Showing entry for Pyruvate dehydrogenase protein X component, mitochondrial


　 　 　 　 　 　 　 　 　 　 　 　
General Target Information
BXGT IdBXGT003910
Protein NamePyruvate dehydrogenase protein X component, mitochondrial
Uniport IdO00330
GenePDHX
Gene Id8050
Domain2-oxoacid_dh; Biotin_lipoyl; E3_binding
Pfam PF00198   PF00364   PF02817  
OrganismHomo sapiens (Human)
Target protein  affiliated  biological  pathways
KEGG
Class Subclass KEGG Id Pathway Name
1. Metabolism 1.0 Global and overview maps hsa01100 Metabolic pathways
Gene Ontology
GO Type GO Id Gene Ontology
Biological Process GO:0061732 mitochondrial acetyl-CoA biosynthetic process from pyruvate
Biological Process GO:0006090 pyruvate metabolic process
molecular function GO:0016746 transferase activity, transferring acyl groups
cellular component GO:0005759 mitochondrial matrix
cellular component GO:0005739 mitochondrion
cellular component GO:0045254 pyruvate dehydrogenase complex
Reactome
Pathway Id Pathway Name
R-HSA-1428517 The citric acid (TCA) cycle and respiratory electron transport
R-HSA-1430728 Metabolism
R-HSA-162582 Signal Transduction
R-HSA-204174 Regulation of pyruvate dehydrogenase (PDH) complex
R-HSA-389661 Glyoxylate metabolism and glycine degradation
R-HSA-5362517 Signaling by Retinoic Acid
R-HSA-70268 Pyruvate metabolism
R-HSA-71291 Metabolism of amino acids and derivatives
R-HSA-71406 Pyruvate metabolism and Citric Acid (TCA) cycle
R-HSA-9006931 Signaling by Nuclear Receptors
Target protein-related  diseases
Disease
CUI Id BXGD Id Disease Name Disease Class
C0000846 BXGD000015 Agenesis
C0001125 BXGD000024 Acidosis, Lactic Nutritional and Metabolic Diseases
C0001418 BXGD000050 Adenocarcinoma Neoplasms
C0004134 BXGD000256 Ataxia Pathological Conditions, Signs and Symptoms; Nervous System Diseases
C0006142 BXGD000374 Malignant neoplasm of breast Neoplasms; Skin and Connective Tissue Diseases
C0006826 BXGD000408 Malignant Neoplasms Neoplasms
C0008312 BXGD000527 Primary biliary cirrhosis Pathological Conditions, Signs and Symptoms; Digestive System Diseases
C0009402 BXGD000605 Colorectal Carcinoma Digestive System Diseases; Neoplasms
C0009404 BXGD000606 Colorectal Neoplasms Digestive System Diseases; Neoplasms
C0011847 BXGD000749 Diabetes Endocrine System Diseases
C0011849 BXGD000751 Diabetes Mellitus Nutritional and Metabolic Diseases; Endocrine System Diseases
C0011854 BXGD000753 Diabetes Mellitus, Insulin-Dependent Nutritional and Metabolic Diseases; Immune System Diseases; Endocrine System Diseases
C0011860 BXGD000755 Diabetes Mellitus, Non-Insulin-Dependent Nutritional and Metabolic Diseases; Endocrine System Diseases
C0013274 BXGD000809 Patent ductus arteriosus Congenital, Hereditary, and Neonatal Diseases and Abnormalities; Cardiovascular Diseases
C0013421 BXGD000837 Dystonia Pathological Conditions, Signs and Symptoms; Nervous System Diseases
C0015934 BXGD001026 Fetal Growth Retardation Pathological Conditions, Signs and Symptoms; Congenital, Hereditary, and Neonatal Diseases and Abnormalities; Female Urogenital Diseases and Pregnancy Complications
C0016842 BXGD001083 Congenital pectus excavatum Congenital, Hereditary, and Neonatal Diseases and Abnormalities; Musculoskeletal Diseases
C0020456 BXGD001392 Hyperglycemia Nutritional and Metabolic Diseases
C0020459 BXGD001394 Hyperinsulinism Nutritional and Metabolic Diseases
C0020534 BXGD001421 Orbital separation excessive Congenital, Hereditary, and Neonatal Diseases and Abnormalities; Musculoskeletal Diseases
C0020796 BXGD001468 Profound Mental Retardation Pathological Conditions, Signs and Symptoms; Nervous System Diseases; Mental Disorders; Behavior and Behavior Mechanisms
