Showing entry for Hydroxymethylglutaryl-CoA lyase, mitochondrial


　 　 　 　 　 　 　 　 　 　 　 　
General Target Information
BXGT IdBXGT009880
Protein NameHydroxymethylglutaryl-CoA lyase, mitochondrial
Uniport IdP35914
GeneHMGCL
Gene Id3155
DomainHMGL-like
Pfam PF00682  
OrganismHomo sapiens (Human)
Target protein  affiliated  biological  pathways
KEGG
Class Subclass KEGG Id Pathway Name
1. Metabolism 1.3 Lipid metabolism hsa00072 Synthesis and degradation of ketone bodies
1. Metabolism 1.5 Amino acid metabolism hsa00280 Valine, leucine and isoleucine degradation
1. Metabolism 1.1 Carbohydrate metabolism hsa00650 Butanoate metabolism
1. Metabolism 1.0 Global and overview maps hsa01100 Metabolic pathways
4. Cellular Processes 4.1 Transport and catabolism hsa04146 Peroxisome
Gene Ontology
GO Type GO Id Gene Ontology
Biological Process GO:0006637 acyl-CoA metabolic process
Biological Process GO:0046951 ketone body biosynthetic process
Biological Process GO:0006552 leucine catabolic process
Biological Process GO:0006629 lipid metabolic process
Biological Process GO:0001889 liver development
Biological Process GO:0007005 mitochondrion organization
Biological Process GO:0006625 protein targeting to peroxisome
Biological Process GO:0070542 response to fatty acid
Biological Process GO:0007584 response to nutrient
Biological Process GO:0042594 response to starvation
molecular function GO:0031406 carboxylic acid binding
molecular function GO:0000062 fatty-acyl-CoA binding
molecular function GO:0004419 hydroxymethylglutaryl-CoA lyase activity
molecular function GO:0000287 magnesium ion binding
molecular function GO:0030145 manganese ion binding
molecular function GO:0046872 metal ion binding
molecular function GO:0005198 structural molecule activity
cellular component GO:0005829 cytosol
cellular component GO:0005759 mitochondrial matrix
cellular component GO:0005739 mitochondrion
cellular component GO:0005782 peroxisomal matrix
cellular component GO:0005777 peroxisome
cellular component GO:0032991 protein-containing complex
Reactome
Pathway Id Pathway Name
R-HSA-1430728 Metabolism
R-HSA-556833 Metabolism of lipids
R-HSA-74182 Ketone body metabolism
R-HSA-77111 Synthesis of Ketone Bodies
R-HSA-9033241 Peroxisomal protein import
R-HSA-9033241 Peroxisomal protein import
R-HSA-9609507 Protein localization
R-HSA-9609507 Protein localization
Target protein-related  diseases
Disease
CUI Id BXGD Id Disease Name Disease Class
C0001339 BXGD000041 Acute pancreatitis Digestive System Diseases
C0002871 BXGD000132 Anemia Hemic and Lymphatic Diseases
C0003123 BXGD000178 Anorexia Pathological Conditions, Signs and Symptoms
C0003578 BXGD000211 Apnea Pathological Conditions, Signs and Symptoms; Respiratory Tract Diseases
C0004134 BXGD000256 Ataxia Pathological Conditions, Signs and Symptoms; Nervous System Diseases
C0007193 BXGD000451 Cardiomyopathy, Dilated Cardiovascular Diseases
C0009421 BXGD000608 Comatose Pathological Conditions, Signs and Symptoms; Nervous System Diseases
C0011175 BXGD000701 Dehydration Pathological Conditions, Signs and Symptoms; Nutritional and Metabolic Diseases
C0011853 BXGD000752 Diabetes Mellitus, Experimental Nutritional and Metabolic Diseases; Endocrine System Diseases
C0011991 BXGD000766 Diarrhea Pathological Conditions, Signs and Symptoms
C0013362 BXGD000817 Dysarthria Pathological Conditions, Signs and Symptoms; Nervous System Diseases
C0013604 BXGD000859 Edema Pathological Conditions, Signs and Symptoms
C0015672 BXGD001011 Fatigue Pathological Conditions, Signs and Symptoms
C0015967 BXGD001030 Fever Pathological Conditions, Signs and Symptoms
C0018790 BXGD001221 Cardiac Arrest Cardiovascular Diseases
C0019209 BXGD001305 Hepatomegaly Pathological Conditions, Signs and Symptoms; Digestive System Diseases
C0020615 BXGD001445 Hypoglycemia Nutritional and Metabolic Diseases
C0020617 BXGD001446 Hypoglycemic coma Pathological Conditions, Signs and Symptoms; Nutritional and Metabolic Diseases; Nervous System Diseases; Endocrine System Diseases
C0020649 BXGD001459 Hypotension Cardiovascular 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
C0023518 BXGD001677 Leukocytosis Pathological Conditions, Signs and Symptoms; Hemic and Lymphatic Diseases
