Showing entry for Methylcrotonoyl-CoA carboxylase beta chain, mitochondrial


　 　 　 　 　 　 　 　 　 　 　 　
General Target Information
BXGT IdBXGT020530
Protein NameMethylcrotonoyl-CoA carboxylase beta chain, mitochondrial
Uniport IdQ9HCC0
GeneMCCC2
Gene Id64087
DomainCarboxyl_trans
Pfam PF01039  
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 1.0 Global and overview maps hsa01100 Metabolic pathways
Gene Ontology
GO Type GO Id Gene Ontology
Biological Process GO:0006768 biotin metabolic process
Biological Process GO:0009083 branched-chain amino acid catabolic process
Biological Process GO:0015936 coenzyme A metabolic process
Biological Process GO:0006552 leucine catabolic process
molecular function GO:0005524 ATP binding
molecular function GO:0004485 methylcrotonoyl-CoA carboxylase activity
cellular component GO:0002169 3-methylcrotonyl-CoA carboxylase complex, mitochondrial
cellular component GO:0005829 cytosol
cellular component GO:1905202 methylcrotonoyl-CoA carboxylase complex
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-5668914 Diseases of 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
C0002170 BXGD000105 Alopecia Pathological Conditions, Signs and Symptoms; Skin and Connective Tissue Diseases
C0006142 BXGD000374 Malignant neoplasm of breast Neoplasms; Skin and Connective Tissue Diseases
C0009421 BXGD000608 Comatose Pathological Conditions, Signs and Symptoms; Nervous System Diseases
C0013146 BXGD000801 Drug abuse Chemically-Induced Disorders; Mental Disorders
C0015544 BXGD001003 Failure to Thrive Pathological Conditions, Signs and Symptoms
C0020615 BXGD001445 Hypoglycemia Nutritional and Metabolic Diseases
C0022638 BXGD001567 Ketosis Nutritional and Metabolic Diseases
C0023380 BXGD001640 Lethargy Pathological Conditions, Signs and Symptoms; Nervous System Diseases; Behavior and Behavior Mechanisms
C0026650 BXGD001913 Movement Disorders Nervous System Diseases
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
C0027051 BXGD001963 Myocardial Infarction Pathological Conditions, Signs and Symptoms; Cardiovascular Diseases
C0027651 BXGD002009 Neoplasms Neoplasms
C0035229 BXGD002516 Respiratory Insufficiency Respiratory Tract Diseases
C0036337 BXGD002598 Schizoaffective Disorder Mental Disorders
C0036341 BXGD002600 Schizophrenia Mental Disorders
C0036508 BXGD002623 Seborrheic dermatitis Skin and Connective Tissue Diseases
C0036572 BXGD002625 Seizures Pathological Conditions, Signs and Symptoms; Nervous System Diseases
C0042963 BXGD003018 Vomiting Pathological Conditions, Signs and Symptoms
C0151818 BXGD003480 Opisthotonus Pathological Conditions, Signs and Symptoms; Musculoskeletal Diseases; Nervous System Diseases
C0151889 BXGD003498 Hyperreflexia Pathological Conditions, Signs and Symptoms; Nervous System Diseases
C0162275 BXGD003924 Ketonuria Nutritional and Metabolic Diseases
C0220981 BXGD004348 Metabolic acidosis Nutritional and Metabolic Diseases
C0220994 BXGD004357 Hyperammonemia Pathological Conditions, Signs and Symptoms
C0221765 BXGD004477 Chronic schizophrenia Mental Disorders
C0232466 BXGD004543 Feeding difficulties
C0241775 BXGD005113 Organic aciduria
C0242379 BXGD005157 Malignant neoplasm of lung Neoplasms; Respiratory Tract Diseases
C0268579 BXGD005995 Propionic acidemia Congenital, Hereditary, and Neonatal Diseases and Abnormalities; Nutritional and Metabolic Diseases
C0268600 BXGD006001 3-methylcrotonyl CoA carboxylase 1 deficiency Congenital, Hereditary, and Neonatal Diseases and Abnormalities; Nutritional and Metabolic Diseases; Nervous System Diseases
C0376358 BXGD007992 Malignant neoplasm of prostate Neoplasms; Male Urogenital Diseases
C0424230 BXGD008522 Motor retardation Pathological Conditions, Signs and Symptoms; Nervous System Diseases; Behavior and Behavior Mechanisms
C0541794 BXGD009262 Skeletal muscle atrophy
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
C0600139 BXGD009695 Prostate carcinoma Neoplasms; Male Urogenital Diseases
C0678222 BXGD009749 Breast Carcinoma Neoplasms; Skin and Connective Tissue Diseases
C0684249 BXGD009790 Carcinoma of lung Neoplasms; Respiratory Tract Diseases
C0686619 BXGD009835 Secondary malignant neoplasm of lymph node Pathological Conditions, Signs and Symptoms; Neoplasms
C1306460 BXGD012362 Primary malignant neoplasm of lung Neoplasms; Respiratory Tract Diseases
C1858120 BXGD015774 Generalized hypotonia Pathological Conditions, Signs and Symptoms; Nervous System Diseases
C1859499 BXGD015903 3-methylcrotonyl CoA carboxylase 2 deficiency Congenital, Hereditary, and Neonatal Diseases and Abnormalities; Nutritional and Metabolic Diseases; Nervous System Diseases
C1859506 BXGD015904 Acute hyperammonemia Pathological Conditions, Signs and Symptoms
C1864399 BXGD016227 Peroxisome Biogenesis Disorder, Complementation Group 7 Congenital, Hereditary, and Neonatal Diseases and Abnormalities; Nutritional and Metabolic Diseases
C1867873 BXGD016473 Failure to thrive in infancy
C2364135 BXGD017125 Discomfort
C3714756 BXGD019429 Intellectual Disability Pathological Conditions, Signs and Symptoms; Nervous System Diseases; Mental Disorders; Behavior and Behavior Mechanisms
C4022001 BXGD020863 Abnormality of the cerebral vasculature
C4551505 BXGD023325 Methylcrotonyl-CoA carboxylase deficiency Congenital, Hereditary, and Neonatal Diseases and Abnormalities; Nutritional and Metabolic Diseases
C4553743 BXGD023548 Spasticity, CTCAE
Target protein-related  compounds
Compounds
BXGC Id Compound Name Strcuture Molecular Weight
BXGC0026123 Biotin 244.09
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