C0021670 BXGD001509 insulinoma Digestive System Diseases; Neoplasms; Endocrine System Diseases
C0023264 BXGD001625 Leigh Disease Congenital, Hereditary, and Neonatal Diseases and Abnormalities; Nutritional and Metabolic Diseases; Nervous System Diseases
C0023892 BXGD001715 Biliary cirrhosis Pathological Conditions, Signs and Symptoms; Digestive System Diseases
C0024141 BXGD001740 Lupus Erythematosus, Systemic Skin and Connective Tissue Diseases; Immune System Diseases
C0025363 BXGD001867 Mental Retardation, Psychosocial Pathological Conditions, Signs and Symptoms; Nervous System Diseases; Mental Disorders; Behavior and Behavior Mechanisms
C0025517 BXGD001875 Metabolic Diseases Nutritional and Metabolic Diseases
C0025958 BXGD001882 Microcephaly Congenital, Hereditary, and Neonatal Diseases and Abnormalities; Musculoskeletal Diseases; Nervous System Diseases
C0027627 BXGD002006 Neoplasm Metastasis Pathological Conditions, Signs and Symptoms; Neoplasms
C0027651 BXGD002009 Neoplasms Neoplasms
C0027773 BXGD002035 Nesidioblastosis Congenital, Hereditary, and Neonatal Diseases and Abnormalities; Digestive System Diseases; Nutritional and Metabolic Diseases
C0029124 BXGD002113 Optic Atrophy Eye Diseases; Nervous System Diseases
C0030297 BXGD002204 Pancreatic Neoplasm Digestive System Diseases; Neoplasms; Endocrine System Diseases
C0034345 BXGD002477 Pyruvate Dehydrogenase Complex Deficiency Disease Congenital, Hereditary, and Neonatal Diseases and Abnormalities; Nutritional and Metabolic Diseases; Nervous System Diseases
C0036572 BXGD002625 Seizures Pathological Conditions, Signs and Symptoms; Nervous System Diseases
C0037772 BXGD002703 Spastic Paraplegia Pathological Conditions, Signs and Symptoms; Nervous System Diseases
C0038826 BXGD002779 Superinfection Infections
C0079731 BXGD003087 B-Cell Lymphomas Neoplasms; 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
C0149521 BXGD003328 Pancreatitis, Chronic Digestive System Diseases
C0158981 BXGD003914 Neonatal diabetes mellitus Congenital, Hereditary, and Neonatal Diseases and Abnormalities; Nutritional and Metabolic Diseases; Endocrine System Diseases
C0220981 BXGD004348 Metabolic acidosis Nutritional and Metabolic Diseases
C0231686 BXGD004508 Gait, Unsteady Pathological Conditions, Signs and Symptoms; Nervous System Diseases
C0235974 BXGD004796 Pancreatic carcinoma Digestive System Diseases; Neoplasms; Endocrine System Diseases
C0240635 BXGD005047 Byzanthine arch palate Congenital, Hereditary, and Neonatal Diseases and Abnormalities; Musculoskeletal Diseases; Nervous System Diseases; Stomatognathic Diseases
C0265535 BXGD005544 Trigonocephaly Congenital, Hereditary, and Neonatal Diseases and Abnormalities; Musculoskeletal Diseases
C0268630 BXGD006016 Hyper-beta-alaninemia Pathological Conditions, Signs and Symptoms; Nervous System Diseases; Mental Disorders
C0271633 BXGD006223 Disorder of endocrine pancreas Pathological Conditions, Signs and Symptoms; Digestive System Diseases; Endocrine System Diseases
C0281361 BXGD006774 Adenocarcinoma of pancreas Digestive System Diseases; Neoplasms; Endocrine System Diseases
C0342276 BXGD007449 Maturity onset diabetes mellitus in young Nutritional and Metabolic Diseases; Endocrine System Diseases
C0346647 BXGD007832 Malignant neoplasm of pancreas Digestive System Diseases; Neoplasms; Endocrine System Diseases
C0424605 BXGD008535 Developmental delay (disorder) Mental Disorders
C0426970 BXGD008598 Spastic Quadriplegia Pathological Conditions, Signs and Symptoms; Nervous System Diseases
C0431368 BXGD008673 Partial agenesis of corpus callosum Pathological Conditions, Signs and Symptoms; Congenital, Hereditary, and Neonatal Diseases and Abnormalities; Nervous System Diseases
