Details of Drug-Metabolizing Enzyme (DME)

General Information of Drug-Metabolizing Enzyme (DME) (ID: DESWQMH)

DME Name Propionyl-CoA carboxylase beta (PCCB)
Synonyms Propanoyl-CoA:carbon dioxide ligase beta; Mitochondrial propionyl-CoA carboxylase beta; PCCase subunit beta; PCCB
Gene Name PCCB
UniProt ID
PCCB_HUMAN
INTEDE ID
DME0178
3D Structure
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2D Sequence (FASTA)
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3D Structure (PDB)
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Gene ID
5096
EC Number EC: 6.4.1.3
Ligase
Carbon-carbon ligase
Carbon-carbon ligase
EC: 6.4.1.3
Lineage Species: Homo sapiens
Kingdom: Metazoa
Phylum: Chordata
Class: Mammalia
Order: Primates
Family: Hominidae
Genus: Homo
Species: Homo sapiens
Sequence
MAAALRVAAVGARLSVLASGLRAAVRSLCSQATSVNERIENKRRTALLGGGQRRIDAQHK
RGKLTARERISLLLDPGSFVESDMFVEHRCADFGMAADKNKFPGDSVVTGRGRINGRLVY
VFSQDFTVFGGSLSGAHAQKICKIMDQAITVGAPVIGLNDSGGARIQEGVESLAGYADIF
LRNVTASGVIPQISLIMGPCAGGAVYSPALTDFTFMVKDTSYLFITGPDVVKSVTNEDVT
QEELGGAKTHTTMSGVAHRAFENDVDALCNLRDFFNYLPLSSQDPAPVRECHDPSDRLVP
ELDTIVPLESTKAYNMVDIIHSVVDEREFFEIMPNYAKNIIVGFARMNGRTVGIVGNQPK
VASGCLDINSSVKGARFVRFCDAFNIPLITFVDVPGFLPGTAQEYGGIIRHGAKLLYAFA
EATVPKVTVITRKAYGGAYDVMSSKHLCGDTNYAWPTAEIAVMGAKGAVEIIFKGHENVE
AAQAEYIEKFANPFPAAVRGFVDDIIQPSSTRARICCDLDVLASKKVQRPWRKHANIPL
Function
This enzyme is one of the 2 subunits of the biotin-dependent propionyl-CoA carboxylase (PCC), a mitochondrial enzyme involved in the catabolism of odd chain fatty acids, branched-chain amino acids isoleucine, threonine, methionine, and valine and other metabolites. Propionyl-CoA carboxylase catalyzes the carboxylation of propionyl-CoA/propanoyl-CoA to D-methylmalonyl- CoA/(S)-methylmalonyl-CoA. Within the holoenzyme, the alpha subunit catalyzes the ATP-dependent carboxylation of the biotin carried by the biotin carboxyl carrier (BCC) domain, while the beta subunit then transfers the carboxyl group from carboxylated biotin to propionyl-CoA. Propionyl-CoA carboxylase also significantly acts on butyryl-CoA/butanoyl-CoA, which is converted to ethylmalonyl-CoA/(2S)-ethylmalonyl-CoA at a much lower rate. Other alternative minor substrates include (2E)- butenoyl-CoA/crotonoyl-CoA (By similarity).
KEGG Pathway
Carbon metabolism (hsa01200 )
Glyoxylate and dicarboxylate metabolism (hsa00630 )
Metabolic pathways (hsa01100 )
Propanoate metabolism (hsa00640 )
Valine, leucine and isoleucine degradation (hsa00280 )
Reactome Pathway
Defective HLCS causes multiple carboxylase deficiency (R-HSA-3371599 )
Propionyl-CoA catabolism (R-HSA-71032 )
Biotin transport and metabolism (R-HSA-196780 )

Molecular Interaction Atlas (MIA) of This DME

Molecular Interaction Atlas (MIA) Jump to Detail Molecular Interaction Atlas of This DME
1 Investigative Drug(s) Metabolized by This DME
Drug Name Drug ID Indication ICD 11 Highest Status REF
L-valine DM68RPD Discovery agent N.A. Investigative [1]

