory Tract Diseases
C0003811 BXGD000222 Cardiac Arrhythmia Pathological Conditions, Signs and Symptoms; Cardiovascular Diseases
C0003872 BXGD000235 Arthritis, Psoriatic Skin and Connective Tissue Diseases; Musculoskeletal Diseases
C0009402 BXGD000605 Colorectal Carcinoma Digestive System Diseases; Neoplasms
C0009421 BXGD000608 Comatose Pathological Conditions, Signs and Symptoms; Nervous System Diseases
C0009806 BXGD000633 Constipation Pathological Conditions, Signs and Symptoms
C0011175 BXGD000701 Dehydration Pathological Conditions, Signs and Symptoms; Nutritional and Metabolic Diseases
C0013336 BXGD000815 Dwarfism Congenital, Hereditary, and Neonatal Diseases and Abnormalities; Musculoskeletal Diseases; Endocrine System Diseases
C0013421 BXGD000837 Dystonia Pathological Conditions, Signs and Symptoms; Nervous System Diseases
C0013595 BXGD000858 Eczema Skin and Connective Tissue Diseases
C0015544 BXGD001003 Failure to Thrive Pathological Conditions, Signs and Symptoms
C0019209 BXGD001305 Hepatomegaly Pathological Conditions, Signs and Symptoms; Digestive System Diseases
C0020615 BXGD001445 Hypoglycemia Nutritional and Metabolic Diseases
C0023380 BXGD001640 Lethargy Pathological Conditions, Signs and Symptoms; Nervous System Diseases; Behavior and Behavior Mechanisms
C0024623 BXGD001791 Malignant neoplasm of stomach Digestive System Diseases; Neoplasms
C0025517 BXGD001875 Metabolic Diseases Nutritional and Metabolic Diseases
C0027947 BXGD002061 Neutropenia Hemic and Lymphatic Diseases
C0029456 BXGD002157 Osteoporosis Nutritional and Metabolic Diseases; Musculoskeletal Diseases
C0030305 BXGD002206 Pancreatitis Digestive System Diseases
C0030312 BXGD002207 Pancytopenia Hemic and Lymphatic Diseases
C0034951 BXGD002498 Refractive Errors Eye Diseases
C0036572 BXGD002625 Seizures Pathological Conditions, Signs and Symptoms; Nervous System Diseases
C0037369 BXGD002691 Smoking Behavior and Behavior Mechanisms
C0040034 BXGD002836 Thrombocytopenia Hemic and Lymphatic Diseases
C0042963 BXGD003018 Vomiting Pathological Conditions, Signs and Symptoms
C0220981 BXGD004348 Metabolic acidosis Nutritional and Metabolic Diseases
C0220994 BXGD004357 Hyperammonemia Pathological Conditions, Signs and Symptoms
C0231835 BXGD004527 Tachypnea Pathological Conditions, Signs and Symptoms; Respiratory Tract Diseases
C0232462 BXGD004542 Decrease in appetite Digestive System Diseases; Nervous System Diseases; Mental Disorders
C0235946 BXGD004789 Cerebral atrophy Pathological Conditions, Signs and Symptoms; Nervous System Diseases
C0241775 BXGD005113 Organic aciduria
C0268559 BXGD005984 Hyperglycinemia Congenital, Hereditary, and Neonatal Diseases and Abnormalities; Nutritional and Metabolic Diseases
C0268579 BXGD005995 Propionic acidemia Congenital, Hereditary, and Neonatal Diseases and Abnormalities; Nutritional and Metabolic Diseases
C0276096 BXGD006401 Mastitis-metritis-agalactia syndrome Female Urogenital Diseases and Pregnancy Complications; Infections; Skin and Connective Tissue Diseases
C0349588 BXGD007933 Short stature
C0424230 BXGD008522 Motor retardation Pathological Conditions, Signs and Symptoms; Nervous System Diseases; Behavior and Behavior Mechanisms
C0543541 BXGD009285 HYPERGLYCINURIA (disorder) Congenital, Hereditary, and Neonatal Diseases and Abnormalities; Nutritional and Metabolic Diseases; Female Urogenital Diseases and Pregnancy Complications; Male Urogenital Diseases
C0557874 BXGD009444 Global developmental delay
C0596887 BXGD009648 mathematical ability
C0699791 BXGD009867 Stomach Carcinoma Digestive System Diseases; Neoplasms
C0878544 BXGD011368 Cardiomyopathies Cardiovascular Diseases
C1306587 BXGD012368 Acute encephalopathy Nervous System Diseases
C1449563 BXGD013086 Cardiomyopathy, Familial Idiopathic Cardiovascular Diseases
C1838391 BXGD014313 Limb hypertonia Pathological Conditions, Signs and Symptoms; Nervous System Diseases
C1853743 BXGD015358 Muscular hypotonia of the trunk Pathological Conditions, Signs and Symptoms; Nervous System Diseases
C2674608 BXGD017250 Feeding difficulties in infancy
C2717876 BXGD017515 Propionicaciduria Congenital, Hereditary, and Neonatal Diseases and Abnormalities; Nutritional and Metabolic Diseases
C2919142 BXGD017867 Short Stature, CTCAE
C3151140 BXGD018399 PONTOCEREBELLAR HYPOPLASIA, TYPE 2D
C3714756 BXGD019429 Intellectual Disability Pathological Conditions, Signs and Symptoms; Nervous System Diseases; Mental Disorders; Behavior and Behavior Mechanisms
C4023616 BXGD021229 Abnormality of immune system physiology
C4025843 BXGD021823 Abnormality of refraction
C4551584 BXGD023362 Brain atrophy Nervous System Diseases
C4703632 BXGD023670 Increased level of hippuric acid in urine
Target protein-related  compounds
Compounds
BXGC Id Compound Name Strcuture Molecular Weight
BXGC0026123 Biotin 244.09
BXGC0030612 tetraethylene glycol 194.12
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