Showing entry for Very long-chain specific acyl-CoA dehydrogenase, mitochondrial


　 　 　 　 　 　 　 　 　 　 　 　
General Target Information
BXGT IdBXGT010733
Protein NameVery long-chain specific acyl-CoA dehydrogenase, mitochondrial
Uniport IdP49748
GeneACADVL
Gene Id37
DomainAcyl-CoA_dh_1; Acyl-CoA_dh_M; Acyl-CoA_dh_N
Pfam PF00441   PF02770   PF02771  
OrganismHomo sapiens (Human)
Target protein  affiliated  biological  pathways
KEGG
Class Subclass KEGG Id Pathway Name
1. Metabolism 1.3 Lipid metabolism hsa00071 Fatty acid degradation
1. Metabolism 1.0 Global and overview maps hsa01100 Metabolic pathways
1. Metabolism 1.0 Global and overview maps hsa01212 Fatty acid metabolism
Gene Ontology
GO Type GO Id Gene Ontology
Biological Process GO:0015980 energy derivation by oxidation of organic compounds
Biological Process GO:0030855 epithelial cell differentiation
Biological Process GO:0006635 fatty acid beta-oxidation
Biological Process GO:0033539 fatty acid beta-oxidation using acyl-CoA dehydrogenase
Biological Process GO:0036498 IRE1-mediated unfolded protein response
Biological Process GO:0045717 negative regulation of fatty acid biosynthetic process
Biological Process GO:0046322 negative regulation of fatty acid oxidation
Biological Process GO:0090181 regulation of cholesterol metabolic process
Biological Process GO:0009409 response to cold
Biological Process GO:0001659 temperature homeostasis
molecular function GO:0000062 fatty-acyl-CoA binding
molecular function GO:0050660 flavin adenine dinucleotide binding
molecular function GO:0004466 long-chain-acyl-CoA dehydrogenase activity
molecular function GO:0017099 very-long-chain-acyl-CoA dehydrogenase activity
cellular component GO:0005829 cytosol
cellular component GO:0005743 mitochondrial inner membrane
cellular component GO:0005759 mitochondrial matrix
cellular component GO:0031966 mitochondrial membrane
cellular component GO:0042645 mitochondrial nucleoid
cellular component GO:0005739 mitochondrion
cellular component GO:0005730 nucleolus
cellular component GO:0005654 nucleoplasm
Reactome
Pathway Id Pathway Name
R-HSA-1430728 Metabolism
R-HSA-381038 XBP1(S) activates chaperone genes
R-HSA-381070 IRE1alpha activates chaperones
R-HSA-381119 Unfolded Protein Response (UPR)
R-HSA-392499 Metabolism of proteins
R-HSA-556833 Metabolism of lipids
R-HSA-77286 mitochondrial fatty acid beta-oxidation of saturated fatty acids
R-HSA-77289 Mitochondrial Fatty Acid Beta-Oxidation
R-HSA-77305 Beta oxidation of palmitoyl-CoA to myristoyl-CoA
R-HSA-8978868 Fatty acid metabolism
Target protein-related  diseases
Disease
CUI Id BXGD Id Disease Name Disease Class
C0001627 BXGD000068 Congenital adrenal hyperplasia Congenital, Hereditary, and Neonatal Diseases and Abnormalities; Nutritional and Metabolic Diseases; Female Urogenital Diseases and Pregnancy Complications; Male Urogenital Diseases; Endocrine System Diseases
C0002395 BXGD000111 Alzheimer's Disease Nervous System Diseases; Mental Disorders
C0003129 BXGD000182 Anoxemia Pathological Conditions, Signs and Symptoms
C0003130 BXGD000183 Anoxia Pathological Conditions, Signs and Symptoms
C0004245 BXGD000264 Atrioventricular Block Pathological Conditions, Signs and Symptoms; Cardiovascular Diseases
C0007193 BXGD000451 Cardiomyopathy, Dilated Cardiovascular Diseases
C0007194 BXGD000452 Hypertrophic Cardiomyopathy Cardiovascular Diseases
C0015695 BXGD001013 Fatty Liver Digestive System Diseases
C0016522 BXGD001067 Foramen Ovale, Patent Congenital, Hereditary, and Neonatal Diseases and Abnormalities; Cardiovascular Diseases
C0018784 BXGD001220 Sensorineural Hearing Loss (disorder) Pathological Conditions, Signs and Symptoms; Nervous System Diseases; Otorhinolaryngologic Diseases
