Biochemistry Flashcards
In sickle cell anemia, which amino acid is replaced and at which number of position?
Glutamate with valine, position 6
Mode of inheritance of Sickle cell disease:
Homozygous recessive
What are the stop codons?
UAG, UGA, UAA
Syndrome characterized by Hematuria, Bilateral hearing loss, and Lens defect:
Alport’s syndrome
The most common form of Osteogenesis imperfecta is characterized by an abnormality in the what type of collagen and what pattern of inheritance?
Collagen Type I, Autosomal Dominant
Reticulin is formed by which type of collagen?
Type III
What is the earliest symptoms of Vitamin A deficiency?
Night blindness
Demyelination of the posterior columns and spinocerebellar tracts happens when one is deficient in which Vitamin?
Vitamin E
True or false, vitamin B9 deficiency leads to neurologic symptoms.
False (Only Vit B12 deficiency leads to neurologic symptoms
Dermatan sulfate is composed of sugars:
Iduronic acid and N acetyl galactosamine (Heparan sulfate - Glucosamine and glucuronic acid, Heparin - iduronic acid and glucosamine, Keratan sulfate - galactose and n acetylglucosamine, Chondroitin sulfate - n acetyl galactosamine and glucuronic acid, Hyaluronic acid - n acetyl glucosamine and glucuronic acid)
Lipid enzyme involved in hydrolyzing the 1st and 3rd position of triglycerides into free fatty mono acyl glycerides:
Pancreatic lipase
Dimercaprol and antimycin A inhibits which complex at the Electron transport chain?
Complex III - Coq-b-c1 complex/oxidoreductase
What are the enzymes utilized in the Electron Transport chain? Enumerate
Complex 1 NADH, Complex 2 succinate dehydrogenase, Complex 3 oxidoreductase, Complex 4 cytochrome c oxidase
What is the intermediate of Pyruvate in gluconeogenesis?
Oxaloacetate - by action of enzyme pyruvate carboxylase (Fermentation intermediate - Ethanol, Citric acid cycle - acetyl coa, lactate - anaerobic glycolysis)
Rate limiting enzyme in glycolysis:
Phosphofructokinase 1
Rate limiting enzyme in gluconeogenesis:
Phosphoenolpyruvate carboxykinase
Rate limiting enzyme in TCA cycle:
Isocitrate dehydrogenase
What is the rate limiting enzyme in glycogenolysis?
Glycogen phosphorylase
Rate limiting enzyme in glycogenesis
Glycogen synthase
Rate limiting enzyme in Purine synthesis:
Glutamine PRPP amido transferase
Rate limiting enzyme in Pyrimidine syntehsis:
CPS II
Rate limiting enzyme in Urea cycle:
CPS I
Rate limiting enzyme in Fatty acid synthesis:
Acetyl-Coa Carboxylase
Rate limiting enzyme in Ketogenesis:
HMG CoA Synthetase
Rate limiting enzyme in Cholesterol synthesis
HMG CoA Reductase
Rate limiting enzyme in beta oxidation:
Carnitine acyltransferase I
Refers to the transfer of energy (thermal) between to bodies that are of different temperatures:
Heat (Exergonic or Endergonic)
Mode of heat loss if the skin temperature is greater than the environment:
Radiation, Conduction
Heat transfer by movement of fluid, air, or water:
Convection
High intensity shivering uses glucose in generating heat for short term use as opposed to that of low intensity shivering which uses what for long term use?
Lipids/fats
Achiral amino acid:
Glycine
Polar uncharged amino acids:
Serine, Threonine, Tyrosine, Cysteine, Asparagine, Glutamine
Amino acid that disrupt the alpha helix of proteins
Glycine (also major inhibitory NTA, Precursor for heme synthesis)
Amino acid that carries ammonia from tissues to liver:
Alanine
MSUD deficient enzyme
a-keto acid dehydrogenase
Amino acid with the largest side chain:
Tryptophan
Aromatic amino acids:
Tryptophan, Phenylalanine, Tyrosine
Imino acid:
Proline
Specialized protein that is required for the proper folding of proteins:
Chaperones
Amino acid structure not affected by denaturation:
Peptide bond (denaturation affects the secondary and tertiary structures)
Bonds that stabilize secondary structures:
Hydrogen bonding
Treatment for methemoblobinemia:
Methylene blue (chocolate cyanosis - meth, co2 poisoning - cherry red)
How many alleles are missing in Barts hemoglobinemia:
3
Chromosome location of alleles of Alpha and Beta chain of Hgb:
16 and 11
Collagens are rich in which amino acids:
Glycine and Proline
Collagen deficient in Epidermolysis Bullosa
Collagen type VII
The cartilage are made up of which type of collagen:
Type II
Collagen deficient in severe type of Ehlers Danlos Syndrome:
Collagen Type III
Mineral required for Cross linking of tropocollagen:
Copper
Defective collagen in syndrome characterized by bilateral hearing loss, ocular lesions, and gross hematuria:
Collagen Type IV (Alport syndrome, X linked)
Defective gene in Marfan syndrome:
Fibrillin (Autosomal dominant)
In emphysema, elastase destroys the alveolar walls. What protein is deficient in this case?
