General Information of Drug-Metabolizing Enzyme (DME) (ID: DESO5ZY)

DME Name Prolyl 4-hydroxylase alpha-1 (P4HA1)
Synonyms Procollagen-proline,2-oxoglutarate-4-dioxygenase subunit alpha-1; Prolyl 4-hydroxylase subunit alpha-1; 4-PH alpha-1; P4HA; P4HA1
Gene Name P4HA1
UniProt ID
P4HA1_HUMAN
INTEDE ID
DME0461
3D Structure
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2D Sequence (FASTA)
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3D Structure (PDB)
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Gene ID
5033
EC Number EC: 1.14.11.2
Oxidoreductase
Oxygen paired donor oxidoreductase
2-oxoglutarate donor oxidoreductase
EC: 1.14.11.2
Lineage Species: Homo sapiens
Kingdom: Metazoa
Phylum: Chordata
Class: Mammalia
Order: Primates
Family: Hominidae
Genus: Homo
Species: Homo sapiens
Sequence
MIWYILIIGILLPQSLAHPGFFTSIGQMTDLIHTEKDLVTSLKDYIKAEEDKLEQIKKWA
EKLDRLTSTATKDPEGFVGHPVNAFKLMKRLNTEWSELENLVLKDMSDGFISNLTIQRQY
FPNDEDQVGAAKALLRLQDTYNLDTDTISKGNLPGVKHKSFLTAEDCFELGKVAYTEADY
YHTELWMEQALRQLDEGEISTIDKVSVLDYLSYAVYQQGDLDKALLLTKKLLELDPEHQR
ANGNLKYFEYIMAKEKDVNKSASDDQSDQKTTPKKKGVAVDYLPERQKYEMLCRGEGIKM
TPRRQKKLFCRYHDGNRNPKFILAPAKQEDEWDKPRIIRFHDIISDAEIEIVKDLAKPRL
RRATISNPITGDLETVHYRISKSAWLSGYENPVVSRINMRIQDLTGLDVSTAEELQVANY
GVGGQYEPHFDFARKDEPDAFKELGTGNRIATWLFYMSDVSAGGATVFPEVGASVWPKKG
TAVFWYNLFASGEGDYSTRHAACPVLVGNKWVSNKWLHERGQEFRRPCTLSELE
Function This enzyme catalyzes the post-translational formation of 4- hydroxyproline in -Xaa-Pro-Gly- sequences in collagens and other proteins.
KEGG Pathway
( )
( )
Reactome Pathway
Collagen biosynthesis and modifying enzymes (R-HSA-1650814 )

Molecular Interaction Atlas (MIA) of This DME

Molecular Interaction Atlas (MIA) Jump to Detail Molecular Interaction Atlas of This DME
1 Approved Drug(s) Metabolized by This DME
Drug Name Drug ID Indication ICD 11 Highest Status REF
Vitamin C DMXJ7O8 Vitamin C deficiency 5B56 Approved [3]
1 Investigative Drug(s) Metabolized by This DME
Drug Name Drug ID Indication ICD 11 Highest Status REF
Alpha-ketoglutaric acid DM5LFYN Discovery agent N.A. Investigative [3]
Experimental Enzyme Kinetic Data of Drugs
Drug Name Indication Highest Status Kinetic Data REF
Alpha-ketoglutaric acid Discovery agent [N.A.] Investigative Km = 0.022 microM [3]
Vitamin C Vitamin C deficiency [5B56] Approved Km = 0.33 microM [3]