C0494165 BXGD009022 Secondary malignant neoplasm of liver Digestive System Diseases; Neoplasms
C0497202 BXGD009055 Abnormal ocular motility
C0556280 BXGD009440 Gross motor impairment
C0557874 BXGD009444 Global developmental delay
C0596263 BXGD009639 Carcinogenesis Pathological Conditions, Signs and Symptoms; Neoplasms
C0677898 BXGD009735 invasive cancer Neoplasms
C0678222 BXGD009749 Breast Carcinoma Neoplasms; Skin and Connective Tissue Diseases
C0678230 BXGD009750 Congenital Epicanthus
C0740457 BXGD010000 Malignant neoplasm of kidney Neoplasms; Female Urogenital Diseases and Pregnancy Complications; Male Urogenital Diseases
C0878500 BXGD011365 Intraepithelial Neoplasia Neoplasms
C0887833 BXGD011398 Carcinoma, Pancreatic Ductal Digestive System Diseases; Neoplasms; Endocrine System Diseases
C0917816 BXGD011419 Mental deficiency Pathological Conditions, Signs and Symptoms; Nervous System Diseases; Mental Disorders; Behavior and Behavior Mechanisms
C1269955 BXGD012005 Tumor Cell Invasion
C1301034 BXGD012282 Pancreatic intraepithelial neoplasia Neoplasms
C1306459 BXGD012361 Primary malignant neoplasm Neoplasms
C1319315 BXGD012415 Adenocarcinoma of large intestine Digestive System Diseases; Neoplasms
C1335168 BXGD012769 Ovarian mucinous tumor
C1335302 BXGD012775 Pancreatic Ductal Adenocarcinoma Digestive System Diseases; Neoplasms; Endocrine System Diseases
C1458155 BXGD013136 Mammary Neoplasms Neoplasms; Skin and Connective Tissue Diseases
C1511789 BXGD013183 Desmoplastic
C1832661 BXGD013866 ANOPHTHALMIA AND PULMONARY HYPOPLASIA Congenital, Hereditary, and Neonatal Diseases and Abnormalities; Eye Diseases; Respiratory Tract Diseases
C1833382 BXGD013922 MATURITY-ONSET DIABETES OF THE YOUNG, TYPE IV (disorder) Nutritional and Metabolic Diseases; Endocrine System Diseases
C1833431 BXGD013924 Subependymal cysts Pathological Conditions, Signs and Symptoms; Nervous System Diseases
C1839413 BXGD014372 Pyruvate Dehydrogenase E1 Alpha Deficiency Nutritional and Metabolic Diseases
C1839888 BXGD014424 Decreased activity of the pyruvate dehydrogenase complex
C1849488 BXGD015058 Increased serum pyruvate
C1855553 BXGD015510 Pyruvate Dehydrogenase E3-Binding Protein Deficiency Congenital, Hereditary, and Neonatal Diseases and Abnormalities; Nutritional and Metabolic Diseases; Nervous System Diseases
C1861403 BXGD016045 Variable expressivity
C1867864 BXGD016472 Poor fine motor coordination
C2267233 BXGD017017 Neonatal Hypotonia Pathological Conditions, Signs and Symptoms; Nervous System Diseases
C3149841 BXGD018287 POLYCYSTIC KIDNEY DISEASE 1 Congenital, Hereditary, and Neonatal Diseases and Abnormalities; Female Urogenital Diseases and Pregnancy Complications; Male Urogenital Diseases
C3714756 BXGD019429 Intellectual Disability Pathological Conditions, Signs and Symptoms; Nervous System Diseases; Mental Disorders; Behavior and Behavior Mechanisms
C3888018 BXGD019942 Congenital Hyperinsulinism Congenital, Hereditary, and Neonatal Diseases and Abnormalities; Digestive System Diseases; Nutritional and Metabolic Diseases
C4025276 BXGD021618 Congenital lactic acidosis Nutritional and Metabolic Diseases
C4237343 BXGD022309 Overweight or obesity
C4282128 BXGD022420 PATENT DUCTUS ARTERIOSUS 1 Congenital, Hereditary, and Neonatal Diseases and Abnormalities; Cardiovascular Diseases
C4721610 BXGD023760 Carcinoma, Ovarian Epithelial Neoplasms; Female Urogenital Diseases and Pregnancy Complications; Endocrine System Diseases
C4722085 BXGD023788 Malignant neoplasm of colon and/or rectum
Target protein-related  compounds
Compounds
BXGC Id Compound Name Strcuture Molecular Weight
BXGC0000436 Glycerol 92.09
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