C0018818 BXGD001236 Ventricular Septal Defects Congenital, Hereditary, and Neonatal Diseases and Abnormalities; Cardiovascular Diseases
C0019209 BXGD001305 Hepatomegaly Pathological Conditions, Signs and Symptoms; Digestive System Diseases
C0020598 BXGD001440 Hypocalcemia Nutritional and Metabolic Diseases
C0020615 BXGD001445 Hypoglycemia Nutritional and Metabolic Diseases
C0020639 BXGD001457 Hypoproteinemia Hemic and Lymphatic Diseases
C0020672 BXGD001461 Hypothermia, natural Pathological Conditions, Signs and Symptoms
C0022346 BXGD001537 Icterus Pathological Conditions, Signs and Symptoms
C0023380 BXGD001640 Lethargy Pathological Conditions, Signs and Symptoms; Nervous System Diseases; Behavior and Behavior Mechanisms
C0023895 BXGD001717 Liver diseases Digestive System Diseases
C0026827 BXGD001936 Muscle hypotonia Pathological Conditions, Signs and Symptoms; Nervous System Diseases
C0026848 BXGD001941 Myopathy Musculoskeletal Diseases; Nervous System Diseases
C0028754 BXGD002082 Obesity Pathological Conditions, Signs and Symptoms; Nutritional and Metabolic Diseases
C0030193 BXGD002191 Pain Pathological Conditions, Signs and Symptoms
C0031039 BXGD002274 Pericardial effusion Cardiovascular Diseases
C0032285 BXGD002344 Pneumonia Infections; Respiratory Tract Diseases
C0033626 BXGD002412 Protein Deficiency Nutritional and Metabolic Diseases
C0035410 BXGD002546 Rhabdomyolysis Musculoskeletal Diseases
C0037763 BXGD002699 Spasm Pathological Conditions, Signs and Symptoms; Nervous System Diseases
C0042510 BXGD002987 Ventricular Fibrillation Pathological Conditions, Signs and Symptoms; Cardiovascular Diseases
C0042514 BXGD002989 Tachycardia, Ventricular Pathological Conditions, Signs and Symptoms; Cardiovascular Diseases
C0042963 BXGD003018 Vomiting Pathological Conditions, Signs and Symptoms
C0085298 BXGD003155 Sudden Cardiac Death Pathological Conditions, Signs and Symptoms; Cardiovascular Diseases
C0151786 BXGD003475 Muscle Weakness Pathological Conditions, Signs and Symptoms; Musculoskeletal Diseases; Nervous System Diseases
C0151878 BXGD003495 Prolonged QT interval Pathological Conditions, Signs and Symptoms; Cardiovascular Diseases
C0175754 BXGD004015 Agenesis of corpus callosum Pathological Conditions, Signs and Symptoms; Congenital, Hereditary, and Neonatal Diseases and Abnormalities; Nervous System Diseases
C0206667 BXGD004228 Adrenal Cortical Adenoma Neoplasms; Endocrine System Diseases
C0220981 BXGD004348 Metabolic acidosis Nutritional and Metabolic Diseases
C0220994 BXGD004357 Hyperammonemia Pathological Conditions, Signs and Symptoms
C0221170 BXGD004400 Muscular stiffness Nervous System Diseases
C0221355 BXGD004447 Macrocephaly Congenital, Hereditary, and Neonatal Diseases and Abnormalities; Musculoskeletal Diseases; Nervous System Diseases
C0221757 BXGD004472 alpha 1-Antitrypsin Deficiency Pathological Conditions, Signs and Symptoms; Congenital, Hereditary, and Neonatal Diseases and Abnormalities; Digestive System Diseases; Respiratory Tract Diseases
C0231835 BXGD004527 Tachypnea Pathological Conditions, Signs and Symptoms; Respiratory Tract Diseases
C0232466 BXGD004543 Feeding difficulties
C0235991 BXGD004802 Small for gestational age (disorder) Pathological Conditions, Signs and Symptoms; Female Urogenital Diseases and Pregnancy Complications
C0241005 BXGD005072 Creatine phosphokinase serum increased
C0242184 BXGD005140 Hypoxia Pathological Conditions, Signs and Symptoms
C0270984 BXGD006149 Metabolic myopathy Musculoskeletal Diseases; Nervous System Diseases
C0282160 BXGD006797 Aplasia Cutis Congenita Congenital, Hereditary, and Neonatal Diseases and Abnormalities; Skin and Connective Tissue Diseases
C0282507 BXGD006806 Heat Stress Disorders Wounds and Injuries