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-22 2.79E-01 1.29E+00
Alopecia ED70 Skin from scalp 1.80E-01 1.47E-02 7.72E-02
Alzheimer's disease 8A20 Entorhinal cortex 2.64E-04 8.06E-02 3.08E-01
Ankylosing spondylitis FA92.0 Pheripheral blood 5.44E-02 -1.63E-01 -5.29E-01
Aortic stenosis BB70 Calcified aortic valve 3.29E-01 4.55E-02 2.78E-01
Apnea 7A40 Hyperplastic tonsil 9.51E-02 3.64E-01 2.03E+00
Arthropathy FA00-FA5Z Peripheral blood 8.29E-01 -5.89E-03 -5.54E-02
Asthma CA23 Nasal and bronchial airway 3.37E-08 -3.40E-01 -3.42E-01
Atopic dermatitis EA80 Skin 1.99E-02 -2.07E-01 -1.20E+00
Autism 6A02 Whole blood 3.12E-01 -5.98E-02 -3.48E-01
Autoimmune uveitis 9A96 Peripheral monocyte 6.43E-01 1.50E-02 1.25E-01
Autosomal dominant monocytopenia 4B04 Whole blood 9.42E-02 2.44E-01 1.13E+00
Bacterial infection of gingival 1C1H Gingival tissue 1.32E-04 1.40E-01 6.11E-01
Batten disease 5C56.1 Whole blood 2.50E-01 -1.22E-01 -1.06E+00
Behcet's disease 4A62 Peripheral blood 8.82E-01 -6.61E-03 -3.37E-02
Bipolar disorder 6A60-6A6Z Prefrontal cortex 5.19E-01 4.17E-03 2.53E-02
Bladder cancer 2C94 Bladder tissue 9.22E-01 -5.33E-03 -2.90E-02
Breast cancer 2C60-2C6Z Breast tissue 2.11E-41 2.84E-01 1.05E+00
Cardioembolic stroke 8B11.20 Whole blood 2.22E-07 4.18E-01 1.92E+00
Cervical cancer 2C77 Cervical tissue 8.51E-02 1.30E-01 3.70E-01
Childhood onset rheumatic disease FA20.Z Peripheral blood 5.95E-02 -8.59E-02 -4.56E-01
Chronic hepatitis C 1E51.1 Whole blood 3.75E-02 -1.67E-01 -1.52E+00
Chronic obstructive pulmonary disease CA22 Lung tissue 1.56E-01 3.73E-02 1.58E-01
Chronic obstructive pulmonary disease CA22 Small airway epithelium 7.76E-01 -1.92E-02 -8.12E-02
Chronic rhinosinusitis CA0A Sinus mucosa tissue 8.30E-01 -7.12E-02 -2.79E-01
Colon cancer 2B90 Colon tissue 6.92E-47 4.01E-01 1.71E+00
Coronary artery disease BA80-BA8Z Peripheral blood 1.39E-01 -1.98E-01 -8.99E-01
Diffuse large B-cell lymphoma 2A81 Tonsil tissue 9.57E-01 -9.39E-02 -3.86E-01
Endometriosis GA10 Endometrium tissue 7.52E-01 -3.98E-02 -1.01E-01
Familial hypercholesterolemia 5C80.00 Peripheral blood 3.01E-01 -2.29E-02 -1.64E-01
Familial hypercholesterolemia 5C80.00 Whole blood 5.76E-12 6.20E-01 1.97E+00
Gastric cancer 2B72 Gastric tissue 9.44E-02 8.82E-02 1.02E+00
Glioblastopma 2A00.00 Nervous tissue 7.44E-65 4.34E-01 1.37E+00
Glioma 2A00.0Y-2A00.0Z Brain stem tissue 9.19E-01 3.21E-02 2.66E-02
Glioma 2A00.0Y-2A00.0Z White matter tissue 7.01E-02 2.44E-01 8.51E-01
Head and neck cancer 2D42 Head and neck tissue 5.74E-09 3.38E-01 7.62E-01
HIV-associated neurocognitive impairment 8A2Y-8A2Z White matter tissue 3.04E-01 -2.14E-02 -4.90E-02
Huntington's disease 8A01.10 Whole blood 3.47E-01 -2.78E-02 -1.80E-01
Idiopathic pulmonary fibrosis CB03.4 Lung tissue 1.60E-01 -2.14E-01 -1.11E+00