A1 antitrypsin
IN alpha thalassemia, how many alleles are missing for Hemoglobin H disease?
3 alleles
Immunoglobulin region that has both a constant and a variable region and is also the antigen binding site:
Fab region (Fc determines the isotype, only constant region, site of complement and macrophage binding)
Oligosaccharides have how many carbohydrate units?
3-10
Non reducing sugars:
Sucrose and Trehalose
Isomers that differ only in the configuration around 1 carbon atom:
Epimers
Mirror image of carbohydrates:
Enantiomers
Type of Glucose transporters in the Brain and Placenta:
Glut 3
Short chain FAs have _ number of carbon atoms while VLCFA have _ number of carbon atoms:
2-6, 22
Carnitine precursor:
Lysine
Essential FA that is the parent compound of arachidonic acid:
Linoleic acid
Essential FA that is needed for the development of fetal brain and retina:
a-Linolenic acid (where DHA is produced from)
Position of double bonds, hydroxyl, and hydrocarbon chains in cholesterol:
Double bonds: Carbon 5 and 6 B ring, Hydrocarbon chain on Carbon 17, Hydroxyl group on ring A with Hydroxyl group on Carbon 3
Primary bile acids:
Cholic acid, Chenodeoxycholic acid, (RE 7 a hydroxylase_
Where does glycolysis happen?
Cytosol
End product of glycolysis:
Pyruvate or lactate (2 molecules)
Enzyme in the conversion of Pyruvate to oxaloacetate:
Pyruvate carboxylase
Biochemical pathways that occur in the mitochondria and the cytosol:
Heme synthesis, Urea cycle, Gluconeogenesis
Lysosomal acid maltase deficiency:
Pompe disease
Glucose 6 phosphatase deficiency:
Von Gierke disease
Liver phosphorylase deficiency:
Hers disease
Most common disease producing enzyme abnormality in humans:
G6PD
Antibiotics associated with Serotonin syndrome:
Oxazolidinones
Notable side effect of Streptogramins (Quinupristin and Dalfopristin)
Arthralgia myalgia syndrome
Aminoglycosides are ineffective for anaerobes because they require _ fir uptake:
Oxygen
Enumerate 5 aminoglycosides:
GNATS (Gentamycin, Neomycin, Amikacin, Tobramycin, Streptomycin)
Aminoglycoside for hepatic encephalopathy:
Neomycin
Aminoglycoside for Visceral leishmaniasis:
Paromomycin
Aminoglycoside with the narrowest TI:
Amikacin
Vestibulotoxic aminoglycosides:
Gentamycin, Streptomycin, Tobramycin
DOC for Toxoplasmosis:
Sulfadiazine + Pyrimethamine (Plus Leucoverin)
DOC for PCP pneumonia and Nocardiosis:
Co-Trimoxazole
Antibacterial that inhibits DNA gyrase (Topoisomerase) that also exhibits a post antibiotic effect)
Quinolones
Most common type of glycolysis:
Aerobic (EMP pathway)
Pathway in the synthesis of acidic sugars:
Uronic acid pathway
Pathway in the synthesis of sorbitol (reduced sugar):
Polyol pathway
Final common pathway for the oxidation of lipids, carbohydrates, and proteins:
TCA cycle (Krebs cycle)
Pathway in the synthesis of 16 carbon Palmitate from acetyl CoA:
Lipogenesis
Pathway in the breakdown of palmitate to acetyl CoA:
Lipolysis (Beta oxidation)
Lactate in muscle is converted back to glucose in the liver:
Cori cycle
In the muscle, pyruvate is converted to alanine where it is transferred to the liver where the u
Glucose-Alanine pathway
Pathway that provides the energy required for gluconeogenesis:
Beta oxidation
The irreversible steps in glycolysis are catalyzed by the following enzymes:
Glucokinase, PFCK, Pyruvate kinase
