Showing entry for 2-oxoisovalerate dehydrogenase subunit beta, mitochondrial


　 　 　 　 　 　 　 　 　 　 　 　
General Target Information
BXGT IdBXGT008711
Protein Name2-oxoisovalerate dehydrogenase subunit beta, mitochondrial
Uniport IdP21953
GeneBCKDHB
Gene Id594
DomainTransketolase_C; Transket_pyr
Pfam PF02779   PF02780  
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 1.1 Carbohydrate metabolism hsa00640 Propanoate metabolism
1. Metabolism 1.0 Global and overview maps hsa01100 Metabolic pathways
Gene Ontology
GO Type GO Id Gene Ontology
Biological Process GO:0009083 branched-chain amino acid catabolic process
Biological Process GO:0007584 response to nutrient
molecular function GO:0003863 3-methyl-2-oxobutanoate dehydrogenase (2-methylpropanoyl-transferring) activity
molecular function GO:0003826 alpha-ketoacid dehydrogenase activity
cellular component GO:0005947 mitochondrial alpha-ketoglutarate dehydrogenase complex
cellular component GO:0005759 mitochondrial matrix
cellular component GO:0005739 mitochondrion
cellular component GO:0005730 nucleolus
Reactome
Pathway Id Pathway Name
R-HSA-1430728 Metabolism
R-HSA-389661 Glyoxylate metabolism and glycine degradation
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
C0001125 BXGD000024 Acidosis, Lactic Nutritional and Metabolic Diseases
C0001486 BXGD000056 Adenovirus Infections Infections
C0004134 BXGD000256 Ataxia Pathological Conditions, Signs and Symptoms; Nervous System Diseases
C0005890 BXGD000345 Body Height
C0006114 BXGD000371 Cerebral Edema Nervous System Diseases
C0006826 BXGD000408 Malignant Neoplasms Neoplasms
C0007570 BXGD000467 Celiac Disease Digestive System Diseases; Nutritional and Metabolic Diseases
C0009421 BXGD000608 Comatose Pathological Conditions, Signs and Symptoms; Nervous System Diseases
C0011853 BXGD000752 Diabetes Mellitus, Experimental Nutritional and Metabolic Diseases; Endocrine System Diseases
C0018524 BXGD001200 Hallucinations Pathological Conditions, Signs and Symptoms; Nervous System Diseases; Behavior and Behavior Mechanisms
C0019163 BXGD001295 Hepatitis B Digestive System Diseases; Infections
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
C0024141 BXGD001740 Lupus Erythematosus, Systemic Skin and Connective Tissue Diseases; Immune System Diseases
C0024776 BXGD001802 Maple Syrup Urine Disease Congenital, Hereditary, and Neonatal Diseases and Abnormalities; Nutritional and Metabolic Diseases; Nervous System Diseases
C0025322 BXGD001863 Premature Menopause Female Urogenital Diseases and Pregnancy Complications
C0026826 BXGD001935 Muscle Hypertonia Pathological Conditions, Signs and Symptoms; Nervous System Diseases
C0026827 BXGD001936 Muscle hypotonia Pathological Conditions, Signs and Symptoms; Nervous System Diseases
C0027651 BXGD002009 Neoplasms Neoplasms
C0030305 BXGD002206 Pancreatitis Digestive System Diseases
C0036572 BXGD002625 Seizures Pathological Conditions, Signs and Symptoms; Nervous System Diseases
C0042963 BXGD003018 Vomiting Pathological Conditions, Signs and Symptoms
C0085215 BXGD003141 Ovarian Failure, Premature Female Urogenital Diseases and Pregnancy Complications; Endocrine System Diseases
C0205682 BXGD004105 Waist-Hip Ratio
C0235974 BXGD004796 Pancreatic carcinoma Digestive System Diseases; Neoplasms; Endocrine System Diseases
C0238461 BXGD004936 Anaplastic thyroid carcinoma Neoplasms
C0262361 BXGD005229 Growth abnormality
C0268568 BXGD005989 Classic Maple Syrup Urine Disease Congenital, Hereditary, and Neonatal Diseases and Abnormalities; Nutritional and Metabolic Diseases; Nervous System Diseases
C0268569 BXGD005990 Intermittent Maple Syrup Urine Disease Congenital, Hereditary, and Neonatal Diseases and Abnormalities; Nutritional and Metabolic Diseases; Nervous System Diseases
C0276535 BXGD006429 AIDS with Kaposi's sarcoma Neoplasms; Infections; Immune System Diseases
C0346647 BXGD007832 Malignant neoplasm of pancreas Digestive System Diseases; Neoplasms; Endocrine System Diseases
C0424678 BXGD008537 Lean body mass
C0428883 BXGD008639 Diastolic blood pressure
C0489786 BXGD009018 Height
C0751285 BXGD010378 Maple Syrup Urine Disease, Thiamine Responsive Congenital, Hereditary, and Neonatal Diseases and Abnormalities; Nutritional and Metabolic Diseases; Nervous System Diseases
C0851887 BXGD010930 Adenoviral infections
C1306459 BXGD012361 Primary malignant neoplasm Neoplasms
C1621920 BXGD013467 Intermediate Maple Syrup Urine Disease Congenital, Hereditary, and Neonatal Diseases and Abnormalities; Nutritional and Metabolic Diseases; Nervous System Diseases
C1858120 BXGD015774 Generalized hypotonia Pathological Conditions, Signs and Symptoms; Nervous System Diseases
C1970209 BXGD016823 Alzheimer Disease 12 Nervous System Diseases; Mental Disorders
C2239176 BXGD016965 Liver carcinoma Digestive System Diseases; Neoplasms
C2362324 BXGD017091 Pediatric Obesity Pathological Conditions, Signs and Symptoms; Nutritional and Metabolic Diseases
C2674608 BXGD017250 Feeding difficulties in infancy
C2930990 BXGD017930 Maple syrup urine disease, type 1B Congenital, Hereditary, and Neonatal Diseases and Abnormalities; Nutritional and Metabolic Diseases; Nervous System Diseases
C3714756 BXGD019429 Intellectual Disability Pathological Conditions, Signs and Symptoms; Nervous System Diseases; Mental Disorders; Behavior and Behavior Mechanisms
C4016442 BXGD020335 MAPLE SYRUP URINE DISEASE, CLASSIC, TYPE IB
C4024697 BXGD021373 Elevated plasma branched chain amino acids
C4049938 BXGD021956 Physical Activity Measurement
C4703632 BXGD023670 Increased level of hippuric acid in urine
Target protein-related  compounds
Compounds
BXGC Id Compound Name Strcuture Molecular Weight
BXGC0000436 Glycerol 92.09
BXGC0002591 Potassium 39.1
BXGC0003705 Chloride 35.45
BXGC0037687 (4S)-2-methylpentane-2,4-diol 118.1
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