Immunodeficiency 4A00-4A20 Peripheral blood 1.43E-02 -7.57E-02 -7.73E-01
Influenza 1E30 Whole blood 5.44E-01 -1.87E-01 -5.34E-01
Interstitial cystitis GC00.3 Bladder tissue 1.30E-01 -1.55E-01 -1.31E+00
Intracranial aneurysm 8B01.0 Intracranial artery 9.25E-07 4.54E-01 2.76E+00
Irritable bowel syndrome DD91.0 Rectal colon tissue 1.77E-01 -5.83E-03 -2.09E-02
Ischemic stroke 8B11 Peripheral blood 3.51E-01 -3.93E-02 -3.44E-01
Juvenile idiopathic arthritis FA24 Peripheral blood 2.28E-02 5.16E-02 2.58E-01
Lateral sclerosis 8B60.4 Skin 1.87E-01 -7.49E-02 -7.36E-01
Lateral sclerosis 8B60.4 Cervical spinal cord 2.92E-01 1.66E-01 6.54E-01
Liver cancer 2C12.0 Liver tissue 7.08E-04 -3.80E-01 -7.33E-01
Liver failure DB99.7-DB99.8 Liver tissue 1.21E-03 -9.87E-01 -1.77E+00
Lung cancer 2C25 Lung tissue 2.77E-53 4.93E-01 1.79E+00
Lupus erythematosus 4A40 Whole blood 1.19E-01 9.20E-02 1.99E-01
Major depressive disorder 6A70-6A7Z Hippocampus 4.31E-02 -4.26E-02 -2.86E-01
Major depressive disorder 6A70-6A7Z Whole blood 4.82E-01 3.00E-02 1.34E-01
Melanoma 2C30 Skin 8.18E-01 2.73E-01 5.55E-01
Multiple myeloma 2A83.1 Peripheral blood 6.25E-01 2.33E-02 1.18E-01
Multiple myeloma 2A83.1 Bone marrow 6.39E-01 -1.71E-01 -7.52E-01
Multiple sclerosis 8A40 Plasmacytoid dendritic cells 4.72E-01 -1.42E-01 -8.47E-01
Myelodysplastic syndrome 2A36-2A3Z Bone marrow 8.16E-01 3.91E-02 1.47E-01
Myelofibrosis 2A20.2 Whole blood 9.47E-01 1.55E-02 1.37E-01
Myocardial infarction BA41-BA50 Peripheral blood 3.90E-01 -2.78E-02 -3.79E-02
Myopathy 8C70.6 Muscle tissue 1.94E-01 -9.14E-02 -7.03E-01
Neonatal sepsis KA60 Whole blood 3.03E-10 2.43E-01 1.09E+00
Neuroectodermal tumour 2A00.11 Brain stem tissue 1.37E-02 2.26E-01 1.65E+00
Non-alcoholic fatty liver disease DB92 Liver tissue 1.03E-01 -1.05E+00 -1.54E+00
Obesity related type 2 diabetes 5A11 Omental adipose tissue 7.85E-01 -5.54E-02 -3.37E-01
Olive pollen allergy CA08.00 Peripheral blood 2.02E-01 4.35E-01 1.27E+00
Oral cancer 2B6E Oral tissue 4.77E-06 6.01E-01 1.28E+00
Osteoarthritis FA00-FA0Z Synovial tissue 8.08E-01 2.36E-01 3.79E-01
Osteoporosis FB83.1 Bone marrow 2.10E-01 3.19E-01 1.38E+00
Ovarian cancer 2C73 Ovarian tissue 2.10E-03 2.34E-01 1.20E+00
Pancreatic cancer 2C10 Pancreas 8.91E-12 4.21E-01 2.88E+00
Parkinson's disease 8A00.0 Substantia nigra tissue 5.80E-05 4.44E-01 2.49E+00
Pediatric respiratory syncytial virus infection CA40.11 Peripheral blood 4.24E-01 -2.28E-02 -1.93E-01
Pituitary cancer 2D12 Pituitary tissue 4.14E-02 6.39E-01 1.33E+00
Pituitary gonadotrope tumour 2D12 Pituitary tissue 6.83E-02 6.31E-01 1.30E+00
Polycystic ovary syndrome 5A80.1 Vastus lateralis muscle 9.51E-01 -1.02E-02 -4.99E-02
Polycythemia vera 2A20.4 Whole blood 2.68E-03 6.36E-02 6.42E-01