Molecular Expression Atlas (MEA) of This DME

Molecular Expression Atlas (MEA) Jump to Detail Molecular Expression Atlas of This DME
Disease Name ICD 11 Studied Tissue p-value Fold-Change Z-score
Acute myelocytic leukaemia 2B33.1 Bone marrow 2.83E-03 -4.27E-02 -1.32E-01
Alopecia ED70 Skin from scalp 8.21E-01 -3.59E-02 -1.27E-01
Alzheimer's disease 8A20 Entorhinal cortex 1.47E-02 -1.76E-02 -9.60E-02
Ankylosing spondylitis FA92.0 Pheripheral blood 7.54E-02 1.38E-01 5.95E-01
Aortic stenosis BB70 Calcified aortic valve 5.13E-01 2.05E-01 3.55E-01
Apnea 7A40 Hyperplastic tonsil 6.19E-01 -5.56E-02 -1.55E-01
Arthropathy FA00-FA5Z Peripheral blood 3.84E-01 -6.67E-02 -5.44E-01
Asthma CA23 Nasal and bronchial airway 3.58E-06 2.31E-01 3.58E-01
Atopic dermatitis EA80 Skin 7.70E-02 8.77E-02 7.23E-01
Autism 6A02 Whole blood 4.59E-02 -2.29E-02 -9.82E-02
Autoimmune uveitis 9A96 Peripheral monocyte 2.12E-01 -2.14E-01 -1.97E+00
Autosomal dominant monocytopenia 4B04 Whole blood 2.77E-01 -2.24E-02 -1.46E-01
Bacterial infection of gingival 1C1H Gingival tissue 2.90E-01 9.87E-03 5.39E-02
Batten disease 5C56.1 Whole blood 3.52E-01 -7.28E-02 -5.33E-01
Behcet's disease 4A62 Peripheral blood 8.82E-01 7.08E-02 3.55E-01
Bipolar disorder 6A60-6A6Z Prefrontal cortex 6.88E-01 -1.55E-02 -1.37E-01
Bladder cancer 2C94 Bladder tissue 2.19E-02 -2.65E-01 -1.72E+00
Breast cancer 2C60-2C6Z Breast tissue 6.15E-16 2.28E-01 5.98E-01
Cardioembolic stroke 8B11.20 Whole blood 9.32E-01 -8.11E-02 -5.01E-01
Cervical cancer 2C77 Cervical tissue 3.23E-01 -2.44E-03 -8.97E-03
Childhood onset rheumatic disease FA20.Z Peripheral blood 5.12E-01 1.98E-02 4.69E-02
Chronic hepatitis C 1E51.1 Whole blood 3.55E-01 -3.62E-02 -3.02E-01
Chronic obstructive pulmonary disease CA22 Lung tissue 8.36E-01 5.88E-02 2.99E-01
Chronic obstructive pulmonary disease CA22 Small airway epithelium 2.74E-02 -5.12E-02 -2.72E-01
Chronic rhinosinusitis CA0A Sinus mucosa tissue 8.09E-01 -4.16E-02 -3.40E-01
Colon cancer 2B90 Colon tissue 1.20E-14 -1.90E-01 -8.67E-01
Coronary artery disease BA80-BA8Z Peripheral blood 1.45E-01 -3.63E-01 -1.59E+00
Diffuse large B-cell lymphoma 2A81 Tonsil tissue 1.17E-01 4.92E-02 1.34E-01
Endometriosis GA10 Endometrium tissue 7.04E-02 -1.18E-01 -4.19E-01
Familial hypercholesterolemia 5C80.00 Peripheral blood 2.37E-01 -1.15E-01 -6.66E-01
Familial hypercholesterolemia 5C80.00 Whole blood 1.89E-01 9.19E-02 5.08E-01
Gastric cancer 2B72 Gastric tissue 3.28E-01 1.74E-01 4.22E-01
Glioblastopma 2A00.00 Nervous tissue 5.66E-32 1.98E-01 8.22E-01
Glioma 2A00.0Y-2A00.0Z Brain stem tissue 9.63E-02 3.88E-01 2.73E+00