C0023530 BXGD001683 Leukopenia Hemic and Lymphatic Diseases
C0025202 BXGD001832 melanoma Neoplasms
C0025958 BXGD001882 Microcephaly Congenital, Hereditary, and Neonatal Diseases and Abnormalities; Musculoskeletal Diseases; Nervous System Diseases
C0026106 BXGD001889 Mild Mental Retardation Pathological Conditions, Signs and Symptoms; Nervous System Diseases; Mental Disorders; Behavior and Behavior Mechanisms
C0026351 BXGD001900 Moderate intellectual disability Pathological Conditions, Signs and Symptoms; Nervous System Diseases; Mental Disorders; Behavior and Behavior Mechanisms
C0026827 BXGD001936 Muscle hypotonia Pathological Conditions, Signs and Symptoms; Nervous System Diseases
C0026838 BXGD001938 Muscle Spasticity Pathological Conditions, Signs and Symptoms; Musculoskeletal Diseases; Nervous System Diseases
C0027066 BXGD001966 Myoclonus Pathological Conditions, Signs and Symptoms; Nervous System Diseases
C0027651 BXGD002009 Neoplasms Neoplasms
C0030232 BXGD002197 Pallor Pathological Conditions, Signs and Symptoms
C0036572 BXGD002625 Seizures Pathological Conditions, Signs and Symptoms; Nervous System Diseases
C0036857 BXGD002638 Severe intellectual disability Pathological Conditions, Signs and Symptoms; Nervous System Diseases; Mental Disorders; Behavior and Behavior Mechanisms
C0042963 BXGD003018 Vomiting Pathological Conditions, Signs and Symptoms
C0085584 BXGD003195 Encephalopathies Nervous System Diseases
C0085632 BXGD003215 Apathy Behavior and Behavior Mechanisms
C0151611 BXGD003447 Electroencephalogram abnormal Nervous System Diseases
C0151872 BXGD003493 Prothrombin time increased Hemic and Lymphatic Diseases
C0151888 BXGD003497 Hyporeflexia Pathological Conditions, Signs and Symptoms; Nervous System Diseases
C0154694 BXGD003733 Hemiplegia, Spastic Pathological Conditions, Signs and Symptoms; Nervous System Diseases
C0162275 BXGD003924 Ketonuria Nutritional and Metabolic Diseases
C0206161 BXGD004167 Reticulocyte count (procedure)
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
C0268594 BXGD005998 Glutaric aciduria
C0268601 BXGD006002 HMG CoA lyase deficiency Congenital, Hereditary, and Neonatal Diseases and Abnormalities; Nutritional and Metabolic Diseases
C0270612 BXGD006081 Leukoencephalopathy Nervous System Diseases
C0580413 BXGD009558 Prothrombin time low
C0684276 BXGD009793 Hypsarrhythmia Nervous System Diseases
C0694563 BXGD009854 Excessive daytime somnolence
C0740394 BXGD009989 Hyperuricemia Pathological Conditions, Signs and Symptoms
C0751651 BXGD010539 Mitochondrial Diseases Nutritional and Metabolic Diseases
C0836924 BXGD010864 Thrombocytosis Hemic and Lymphatic Diseases
C0948163 BXGD011511 Leukoaraiosis Pathological Conditions, Signs and Symptoms
C1142132 BXGD011733 Carnitine deficiency
C1262477 BXGD011882 Weight decreased Pathological Conditions, Signs and Symptoms
C1291557 BXGD012217 Deficiency of lyase
C1533587 BXGD013315 Hydroxymethylglutaric aciduria
C1836440 BXGD014115 Increased serum lactate Nutritional and Metabolic Diseases
C1837257 BXGD014213 Lipid accumulation in hepatocytes
C1838993 BXGD014351 Episodic vomiting Pathological Conditions, Signs and Symptoms
C1843392 BXGD014582 Death in childhood
C1846288 BXGD014796 Recurrent hypoglycemia Nutritional and Metabolic Diseases
C1848701 BXGD014967 Elevated hepatic transaminase
C1850413 BXGD015141 Reye syndrome-like episodes
C1865292 BXGD016306 Nonketotic hypoglycemia Nutritional and Metabolic Diseases
C3151466 BXGD018434 HEPATIC LIPASE DEFICIENCY (disorder) Nutritional and Metabolic Diseases
C3151952 BXGD018455 3-Methylglutaric aciduria Congenital, Hereditary, and Neonatal Diseases and Abnormalities; Nutritional and Metabolic Diseases
C3714756 BXGD019429 Intellectual Disability Pathological Conditions, Signs and Symptoms; Nervous System Diseases; Mental Disorders; Behavior and Behavior Mechanisms
C4553743 BXGD023548 Spasticity, CTCAE
C4703617 BXGD023664 Increased level of 3-hydroxy-3-methylglutaric acid in urine
C4703632 BXGD023670 Increased level of hippuric acid in urine
Target protein-related  compounds
Compounds
BXGC Id Compound Name Strcuture Molecular Weight
BXGC0002588 Magnesium 24.31
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