Showing entry for 3-hydroxyisobutyryl-CoA hydrolase, mitochondrial


　 　 　 　 　 　 　 　 　 　 　 　
General Target Information
BXGT IdBXGT016432
Protein Name3-hydroxyisobutyryl-CoA hydrolase, mitochondrial
Uniport IdQ6NVY1
GeneHIBCH
Gene Id26275
DomainECH_2
Pfam PF16113  
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 1.6 Metabolism of other amino acids hsa00410 beta-Alanine 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:0009083 branched-chain amino acid catabolic process
Biological Process GO:0006574 valine catabolic process
molecular function GO:0003860 3-hydroxyisobutyryl-CoA hydrolase activity
cellular component GO:0005759 mitochondrial matrix
cellular component GO:0005739 mitochondrion
Reactome
Pathway Id Pathway Name
R-HSA-1430728 Metabolism
R-HSA-70895 Branched-chain amino acid catabolism
R-HSA-71291 Metabolism of amino acids and derivatives
Target protein-related  diseases
Disease
CUI Id BXGD Id Disease Name Disease Class
C0009402 BXGD000605 Colorectal Carcinoma Digestive System Diseases; Neoplasms
C0010417 BXGD000670 Cryptorchidism Congenital, Hereditary, and Neonatal Diseases and Abnormalities; Male Urogenital Diseases; Endocrine System Diseases
C0013421 BXGD000837 Dystonia Pathological Conditions, Signs and Symptoms; Nervous System Diseases
C0015544 BXGD001003 Failure to Thrive Pathological Conditions, Signs and Symptoms
C0022107 BXGD001530 Irritable Mood Behavior and Behavior Mechanisms
C0023264 BXGD001625 Leigh Disease Congenital, Hereditary, and Neonatal Diseases and Abnormalities; Nutritional and Metabolic Diseases; Nervous System Diseases
C0025521 BXGD001876 Inborn Errors of Metabolism Congenital, Hereditary, and Neonatal Diseases and Abnormalities; Nutritional and Metabolic Diseases
C0026827 BXGD001936 Muscle hypotonia Pathological Conditions, Signs and Symptoms; Nervous System Diseases
C0027066 BXGD001966 Myoclonus Pathological Conditions, Signs and Symptoms; Nervous System Diseases
C0027651 BXGD002009 Neoplasms Neoplasms
C0028738 BXGD002081 Nystagmus Eye Diseases; Nervous System Diseases
C0029408 BXGD002137 Degenerative polyarthritis Musculoskeletal Diseases
C0035229 BXGD002516 Respiratory Insufficiency Respiratory Tract Diseases
C0036572 BXGD002625 Seizures Pathological Conditions, Signs and Symptoms; Nervous System Diseases
C0037317 BXGD002689 Sleep disturbances Nervous System Diseases; Mental Disorders; Behavior and Behavior Mechanisms
C0037769 BXGD002701 West Syndrome Nervous System Diseases
C0038379 BXGD002752 Strabismus Eye Diseases; Nervous System Diseases
C0039685 BXGD002825 Tetralogy of Fallot Congenital, Hereditary, and Neonatal Diseases and Abnormalities; Cardiovascular Diseases
C0042963 BXGD003018 Vomiting Pathological Conditions, Signs and Symptoms
C0085584 BXGD003195 Encephalopathies Nervous System Diseases
C0086743 BXGD003308 Osteoarthrosis Deformans Musculoskeletal Diseases
C0151889 BXGD003498 Hyperreflexia Pathological Conditions, Signs and Symptoms; Nervous System Diseases
C0175754 BXGD004015 Agenesis of corpus callosum Pathological Conditions, Signs and Symptoms; Congenital, Hereditary, and Neonatal Diseases and Abnormalities; Nervous System Diseases
C0220981 BXGD004348 Metabolic acidosis Nutritional and Metabolic Diseases
C0232466 BXGD004543 Feeding difficulties
C0234162 BXGD004627 Cerebellar Dysmetria Pathological Conditions, Signs and Symptoms; Nervous System Diseases
C0238621 BXGD004947 Aminoaciduria Congenital, Hereditary, and Neonatal Diseases and Abnormalities; Nutritional and Metabolic Diseases
C0270612 BXGD006081 Leukoencephalopathy Nervous System Diseases
C0342738 BXGD007537 Beta-Hydroxyisobutyryl CoA Deacylase Deficiency Congenital, Hereditary, and Neonatal Diseases and Abnormalities; Nutritional and Metabolic Diseases
C0393593 BXGD008103 Dystonia Disorders Nervous System Diseases
C0424503 BXGD008532 Dysmorphic facies
C0427190 BXGD008611 Ataxia, Truncal Pathological Conditions, Signs and Symptoms; Nervous System Diseases
C0520947 BXGD009126 Clumsiness - motor delay Pathological Conditions, Signs and Symptoms; Nervous System Diseases; Mental Disorders; Behavior and Behavior Mechanisms
C0524851 BXGD009246 Neurodegenerative Disorders Nervous System Diseases
C0557874 BXGD009444 Global developmental delay
C0678230 BXGD009750 Congenital Epicanthus
C0684276 BXGD009793 Hypsarrhythmia Nervous System Diseases
C0700201 BXGD009880 Dyssomnias Nervous System Diseases; Mental Disorders
C0851578 BXGD010925 Sleep Disorders Pathological Conditions, Signs and Symptoms; Nervous System Diseases; Mental Disorders
C1531647 BXGD013301 Cerebral ventriculomegaly Nervous System Diseases
C1608410 BXGD013435 Head titubation
C1834129 BXGD013957 Abnormal vertebral morphology
C1836440 BXGD014115 Increased serum lactate Nutritional and Metabolic Diseases
C1836830 BXGD014165 Developmental regression Mental Disorders
C1854301 BXGD015391 Motor delay Mental Disorders
C1854838 BXGD015438 Progressive neurologic deterioration Mental Disorders
C1858120 BXGD015774 Generalized hypotonia Pathological Conditions, Signs and Symptoms; Nervous System Diseases
C1861866 BXGD016087 Aplasia/Hypoplasia of the corpus callosum
C1963184 BXGD016688 Nystagmus, CTCAE 3.0
C2700617 BXGD017474 Irritation - emotion Behavior and Behavior Mechanisms
C2985280 BXGD018223 Blood Protein Measurement
C3278923 BXGD018748 Dilated ventricles (finding)
C4021159 BXGD020567 Facial shape deformation
C4021734 BXGD020742 Abnormality of mitochondrial metabolism
C4021789 BXGD020781 Abnormality of the vertebral column
C4022769 BXGD020994 Small basal ganglia
C4283745 BXGD022424 Neurodegeneration due to 3-hydroxyisobutyryl coenzyme A hydrolase deficiency
C4552810 BXGD023525 Irritability, CTCAE
C4554036 BXGD023557 Nystagmus, CTCAE 5.0
C4722085 BXGD023788 Malignant neoplasm of colon and/or rectum
Target protein-related  compounds
Compounds
BXGC Id Compound Name Strcuture Molecular Weight
BXGC0005683 Quercetin 302.24
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