Glioma 2A00.0Y-2A00.0Z White matter tissue 6.73E-02 2.79E-01 6.94E-01
Head and neck cancer 2D42 Head and neck tissue 4.68E-05 -1.41E-01 -8.47E-01
HIV-associated neurocognitive impairment 8A2Y-8A2Z White matter tissue 7.28E-01 2.24E-02 8.03E-02
Huntington's disease 8A01.10 Whole blood 7.47E-02 -2.21E-01 -9.73E-01
Idiopathic pulmonary fibrosis CB03.4 Lung tissue 1.07E-02 -1.68E-01 -1.50E+00
Immunodeficiency 4A00-4A20 Peripheral blood 8.17E-01 -5.56E-02 -3.91E-01
Influenza 1E30 Whole blood 3.90E-01 -2.34E-01 -1.25E+00
Interstitial cystitis GC00.3 Bladder tissue 6.64E-01 -3.64E-02 -3.20E-01
Intracranial aneurysm 8B01.0 Intracranial artery 1.47E-01 2.90E-01 9.83E-01
Irritable bowel syndrome DD91.0 Rectal colon tissue 1.37E-01 -2.77E-02 -9.62E-02
Ischemic stroke 8B11 Peripheral blood 8.99E-04 -1.69E-01 -1.69E+00
Juvenile idiopathic arthritis FA24 Peripheral blood 2.20E-04 -1.46E-01 -6.53E-01
Lateral sclerosis 8B60.4 Skin 1.62E-02 2.44E-01 2.37E+00
Lateral sclerosis 8B60.4 Cervical spinal cord 4.43E-01 -7.58E-02 -2.13E-01
Liver cancer 2C12.0 Liver tissue 6.99E-17 -4.85E-01 -1.87E+00
Liver failure DB99.7-DB99.8 Liver tissue 1.52E-03 -7.32E-01 -2.29E+00
Lung cancer 2C25 Lung tissue 1.93E-50 2.83E-01 1.44E+00
Lupus erythematosus 4A40 Whole blood 2.41E-02 1.76E-01 3.67E-01
Major depressive disorder 6A70-6A7Z Hippocampus 4.66E-01 -2.56E-02 -2.51E-01
Major depressive disorder 6A70-6A7Z Whole blood 1.18E-01 -6.93E-02 -2.90E-01
Melanoma 2C30 Skin 4.73E-02 -5.93E-01 -6.23E-01
Multiple myeloma 2A83.1 Peripheral blood 8.86E-01 -3.42E-02 -1.38E-01
Multiple myeloma 2A83.1 Bone marrow 1.69E-05 5.20E-01 3.40E+00
Multiple sclerosis 8A40 Plasmacytoid dendritic cells 4.33E-01 -9.12E-02 -3.12E-01
Myelodysplastic syndrome 2A36-2A3Z Bone marrow 4.04E-01 -2.24E-03 -1.04E-02
Myelofibrosis 2A20.2 Whole blood 5.35E-01 -1.38E-01 -1.15E+00
Myocardial infarction BA41-BA50 Peripheral blood 1.00E-01 -3.90E-01 -5.21E-01
Myopathy 8C70.6 Muscle tissue 7.26E-01 1.48E-02 7.88E-02
Neonatal sepsis KA60 Whole blood 3.81E-08 -2.05E-01 -8.28E-01
Neuroectodermal tumour 2A00.11 Brain stem tissue 6.20E-05 4.02E-01 2.07E+00
Non-alcoholic fatty liver disease DB92 Liver tissue 7.16E-01 -9.07E-02 -3.90E-01
Obesity related type 2 diabetes 5A11 Omental adipose tissue 4.57E-01 2.99E-02 1.91E-01
Olive pollen allergy CA08.00 Peripheral blood 6.70E-02 1.36E-01 1.02E+00
Oral cancer 2B6E Oral tissue 1.61E-05 2.29E-01 9.91E-01
Osteoarthritis FA00-FA0Z Synovial tissue 8.22E-01 -1.53E-01 -4.46E-01
Osteoporosis FB83.1 Bone marrow 2.80E-02 3.13E-01 2.01E+00
Ovarian cancer 2C73 Ovarian tissue 7.94E-07 7.40E-01 3.97E+00
