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Chem 2 Ch.10
Amino Acids and Proteins
| Term | Definition |
|---|---|
| Amino Acids | -building blocks of proteins |
| Peptide bond | amino acids joining together |
| Aminoacidopathies | class of inherited errors of metabolism in which there is an enzyme defect that inhibits the body's ability to metabolize certain amino acids |
| Phenylketonuira | Absence of activity of the enzyme phenylalanine hydroxylase |
| Tyrosinemia | -Inborn metabolic disorders -characterized by the excretion of tyrosine and tyrosine catabolites in the urine. -5 different enzymes that are needed to break down tyrosine |
| Alkaptonuria | -transmitted as an autosomal recessive gene ( HGD), which causes the lack of the enzyme homogentisate oxidase which is needed in the metabolism of tyrosine and phenylalanine. |
| Maple Syrup Urine Disease | -absence or greatly reduced activity of the enzyme branched-chain a-ketoacid decarboxylase -blocks the normal metabolism of the three essential branched-chain amino acids leucine, isolucine and valine. - autosomal recessive genetic inherited disorder. |
| Isovaleric Acidemia | -autosomal recessive metabolic disorder from a deficiency of the enzyme isovaleryl-CoA dehydrogenase -prevents normal metabolism of the branched-chain amino acid leucine |
| Homocystinuria | lack of the enzyme cystathionine-B synthetase -necessary for the metabolism of the amino acid methionine |
| Cystinuria | -defect in the amino acid transport system. -inadequate reabsorption of cystine during the filtering process in the kidneys. |
| Primary Structure | # and types of amino acids in the sequence |
| Secondary Structure | repeating structures stabilized by hydrogen bonds between the amino acids within the protein. ( Pleated sheet or helix) |
| Tertiary Structure | overall shape ( fold) |
| Quaternary Structure | shape or structure that results from the interaction of more than one protein molecule. (3D) |
| Denaturation | loss of its native structure. |
| Proteinuria | |
| Simple proteins | -contain peptide chains composed of only amino acids. -globular or fibrous |
| Conjugated Proteins | -consist of a protein and a nonprotein (prosthetic) group -the prosthetic groups could be lipid, carb, porphyrins & metals -something is JOINED to it |
| Globulins | a1-Globulns a2-Globulins Haptoglobulins B-globulins |
| Alpha 1-Antitrypsin (AAT) | -glycoprotein mainly synthesized in the lover - most important function inhibition of the protease neutrophil elastase. |
| Alpha1- Fetaprotein | -synthesized in the developing embryo and fetus and than by parenchymal cells of the liver. |
| Haptoglobin | -a2-glycoprotein synthesized in the hepatocytes. -elevated in many inflammatory diseases |
| Transferrin | -glycoprotein -negative acute-phase protein -synthesized in liver |
| Siderophilin | transferrin |
| Lipoproteins | -complexes of proteins and lipids -transport cholesterol, triglycerides & phospholipids in blood |
| B2-Microglobulin | -histocompatibility complex -present in high concentrations on lymphocytes |
| Complement | one of the natural defense mechanisms that protects the human body from infections. -synthesized in the liver as single polypeptide chains and circulate blood as nonfunctional precursors. |
| Fibrinogen | -one of the largest proteins in blood plasma -synthesized in liver |
| C-Reactive Protein | -one of the first acute-phase proteins to rise in response to inflammatory disease. |
| Immunoglobulins | -IgG is most abundant -IgG crosses placenta -antibodies produced by the B lymphocyte that confer humoral immunity. |
| Myoglobin | -153 amino acids -primary oxygen-carrying protein -muscle protein -toxic to the kidneys and kidneys can be damaged |
| Troponin | -"gold standard" |
| Brain Natriuretic Peptide | -popular marker for congestive heart failure -found in left ventricle myocardium |
| Cystatin C | -low molecular mass protein -120 amino acids -marker for early assessment of changes to the glomerulus filtration rate |
| Hypoproteinemia | Low total protein and occurs in any condition where a negative nitrogen balance exists. |
| Hyperproteinemia | increase in total plasma proteins, not a disease state but underlying cause of dehydration |
| Growth,repair and maintenance of all cells are dependent on? | Amino acids |
| Amino acids contains? ( basic structure) | at least one of both amino and carboxylic acid functional groups |
| (-NH2) and ( -COOH ) bond to the a-carbon with the amino group of one amino acid linking with the carboxyl group | peptide bond |
