serw-MX  [xml]  
 


    
 DeCS Categories

C06 Digestive System Diseases .
C06.552 Liver Diseases .
C06.552.830 Porphyrias, Hepatic .
C06.552.830.150 Porphyria, Acute Intermittent .
C16 Congenital, Hereditary, and Neonatal Diseases and Abnormalities .
C16.320 Genetic Diseases, Inborn .
C16.320.850 Skin Diseases, Genetic .
C16.320.850.742 Porphyrias, Hepatic .
C16.320.850.742.150 Porphyria, Acute Intermittent .
C17 Skin and Connective Tissue Diseases .
C17.800 Skin Diseases .
C17.800.827 Skin Diseases, Genetic .
C17.800.827.742 Porphyrias, Hepatic .
C17.800.827.742.150 Porphyria, Acute Intermittent .
C18 Nutritional and Metabolic Diseases .
C18.452 Metabolic Diseases .
C18.452.811 Porphyrias .
C18.452.811.400 Porphyrias, Hepatic .
C18.452.811.400.150 Porphyria, Acute Intermittent .
D04 Polycyclic Compounds .
D04.210 Fused-Ring Compounds .
D04.210.500 Steroids .
D04.210.500.247 Cholestanes .
D04.210.500.247.222 Cholestenes .
D04.210.500.247.222.222 Cholestadienes .
D04.210.500.247.222.222.347 Cholestadienols .
D04.210.500.247.222.222.347.231 Desmosterol .
D04.210.500.247.808 Sterols .
D04.210.500.247.808.197 Cholesterol .
D04.210.500.247.808.197.250 Dehydrocholesterols .
D04.210.500.247.808.197.250.281 Desmosterol .
D08 Enzymes and Coenzymes .
D08.811 Enzymes .
D08.811.464 Ligases .
D08.811.464.259 Carbon-Nitrogen Ligases .
D08.811.464.259.300 Argininosuccinate Synthase .
D08.811.682 Oxidoreductases .
D08.811.682.662 Oxidoreductases Acting on CH-NH Group Donors .
D08.811.682.662.693 1-Pyrroline-5-Carboxylate Dehydrogenase .
D08.811.913 Transferases .
D08.811.913.225 Alkyl and Aryl Transferases .
D08.811.913.225.431 Farnesyl-Diphosphate Farnesyltransferase .
D10 Lipids .
D10.570 Membrane Lipids .
D10.570.938 Sterols .
D10.570.938.208 Cholesterol .
D10.570.938.208.320 Dehydrocholesterols .
D10.570.938.208.320.297 Desmosterol .
E02 Therapeutics .
E02.319 Drug Therapy .
E02.319.353 Enzyme Therapy .
G02 Chemical Phenomena .
G02.111 Biochemical Phenomena .
G02.111.570 Molecular Structure .
G02.111.570.120 Binding Sites .
G02.111.570.120.704 Catalytic Domain .
G02.111.570.820 Molecular Conformation .
G02.111.570.820.709 Protein Conformation .
G02.111.570.820.709.275 Protein Structural Elements .
G02.111.570.820.709.275.750 Protein Domains .
G02.111.570.820.709.275.750.188 Catalytic Domain .
G05 Genetic Phenomena .
G05.308 Gene Expression Regulation .
G05.308.320 Gene Expression Regulation, Enzymologic .
G05.308.320.200 Enzyme Induction .
G05.308.320.300 Enzyme Repression .
 
 Terms
 Synonyms & Historicals
Documents
LILACS e MDL
 
Enzyme Repression .
Repression, Enzyme .
The interference in synthesis of an enzyme due to the elevated level of an effector substance, usually a metabolite, whose presence would cause depression of the gene responsible for enzyme synthesis. .
1.00
63879
 
Enzyme Induction .
Induction, Enzyme .
An increase in the rate of synthesis of an enzyme due to the presence of an inducer which acts to derepress the gene responsible for enzyme synthesis. .
0.67
3132236
 
Catalytic Domain .
Active Sites .
Catalytic Cores .
Catalytic Domains .
Catalytic Regions .
Catalytic Sites .
Catalytic Subunits .
Core, Catalytic .
Cores, Catalytic .
Domain, Catalytic .
Domains, Catalytic .
Reactive Sites .
Region, Catalytic .
Regions, Catalytic .
Site, Active .
Site, Catalytic .
Site, Reactive .
Sites, Active .
Sites, Catalytic .
Sites, Reactive .
Subunit, Catalytic .
Subunits, Catalytic .
Active Site .
Catalytic Core .
Catalytic Region .
Catalytic Site .
Catalytic Subunit .
Reactive Site .
The region of an enzyme that interacts with its substrate to cause the enzymatic reaction. .
0.42
824355
 