Pancreatic cancer 2C10 Pancreas 1.32E-03 -2.43E-01 -1.07E+00
Parkinson's disease 8A00.0 Substantia nigra tissue 2.86E-01 -6.33E-02 -3.07E-01
Pediatric respiratory syncytial virus infection CA40.11 Peripheral blood 9.09E-01 3.83E-02 3.62E-01
Pituitary cancer 2D12 Pituitary tissue 1.03E-04 3.57E-01 1.87E+00
Pituitary gonadotrope tumour 2D12 Pituitary tissue 9.86E-07 8.20E-01 4.07E+00
Polycystic ovary syndrome 5A80.1 Vastus lateralis muscle 2.72E-01 -3.35E-02 -2.85E-01
Polycythemia vera 2A20.4 Whole blood 2.69E-12 -1.78E-01 -1.45E+00
Pompe disease 5C51.3 Biceps muscle 4.32E-03 3.95E-01 1.76E+00
Preterm birth KA21.4Z Myometrium 8.04E-01 3.22E-02 4.67E-01
Prostate cancer 2C82 Prostate 2.13E-10 6.62E-01 2.23E+00
Psoriasis EA90 Skin 1.63E-11 2.15E-01 6.24E-01
Rectal cancer 2B92 Rectal colon tissue 2.39E-01 -1.17E-01 -7.82E-01
Renal cancer 2C90-2C91 Kidney 3.45E-04 -3.88E-01 -1.65E+00
Retinoblastoma 2D02.2 Uvea 3.08E-05 4.94E-01 3.15E+00
Rheumatoid arthritis FA20 Synovial tissue 3.58E-01 -2.51E-01 -6.78E-01
Rhinovirus infection CA42.1 Nasal epithelium tissue 3.08E-01 2.95E-02 2.11E-01
Schizophrenia 6A20 Prefrontal cortex 3.19E-02 -2.03E-02 -1.58E-01
Schizophrenia 6A20 Superior temporal cortex 2.10E-01 -2.15E-02 -1.60E-01
Scleroderma 4A42.Z Whole blood 2.04E-01 9.53E-02 7.50E-01
Seizure 8A60-8A6Z Whole blood 8.81E-01 1.62E-01 6.56E-01
Sensitive skin EK0Z Skin 7.60E-01 5.12E-03 2.55E-02
Sepsis with septic shock 1G41 Whole blood 1.79E-02 -6.77E-02 -2.62E-01
Shwachman-Diamond syndrome 3A70.0 Bone marrow 1.56E-01 -1.44E-01 -1.01E+00
Sickle cell disease 3A51.0-3A51.3 Peripheral blood 6.02E-02 -2.78E-01 -1.10E+00
Simpson golabi behmel syndrome LD2C Adipose tissue 6.43E-01 -1.36E-02 -9.24E-02
Sjogren's syndrome 4A43.2 Salivary gland tissue 4.11E-01 -9.10E-02 -2.26E-01
Skin cancer 2C30-2C3Z Skin 1.80E-08 2.97E-01 5.39E-01
Thrombocythemia 3B63 Whole blood 1.04E-06 -1.66E-01 -1.42E+00
Thrombocytopenia 3B64 Whole blood 3.72E-01 -2.20E-01 -5.14E-01
Thyroid cancer 2D10 Thyroid 6.88E-01 -1.63E-02 -7.81E-02
Tibial muscular dystrophy 8C75 Muscle tissue 4.92E-01 3.44E-03 2.19E-02
Tuberous sclerosis complex LD2D.2 Perituberal tissue 8.59E-01 4.12E-02 6.12E-01
Type 2 diabetes 5A11 Liver tissue 6.58E-01 9.58E-03 4.87E-02
Ureter cancer 2C92 Urothelium 4.76E-01 2.11E-02 5.25E-02
Uterine cancer 2C78 Endometrium tissue 5.44E-03 1.10E-01 3.22E-01
Vitiligo ED63.0 Skin 3.92E-01 1.28E-01 4.88E-01
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⏷ Show the Full List of DME Expression Under 107 Diseases

References

1 How many drug targets are there? Nat Rev Drug Discov. 2006 Dec;5(12):993-6.