Chemistry MT AAB

Chemistry MT AAB What are the functions of proteins? - ANSWER -Transportation of metabolic substances. -Part of the cell membranes. -Part of hormones and receptors. -Components of Enzymes. Proteins are composed of: - ANSWER -Carbon -Oxygen -Hydrogen -Nitrogen (differentiates proteins from carbohydrates & lipids). -Sulfur *Proteins have a high molecular weight, making them good immunogens. What proteins are soluble? - ANSWER Albumin- Soluble in water Globulins- Soluble in dilute salt solutions Histones- Soluble in dilute acids Basic unit: Amino Acids are linked by what? - ANSWER Peptide bonds. -Short chain hydrocarbons w/carboxyl and amine group. *Each amino acid may act as an acid or a base. What is the structure of peptide bonds? - ANSWER Primary Stucture: Sequence of amino acids. Secondary Structure: Hydrogen bonding. Tertiary Structure: Disulfide bonding. Quaternary Structure: Electrostatic Attraction. What is protein denaturation? - ANSWER The change of protein structure. Can be caused by: - Change in temperature (rupture weak secondary & tertiary bonds) -Change in pH (alter ionic charge) -Hydrolysis -UV light What are Aminoacidopathies? - ANSWER Inherited disorders of amino acid metabolism such as: -Phenylketonuria -Tyrosinemia -Alkaptonuria -Maple Syrup Disease -Cystinuria (not an enzyme deficiency) Name the disorder: Enzyme deficiency of phenylalanine hydrolase; which results in accumulation of phenylalanine in blood and phenylpyruvic acid in urine? - ANSWER Phenylketonuria *Inherited *Amino acid metabolism disorder (Aminoacidpathy) *Testing includes PKU and Gutherie Tests PKU and Gutherie Tests are used to diagnose what disorder? - ANSWER Phenylketonuria Name the disorder: Enzyme deficiency of tyrosine aminotransfernase that results in the accumulation of tyrosine? - ANSWER Tyrosinemia *Inherited *Amino acid metabolism disorder (Aminoacidpathy) Name the disorder: An enzyme deficiency of homogentisic oxidase that results in the accumulation of homogentisic acid? - ANSWER Alkaptonuria *Inherited *Amino acid metabolism disorder (Aminoacidpathy) Name the disorder: An enzyme deficiency of branched chain keto acid decarboxylase that results in the accumulation of leucine, isoleucine, and valine? Has characteristic Maple syrup smell. - ANSWER Maple Syrup Disease *Inherited *Amino acid metabolism disorder (Aminoacidpathy) Name the disorder: Caused by an amino acid transport system defect that causes renal calculi in the kidney tubules? - ANSWER Cystinuria *Cysteine is insoluble and precipitates (thus forming kidney stones) *NOT an enzyme deficiency *Inherited *Amino acid metabolism disorder (Aminoacidpathy) Simple proteins contain . . ? - ANSWER Peptide chains only. Conjugated proteins contain a protein and . . . ? - ANSWER A prosthetic group. Such as: Lipoproteins Glycoproteins Mucoproteins Nucleoproteins Plasma contains what proteins? - ANSWER Albumin, Alpha1-globulins, Alpha2- globulins, Beta-globulins, Gamma-Globuins. Haptoglobin and Ceruloplasmin are . . . - ANSWER Alpha2-globulins