Pompe disease 5C51.3 Biceps muscle 2.90E-01 -2.40E-01 -1.21E+00
Preterm birth KA21.4Z Myometrium 4.09E-02 -1.93E-01 -8.42E-01
Prostate cancer 2C82 Prostate 5.62E-04 3.49E-01 7.68E-01
Psoriasis EA90 Skin 6.53E-19 -2.88E-01 -1.14E+00
Rectal cancer 2B92 Rectal colon tissue 7.24E-02 3.31E-01 1.26E+00
Renal cancer 2C90-2C91 Kidney 5.76E-07 7.57E-01 3.18E+00
Retinoblastoma 2D02.2 Uvea 2.47E-07 6.97E-01 2.65E+00
Rheumatoid arthritis FA20 Synovial tissue 1.55E-01 -1.20E-01 -6.87E-01
Rhinovirus infection CA42.1 Nasal epithelium tissue 8.91E-01 -5.57E-02 -2.67E-01
Schizophrenia 6A20 Prefrontal cortex 3.15E-04 2.22E-01 8.28E-01
Schizophrenia 6A20 Superior temporal cortex 2.64E-01 7.50E-02 4.13E-01
Scleroderma 4A42.Z Whole blood 1.43E-01 6.87E-02 6.77E-01
Seizure 8A60-8A6Z Whole blood 5.29E-02 1.23E-01 8.43E-01
Sensitive skin EK0Z Skin 1.70E-01 -7.52E-02 -1.10E+00
Sepsis with septic shock 1G41 Whole blood 1.62E-24 2.18E-01 9.50E-01
Shwachman-Diamond syndrome 3A70.0 Bone marrow 3.03E-02 -2.78E-01 -1.37E+00
Sickle cell disease 3A51.0-3A51.3 Peripheral blood 4.51E-02 -2.31E-01 -4.49E-01
Simpson golabi behmel syndrome LD2C Adipose tissue 4.34E-03 5.73E-01 4.48E+00
Sjogren's syndrome 4A43.2 Salivary gland tissue 1.98E-02 2.06E-01 2.72E+00
Skin cancer 2C30-2C3Z Skin 5.22E-30 3.57E-01 1.41E+00
Thrombocythemia 3B63 Whole blood 1.33E-01 -5.02E-02 -4.61E-01
Thrombocytopenia 3B64 Whole blood 6.31E-01 -1.22E-01 -1.88E-01
Thyroid cancer 2D10 Thyroid 3.36E-30 3.39E-01 1.84E+00
Tibial muscular dystrophy 8C75 Muscle tissue 3.48E-04 -3.74E-01 -1.98E+00
Tuberous sclerosis complex LD2D.2 Perituberal tissue 4.60E-01 -1.16E-03 -7.32E-03
Type 2 diabetes 5A11 Liver tissue 7.56E-01 -1.69E-02 -3.80E-02
Ureter cancer 2C92 Urothelium 6.51E-01 -5.14E-02 -1.53E-01
Uterine cancer 2C78 Endometrium tissue 1.07E-01 9.21E-02 2.30E-01
Vitiligo ED63.0 Skin 9.47E-02 5.13E-02 5.77E-01
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⏷ Show the Full List of DME Expression Under 107 Diseases

The Drug Therapeutic Target (DTT) Role of This DME

DME DTT Name Prolyl 4-hydroxylasesubunit alpha-1 (P4HA1) DTT Info
DME DTT Type Clinical trial
2 Clinical Trial Drug(s) Targeting This DTT
Drug Name Drug ID Indication ICD 11 Highest Status REF
Lufironil DMCKNBA Liver cirrhosis DB93.1 Phase 2 [1], [2]
SAFIRONIL DM1UIQP Transplant rejection NE84 Phase 1 [2]

References

1 Interference in clinical laboratory tests, with special regard to the bilirubin assay: effects of a metabolite of the new prolyl 4-hydroxylase inhibitor, Lufironil. Eur J Clin Chem Clin Biochem. 1994Jul;32(7):515-20.
2 Antifibrogenic therapies in chronic HCV infection. Curr Drug Targets Infect Disord. 2001 Aug;1(2):227-40.
3 Prolyl 4-hydroxylases, the key enzymes of collagen biosynthesis. Matrix Biol. 2003 Mar;22(1):15-24.