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84 Cards in this Set
- Front
- Back
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What enzyme is defective in VonGierke's disease?
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glucose-6-phosphatase
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What enzyme is defective in McArdle's disease?
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glycogen phosphorylase
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What enzyme is deficient in Ehler-Danlos syndrome?
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lysyl oxidase
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What enzyme is deficient in Tay-Sachs disease?
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Beta-hexosaminidase A
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What enzyme is deficient in Gaucher's disease?
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glucocerebrosidase
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What enzyme is deficient in Niemann-Pick disease?
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sphingomyelinase
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What are the clinical features of VonGierke's disease?
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liver enlargement, hyperglycemia, jaundice, ketosis
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What is caused by Ehler-Danlos syndrome?
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loose joints and hyperextensible skin
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What is caused by McArdle's disease?
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muscle cramps
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What is caused by Tay-Sachs disease?
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mental retardation, red spot on macula, death by age 2-3
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What is caused by Gaucher's disease?
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liver and spleen enlargement, erosion of long bones and pelvis, infantile retardation
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What is caused by Niemann-Pick disease?
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liver and spleen enlargement, mental retardation
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What does Tay-Sachs disease cause an increase in?
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ganglioside
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What does Gaucher's disease cause an increase in?
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glucocerebroside
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What does Niemann-Pick disease cause an increase in?
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sphingomyelin
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What fatty acid is 18:2^9,12?
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linoleic acid
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What fatty acid is 16:0?
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palmitic acid
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What fatty acid is 20:4^5, 8, 11, 14?
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arachadonic acid
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What fatty acid is 18:0?
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stearic acid
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What fatty acid is 18:1^9
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oleic acid
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What fatty acid is 18:3^9, 12, 15?
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alpha-linolenic acid
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What is the name of the only intracellular glycosaminoglycan?
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heparin
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What is the name of the glycosaminoglycan with no sulfer?
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hyaluronic acid
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What do glycoproteins typically contain?
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glucose, galactose, mannose, NANA
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What is the function of heparin?
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anticoagulant
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What does a proteoglycan consist of?
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core protein with unbranched glycosaminoglycans
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What does a glycoprotein consist of?
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short branched carbs connected to protein via Asn or Ser/Thr
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What are four functions/roles of glycoproteins?
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hormones, transport, cell-cell interaction, plasma proteins
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What is the function of proteoglycans?
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ground substance in ECM, connective tissue, synovial fluid, and cartilage
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What glycosaminoglycans are a major component of joints?
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chondroitin sulfates
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What is peptidoglycan made of?
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NAG and NAM disaccharides
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What is the function of thromboxane A3?
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anti-clotting mechanism
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What are two essential fatty acids?
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linoleic acid - precursor of arachadonic acid
alpha-linolenic acid - precursor of omega-3 fatty acids |
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What are five functions of lipids?
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storage, energy, structure, insulation, signalling
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What phosphoglyceride has a ring in it?
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phosphatidyl inositol
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What phosphoglyceride has a N-(CH3)3?
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phosphatidyl choline
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What phosphoglyceride has a COO- and an NH3?
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phosphatidyl serine
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What phosphoglyceride has just an NH3?
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phosphatidyl ethanolamine
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What phosphoglyceride has nothing after the phosphate group?
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phosphatidic acid
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What sphingolipid contains phosphorylcholine?
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sphingomyelin
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What sphingolipid typically contains a glucose or galactose?
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cerebroside
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What sphingolipid contains many sugar residues?
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ganglioside
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What do O-linked glycoproteins link to?
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serine and threonine
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What do N-linked glycoproteins link to?
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asparagine
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What kind of linkage does cellulose have?
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Beta 1->4
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What kind of linkage does glycogen have?
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alpha 1->4 with alpha 1->6 branches every 8-12 units
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What kind of linkage does starch have?
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alpha 1->4 with alpha 1->6 branches every 24-30 residues
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What does galactokinase deficieny cause?
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early cataract formation
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What does galactose-1-P uridyl transferase deficiency lead to and cause?
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galactose-1-P accumulation causing liver failure
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What does lactase or sucrase deficiency cause?
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diarrhea and flatulence
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What is cholesterol synthesized from?
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acetyl CoA
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What enzyme is the rate-limiting step in cholesterol production?
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HMG-CoA reductase
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What kind of drugs are used to inhibit the production of cholesterol?
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statins
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What enzyme is needed to convert cholesterol into pregnenolone?
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P450
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What is cholesterol excreted as?
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bile acids
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What two things can be derived from cholesterol?
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steroid hormones and bile salts
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What enzyme is necessary for production of prostaglandins and thromboxanes?
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cyclooxygenase (COX)
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What enzyme is neceassary for production of leukotrienes?
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lipoxygenase
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Which vitamines are fat-soluble?
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D, E, A, and K
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Which side of a membrane are glycoproteins and glycolipids found on?
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exoplasmic side
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What 5 carbon sugar has all H's left/up?
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ribose
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What 5 carbon sugar has an -OH left/up on carbon #2?
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arabinose
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What 5 carbon sugar has an -OH left/up on carbon #3?
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xylose
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What 5 carbon sugar has an -OH left/up on carbons #2 and #3?
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lyxose
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What kind of substance is collagen?
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glycoprotein
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What substance can penicillin block the synthesis of?
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peptidoglycan
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What substances does COX catalyze the formation of?
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prostaglandins and thromboxanes
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What substance does lipooxygenase catalyze the production of?
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leukotrienes
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What substance is used as a lubricant in the knee joint?
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hyaluronate
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Where are D-amino acids found in nature?
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peptidoglycans
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When is maximum buffering capacity achieved?
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pH = pKa
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What are three important buffers in the body?
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bicarbonate, hemoglobin, phosphoric acid
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Which amino acids may be covalently modified by carbohydrate chains
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serine, threonine, asparagine
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Which amino acid forms disulfide bonds?
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cysteine
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What kind of interactions can charged amino acids participate in?
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ionic or electrostatic
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What kind of bonds can hydroxyl groups form?
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hydrogen bonds
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What kind of interactions do aliphatic and aromatic amino acids participate in?
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hydrophobic interactions
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What is the typical pK value of the amino group?
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9
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What is the typical value of the carboxyl group?
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2
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What charge will an amino acid have if pH>pI?
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negative
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What charge will an amino acid have if pH<pI?
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positive
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What are five important unusual amino acids?
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ornithin and cirulline - important in urea cycle
histamine - inflammation (from histidine) GABA - neurotransmission (from glutamate) epinephrine - from tyrosine hydoxyproline/hydroxylysine - post-translational modification |
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Which amino acids have a negative charge (acids)?
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aspartate and glutamate
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Which amino acids have a positive charge (bases)?
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histidine, lysine, arginine
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