Farnesyl-Diphosphate Farnesyltransferase .
FPP-FPP Farnesyl Transferase .
Farnesyldiphosphate-Farnesyldiphosphate Farnesyltransferase .
Presqualene-Diphosphate Synthase .
Squalene Synthase .
FPP FPP Farnesyl Transferase .
Farnesyl Diphosphate Farnesyltransferase .
Farnesyl Transferase, FPP-FPP .
Farnesyldiphosphate Farnesyldiphosphate Farnesyltransferase .
Farnesyltransferase, Farnesyl-Diphosphate .
Farnesyltransferase, Farnesyldiphosphate-Farnesyldiphosphate .
Presqualene Diphosphate Synthase .
Synthase, Presqualene-Diphosphate .
Synthase, Squalene .
Synthetase, Squalene .
Transferase, FPP-FPP Farnesyl .
Squalene Synthetase .
The first committed enzyme of the biosynthesis pathway that leads to the production of STEROLS. it catalyzes the synthesis of SQUALENE from farnesyl pyrophosphate via the intermediate PRESQUALENE PYROPHOSPHATE. This enzyme is also a critical branch point enzyme in the biosynthesis of ISOPRENOIDS that is thought to regulate the flux of isoprene intermediates through the sterol pathway. .
0.41
0537
 
Desmosterol .
Demosterol .
24 Dehydrocholesterol .
24-Dehydrocholesterol .
An intermediate in the synthesis of cholesterol. .
0.38
0342
 
Enzyme Therapy .
Enzymotherapy .
Systemic Enzyme Therapy .
Enzyme Therapies .
Enzyme Therapies, Systemic .
Enzyme Therapy, Systemic .
Enzymotherapies .
Systemic Enzyme Therapies .
Therapies, Enzyme .
Therapies, Systemic Enzyme .
Therapy, Enzyme .
Therapy, Systemic Enzyme .
The use of enzymes to correct metabolic and physiological processes. .
0.38
263
 
1-Pyrroline-5-Carboxylate Dehydrogenase .
Delta(1)-Pyrroline-5-Carboxylate Dehydrogenase .
GGS Dehydrogenase .
Glutamic-Gamma-Semialdehyde Dehydrogenase .
Glutamyl-Gamma-Semialdehyde Dehydrogenase .
NADP+ Specific 8-Glutamate Semialdehyde Dehydrogenase .
1 Pyrroline 5 Carboxylate Dehydrogenase .
Dehydrogenase, 1-Pyrroline-5-Carboxylate .
Dehydrogenase, GGS .
Dehydrogenase, Glutamic-Gamma-Semialdehyde .
Dehydrogenase, Glutamyl-Gamma-Semialdehyde .
Glutamic Gamma Semialdehyde Dehydrogenase .
Glutamyl Gamma Semialdehyde Dehydrogenase .
NADP+ Specific 8 Glutamate Semialdehyde Dehydrogenase .
An enzyme that catalyzes the oxidation of 1-pyrroline-5-carboxylate to L-GLUTAMATE in the presence of NAD. Defects in the enzyme are the cause of hyperprolinemia II. .
0.38
0140
 
Argininosuccinate Synthase .
Argininosuccinate Synthetase .
Synthase, Argininosuccinate .
Synthetase, Argininosuccinate .
An enzyme of the urea cycle that catalyzes the formation of argininosuccinic acid from citrulline and aspartic acid in the presence of ATP. Absence or deficiency of this enzyme causes the metabolic disease CITRULLINEMIA in humans. EC 6.3.4.5. .
0.37
1724
 
Porphyria, Acute Intermittent .
Acute Porphyria .
PBGD Deficiency .
Porphobilinogen Deaminase Deficiency .
Porphyria, Swedish Type .
UPS Deficiency .
Acute Intermittent Porphyria .
Acute Intermittent Porphyrias .
Acute Porphyrias .
Deaminase Deficiencies, Porphobilinogen .
Deaminase Deficiency, Porphobilinogen .
Deficiencies, Hydroxymethylbilane Synthase .
Deficiencies, PBGD .
Deficiencies, Porphobilinogen Deaminase .
Deficiencies, UPS .
Deficiencies, Uroporphyrinogen Synthase .
Deficiency, Hydroxymethylbilane Synthase .
Deficiency, PBGD .
Deficiency, Porphobilinogen Deaminase .
Deficiency, UPS .
Deficiency, Uroporphyrinogen Synthase .
Hydroxymethylbilane Synthase Deficiencies .
Intermittent Porphyria, Acute .
Intermittent Porphyrias, Acute .
PBGD Deficiencies .
Porphobilinogen Deaminase Deficiencies .
Porphyria, Acute .
Porphyrias, Acute .
Porphyrias, Acute Intermittent .
Porphyrias, Swedish Type .
Swedish Type Porphyria .
Swedish Type Porphyrias .
Synthase Deficiencies, Hydroxymethylbilane .
Synthase Deficiencies, Uroporphyrinogen .
Synthase Deficiency, Hydroxymethylbilane .
Synthase Deficiency, Uroporphyrinogen .
Type Porphyria, Swedish .
Type Porphyrias, Swedish .
UPS Deficiencies .
Uroporphyrinogen Synthase Deficiencies .
Hydroxymethylbilane Synthase Deficiency .
Uroporphyrinogen Synthase Deficiency .
An autosomal dominant porphyria that is due to a deficiency of HYDROXYMETHYLBILANE SYNTHASE in the LIVER, the third enzyme in the 8-enzyme biosynthetic pathway of HEME. Clinical features are recurrent and life-threatening neurologic disturbances, ABDOMINAL PAIN, and elevated level of AMINOLEVULINIC ACID and PORPHOBILINOGEN in the urine. .
0.37
32725