Amino Acids & IEMs
A medical procedure involving filter paper and a dried blood spot which allows for early detection of IEMs.
What is newborn screening?
Polymer of amino acids linked by peptide bonds with a specific sequence, which has a wide variety of functions in the human body.
What are proteins?
An acute phase reactant synthesized in utero by the developing embryo. Increased levels can indicate spina bifida, and decreased levels can indicate down syndrome.
What is Alpha-1-Fetoprotein?
Glycoproteins produced by B-lymphocytes. There are 5 classes.
What are immunoglobulins?
A cascade of inflammatory events which functions to recruit effector phagocytes for opsonization, and to trigger direct destruction of foreign organisms.
What is the complement system?
The unique side chains responsible for the chemical properties of individual amino acids.
What is the R side chain (variable side chain)?
A negative acute phase reactant and transport protein for thyroid hormones and Vitamin A.
What is Prealbumin?
An acute phase reactant synthesized in the liver which mainly functions to inhibit neutrophil elastase.
What is Alpha-1-Antitrypsin?
A negative acute phase reactant which transports iron.
Hepatic damage, acute-phase inflammatory response, and poor nutritional status.
What are the causes of decreased prealbumin levels?
Isoleucine, Leucine, Lysine, Methionine, Phenylalanine, Threonine, Tryptophan, Valine
What are the essential amino acids?
A process in which triplet codons in mRNA are matched with a complementary sequence in tRNA carrying specific amino acids.
What is protein synthesis?
An acute phase reactant which functions to bind hemoglobin to prevent the loss of iron.
What is Haptoglobin?
A positive acute-phase reactant which forms a fibrin clot when activated by thrombin.
What is fibrinogen?
An acute phase reactant (Alpha-2-Globulin) which functions to transport copper.
What is ceruloplasmin?
Deficiency of the branched-chain alpha-keto acid dehydrogenase complex. Leads to the buildup of leucine, isoleucine, and valine.
What is the cause of Maple Syrup Urine disease?
An analytical technique which separates proteins based on their electrical charge and weight.
What is serum electrophoresis?
An acute phase reactant that inhibits protease. It is increased in inflammation and renal disease secondary to diabetes mellitus.
An analytical technique used to quantify total protein in serum, in which Curpric ions interact with peptide bonds to form a violet-colored complex which can be read spectrophotometrically.
What is the Biuret Method?
A disorder which results in increased phenylalanine, increased phenylketones, and decreased tyrosine. It can result in severe intellectual disabilities if left untreated.
What is phenylketonuria (PKU)?
An acute phase reactant which maintains the barrier function of capillaries and the transport of drugs and hormones.
What is Alpha-1-Acid Glycoprotein?
One of the strongest acute phase reactants which aids in non-specific host defense against infectious organisms by activating the classical complement pathway.
What is C-reactive protein?
Acute inflammatory responses, liver and kidney disease, malnutrition/malabsorption, sepsis, and excessive fluid intake.
What are the causes of decreased albumin levels?