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Blood plasma proteins
Proteins found in the liquid medium of blood after the removal of blood cells, synthesized mainly in the liver, and play various roles in the body.
Albumins
A type of blood plasma protein making up about 60% of total plasma proteins, responsible for maintaining oncotic pressure, transporting compounds, and binding drugs.
Globulins
Another type of blood plasma protein making up 36% of total protein amount, comprising different subtypes like alpha, beta, and gamma globulins.
Free amino acid pool
The collection of amino acids in blood plasma derived from food, intracellular breakdown of proteins, and keto-carboxylic acid formation.
Protein metabolism
The process involving the breakdown (catabolism) and synthesis (anabolism) of proteins in the body.
Deamination
The removal of an amino group from amino acids, which can occur directly or indirectly through transamination.
Transamination
The transfer of an amino group from one molecule to another, often involving the conversion of amino acids.
Ammonia detoxification
The process of converting toxic ammonia into urea for excretion from the body.
Nitrogen balance
The equilibrium between nitrogen intake (mainly from proteins) and excretion (mainly as urea, uric acid, and creatinine) in the body, reflecting protein homeostasis.
Oxidative deamination
A process where an amino acid loses an amine group in the form of ammonia.
Transamination
A reaction where an amino group is transferred from an amino acid to a keto acid to form a new amino acid and keto acid.
Pyridoxal phosphate
A coenzyme derived from Vitamin B6, essential for various reactions including transamination and deamination.
Glucose-Alanine cycle
A process linking the production of alanine in muscles to glucose synthesis in the liver, efficient during starvation.
Glucogenic amino acids
Amino acids whose breakdown leads to the production of intermediates that can be used for glucose synthesis.
Ketogenic amino acids
Amino acids whose breakdown leads to the production of acetyl-CoA or acetoacetyl-CoA, used for ketone body synthesis.
Ammonium
Toxic byproduct produced during amino acid deamination, detoxified through various mechanisms including urea synthesis.
Urea Cycle
A series of enzymatic reactions in the liver that detoxify ammonium by converting it into urea for excretion.
Hyperuremia
Condition where blood plasma urea concentration exceeds the normal range.
Hypouremia
Condition where blood plasma urea concentration falls below the normal range.
Hyperammonemia
A condition characterized by high levels of ammonia in the blood, exceeding 47 micromole/L, posing a threat to life due to the inactivation of the Krebs cycle.
Urea Cycle
A metabolic pathway that eliminates toxic ammonia from the body by converting it into urea for excretion.
Glutamate Dehydrogenase
An enzyme involved in catalyzing reactions related to the metabolism of amino acids and the production of ammonia.
Pyrimidine Nucleotide Metabolism
The biochemical processes involved in the synthesis, breakdown, and regulation of pyrimidine nucleotides essential for DNA and RNA synthesis.
De Novo Synthesis
The process of synthesizing complex molecules from simple precursors, such as the production of pyrimidine nucleotides from basic components.
Carbamoyl Phosphate Synthetase
An enzyme involved in the first step of pyrimidine nucleotide synthesis, distinct from CPSI and crucial for the urea cycle and pyrimidine biosynthesis.
Salvage Pathway
A route for recycling nucleotide bases by converting them back into nucleotides, involving specific enzymes like pyrimidine nucleoside phosphorylase and nucleoside kinases.
Feedback Inhibition
A regulatory mechanism in which the end product of a pathway inhibits an enzyme earlier in the pathway, crucial for controlling pyrimidine and purine nucleotide synthesis.
Orotic aciduria
A rare disorder characterized by the excretion of orotic acid in urine due to defective enzymes involved in pyrimidine nucleotide metabolism.
UMP synthase
Enzyme responsible for converting orotic acid to UMP in the pyrimidine synthesis pathway.
Ribonucleotide reductase
Enzyme involved in the reduction of ribose moieties in nucleotides to deoxyribose for DNA synthesis.
Thymidylate synthase
Enzyme that catalyzes the methylation of dUMP to form dTMP in the synthesis of thymidine phosphates.
Deoxyribonucleotides
Nucleotides with a deoxyribose sugar moiety, essential for DNA synthesis.
PRPP synthetase
Enzyme that catalyzes the production of activated 5-phosphoribose-1-pyrophosphate in the priming reaction of de novo purine nucleotide synthesis.
NADPH
Primary reductant required for the reduction of ribose moieties in nucleotides during DNA synthesis.
Salvage pathway
Pathway for the synthesis of purine nucleotides from purine bases, utilizing preformed nucleobases.
Carbamoyl synthetase II
Enzyme involved in the regulation of pyrimidine production in the urea cycle deficiency-induced orotic aciduria.
Thymidine phosphates
Deoxyribonucleoside phosphates essential for DNA synthesis.
De novo synthesis
The process of synthesizing a compound from simple molecules, typically without the aid of biological templates.
Allosteric enzyme
An enzyme that changes its conformation and activity upon binding to a specific effector molecule at a site other than the active site.
Feedback inhibition
The process in which the end product of a biochemical pathway inhibits an earlier step in the pathway to regulate its own production.
Salvage pathway
A pathway in which a compound is regenerated from its degradation products, allowing for the recycling of molecules and conservation of energy.
PRPP (Phosphoribosyl pyrophosphate)
A key molecule in nucleotide synthesis, serving as a precursor for the de novo synthesis of purine and pyrimidine nucleotides.
APRT (Adenine-phosphoribosyl transferase)
An enzyme involved in the salvage pathway of purine nucleotide synthesis, catalyzing the formation of adenine nucleotides.
HGPRT (Hypoxanthine-guanine phosphoribosyl transferase)
An enzyme that plays a crucial role in the salvage pathway of purine nucleotide synthesis, facilitating the formation of guanine and hypoxanthine nucleotides.
Uric acid
The end product of purine metabolism in humans, excreted primarily through urine, with abnormal levels leading to conditions like gout.