C0302280 BXGD006836 Adrenogenital Syndrome Congenital, Hereditary, and Neonatal Diseases and Abnormalities; Female Urogenital Diseases and Pregnancy Complications; Male Urogenital Diseases; Endocrine System Diseases
C0302511 BXGD006849 Small for gestational age fetus Pathological Conditions, Signs and Symptoms
C0342784 BXGD007551 Pearson's marrow-pancreas syndrome Pathological Conditions, Signs and Symptoms; Congenital, Hereditary, and Neonatal Diseases and Abnormalities; Hemic and Lymphatic Diseases
C0369183 BXGD007960 Erythrocyte Mean Corpuscular Hemoglobin Test
C0476273 BXGD008986 Respiratory distress Pathological Conditions, Signs and Symptoms; Respiratory Tract Diseases
C0497406 BXGD009064 Overweight Pathological Conditions, Signs and Symptoms
C0523465 BXGD009209 Serum albumin measurement
C0524620 BXGD009236 Metabolic Syndrome X Nutritional and Metabolic Diseases
C0700292 BXGD009884 Hypoxemia Pathological Conditions, Signs and Symptoms
C0878544 BXGD011368 Cardiomyopathies Cardiovascular Diseases
C1142132 BXGD011733 Carnitine deficiency
C1145670 BXGD011764 Respiratory Failure Respiratory Tract Diseases
C1261502 BXGD011856 Finding of Mean Corpuscular Hemoglobin
C1282975 BXGD012157 von Willebrand Disease, Type 2N Congenital, Hereditary, and Neonatal Diseases and Abnormalities; Hemic and Lymphatic Diseases
C1838705 BXGD014341 Anteriorly placed anus
C1845155 BXGD014720 Exercise-induced myoglobinuria Musculoskeletal Diseases
C1848701 BXGD014967 Elevated hepatic transaminase
C1850830 BXGD015181 Exercise-induced myalgia Pathological Conditions, Signs and Symptoms; Musculoskeletal Diseases; Nervous System Diseases
C1853377 BXGD015331 Enlarged cisterna magna
C1855038 BXGD015452 Hepatocellular necrosis
C1856432 BXGD015605 Dicarboxylic aciduria
C1856438 BXGD015606 Hypoketotic hypoglycemia Nutritional and Metabolic Diseases
C1858120 BXGD015774 Generalized hypotonia Pathological Conditions, Signs and Symptoms; Nervous System Diseases
C1860834 BXGD016006 Infantile muscular hypotonia Pathological Conditions, Signs and Symptoms; Nervous System Diseases
C1865292 BXGD016306 Nonketotic hypoglycemia Nutritional and Metabolic Diseases
C1969443 BXGD016778 Trifunctional Protein Deficiency With Myopathy And Neuropathy Congenital, Hereditary, and Neonatal Diseases and Abnormalities; Nutritional and Metabolic Diseases; Musculoskeletal Diseases; Nervous System Diseases; Cardiovascular Diseases
C2711227 BXGD017478 Steatohepatitis Digestive System Diseases
C2750440 BXGD017660 FATTY LIVER DISEASE, NONALCOHOLIC, SUSCEPTIBILITY TO, 1
C2750441 BXGD017661 LIVER DISEASE, ALCOHOLIC, SUSCEPTIBILITY TO, 1
C3150275 BXGD018300 COMPLEMENT COMPONENT 2 DEFICIENCY
C3150651 BXGD018316 FATTY LIVER DISEASE, NONALCOHOLIC, SUSCEPTIBILITY TO, 2
C3806218 BXGD019500 Episodic tachypnea Pathological Conditions, Signs and Symptoms; Respiratory Tract Diseases
C3875321 BXGD019874 Inflammatory dermatosis Skin and Connective Tissue Diseases
C3887523 BXGD019891 Very long chain acyl-CoA dehydrogenase deficiency Congenital, Hereditary, and Neonatal Diseases and Abnormalities; Nutritional and Metabolic Diseases; Musculoskeletal Diseases; Nervous System Diseases; Hemic and Lymphatic Diseases
C4021526 BXGD020653 Exercise-induced rhabdomyolysis Musculoskeletal Diseases
C4023591 BXGD021224 Abnormality of circulating enzyme level
C4280773 BXGD022404 Increased circulating free fatty acid level
C4551472 BXGD023303 Hypertrophic obstructive cardiomyopathy Cardiovascular Diseases
Target protein-related  compounds
Compounds
BXGC Id Compound Name Strcuture Molecular Weight
BXGC0042376 Picropodophyllotoxin 414.13
BXGC0047692 Gefitinib 446.15
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