| catalyzes the conversion of phenylalanine to tyrosine | Phenylalanine hydroxylase |
| Type 1 tyrosinemia | -most severe 1 in 100,000 births -caused by low levels of the enzyme fumarylacetoacetate hydrolase |
| Type 2 tyrosinemia | -caused by a deficiency of the enzyme tyrosine aminotransferase |
| Type 3 tyrosinemia | -rare disorder -caused by a deficiency of the enzyme 4-hydroxyphenylpyruvate dioxygenase. |
| Urine turns brownish-black when mixes with air ( aminocidopathy) | Alkaptonuria |
| sweaty feet odor | Isovaleric Acidemia |
| Cause of a1-Antitrypsin | Mutations in the SERPINA1 gene |
| Protein used to evaluate the rheumatic diseases and increased in burns and nephrotic syndrome | Haptoglobin |
| Functions of transferrin | transport of iron and the prevention of loss of iron through the kidney |
| low transferrin levels | -impaired hemoglobin production and lead to anemia. -due to poor production of transferrin as seen in liver disease or malnutrition or excessive loss of transferrin through the kidneys into the urine. |
| increased transferrin levels | iron deficient anemia |
| low prealbumin levels | hepatic damage, acute phase inflammatory response and tissue necrosis. -poor nutrition |
| increased prealbumin levels | patients receiving steroids, in alcoholism and in chronic renal failure. |
| low albumin levels | malnutrition, liver disease, hypothyroidism, dilution by excess, acute disease states, redistribution |
| high albumin levels | seen ONLY with dehydration or after excessive albumin infusion |
| decreased levels of ceruloplasmin | wilsons disease |
| increases levels of ceruloplasmin | inflammation, severe infection & tissue damage |
| elevated serum levels of B2-Microglobulin | impaired clearance by the kidney or overproduction of the protein that occurs in inflammatory diseases. |
| increased complement | inflammatory states |
| decreased complement | malnutrition and hemolytic anemia. |
| most abundant complement protein in human serum | C3 |
| decreased levels of C3 | autoimmune disease, neonatal respiratory distress syndrome, bacteremia, tissue injury and chronic hepatitis. |
| increased fibrinogen levels | inflammatory, pregnancy and use of oral contraceptives |
| decreased fibrinogen | reflect extensive coagulation |
| increased C-Reactive protein | acute rheumatic fever, bacterial infections, myocardial infarctions, rheumatoid arthritis, carcinomatosis, gout and viral infections |
| % of protein composition in immunoglobulins | 82%-96% |
| % of carbohydrate composition in immunoglobulins | 4%-18% |
| increased IgG | liver disease, infections, IgG myeloma, parasitic disease and many rheumatic diseases |
| decreased IgG | acquired immunodeficiency, protein-losing states and non-IgG myeloma |
| Increased IgA | liver disease, infections and autoimmune diseases |
| decreased IgA | depressed protein synthesis and immunodeficiency |
| Increased IgM | toxoplasmosis, primary biiary cirrhosis, cytomegalovirus, rubella, herpes, syphilis and various bacterial and fungal diseases. |
| decreased IgM | protein-losing conditions and hereditary immunodeficiency |
| Increased IgD | infections, liver disease and connective tissue disorders |
| Increased IgE | infectious diseases, asthma, hay fever & IgE myeloma |
| increased myoglobin levels | progressive muscular dystrophy & crushing injury in which skeletal muscle is damaged. & renal failure |
| increased troponin levels | heart injury is present or that the injury took place more than 24 hours in the past. |
| most common test methods for testing proteins | Biuret and Die Binding |
| what test used copper ions that caused a deep blue color during total protein analysis | Biuret |
| Diseases associated with alterations in cerebrospinal fluid proteins | Bacterial, viral and fungal meningitis; traumatic tap; multiple sclerosis; obstruction; neoplasm; disk herniation & cerebral infarcation |
| a1-Antitrypsin Deficiency on elecrophoresis | normal albumin, decrease a1 fraction |
| Monoclonal Increase on elecrophoresis | Increased gamma globulin |
| Nephrotic Syndrome on electrophoresis | Decreased Albumin, Increased a2 and Beta globulins |
| Inflammation on electrophoresis | Increased a1, a2 and beta globulins |
| Cirrhosis on electrophoresis | Beta & Gamma Bridge |
| Order of fractions on electrophoresis | Albumin, a1, a2, beta than gamma |
| Reference range for Total Protein | 65-83 g/L |
| Reference range for Albumin | 35-55 g/L |
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Sdevries0982
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