Review Topnatch 2 Flashcards
Maximal velocity of an enzyme catalyzed reaction
V max
Substrate concentration at which the reaction velocity is equal to 1/2 Vmax
Michaelis constant
Rate independent of substrate concentration
Zero
Rate proportional to substrate concentration
First
Emzyme that catalyze the transfer of the amino groups of amino acids to glutamate
Amino transferase
Oxidatively deaminates glutamate to liberate free ammonia
Glutamate dehydrogenase
Deaminates glutamine to glutamate in the kidneys and intestines
Glutaminase
Compounds in which organisms excrete excess nitrogen
Ammonia
Uric acid
Urea
Amino acids which carry nitrogen fromperiphery to the liver
Glutamate
Glutamine
Alanine
Function of urea cycle
Produces urea from free ammonia
Location of urea cycle
Mitochondria and cytolpasm. Liver only
Reactnats of ureacycle
Ammonia
Aspartate
Carbon dioxide
End productof urea cycle
Urea
Rate limiting step of urea
Carbomyl phosphate synthase 1
3 compounds are the immediate donors of the atoms of urea
Free ammonia
Aspartate
CO2
Heme synthesis location
Mitochondria and cytoplasm, all tissues
Heme reactants
Glycine
Succynl Coa
End product of Heme
e-aminolevulinic acid
Rate limiting step of heme
Glycine+succynl CoA -> ALA
via ALA synthase
Enzyme for the conversion of heme to biliverdin
Heme oxygenase
Enzyme for the converion of biliverdin to bilirubin
Bilverdin reductase
Amino acids degradation and synthesis
Pyruvate
Alpha-ketoglutarate
Oxaloacetate
Alanine
Glutamate
Aspartate
Ketogenic amino acids
Lysine
Leucine
Glucogenic amino acids
Valine
Amino acid of ketogenic and glucogenic
Tyrosine
Pigment derived from porphin chelates with iron, magnesium, zinc, nickel, copper or cobalt
Porphyrin
Hyaluronic acid, dermatin sulfate, heparin, keratansulfate
GAGs
Found in extracellular matrix of connective tissues
Proteoglycans
Component of most connective tissues in the body
Collagen
Found in the ground substance GAG+ protein Core
Proteoglycans
Found in connective tissues which has elastic properties
Elastin
Intermediate filament found in epithelial cells
Keratin
Defense of the body aainst foreign bodies harmful and nonharmful
Immunoglobulins
Thick filaments found in the muscles, involved in muscle contraction
Myosin
Thin filaments found in skeletal muscle.
Could be fibrous or globular
Actin
Protein folding disorder
Prion disease and alzheimers
Gluatmate is replaced by valine at position 6 of the B-globin chain. Causing hemoglobin that polymerizes inside the RBC.
Sickle-cell disease
Synhtesis of alpha chains is decreased or absent
Alpha thalasemia
Spectrin deficiency causes spherical RBC that are rapidly culled by the spleen
Hereditary sphercytosis
Blue sclera, multiple fractures, conductive hearing loss
Osteogenesis imperfecta
Berry aneurysms, hyperextensible skin, hypermobile joints, tnedency to bleed.
Ehlers-pauls syndrome
Loose teeth, sore spongy gums, poor wound healing, petechiae on skin and mucous membranes
Scurvy
Hereditary nephritis with sensorineural hearing loss
Alport syndrome
Aortic dilatation, dolichistenimelia, acromegaly
Marfan syndrome
Panacina emphysema and liver failure
a-1 antitrypsin deficiency
Hepatolenticular degeneration from accumulation of copper in tissues with low levels of ceruplasmin
Wilsons disease
Musty body odor, mental retardation, fair skin, eczema
Phenylketonurin
Urine turns black upon standing, associated with debilating arthralgias
Alkaptonuria
Decreased pigmentation that increase the risk for skin cancer
Albinsim
Atherosclerosis, lens subluxation, stroke, MI, osteoporosis, tall stature
Homocystinuria
Staghorn calculi due to inherited defect of renal tubular amino acid transporter
Cystinuria
Mental retardation from blocked degradation of branched amino acids
Maple syrup urine disease
Photosensitivity, chronic inflammation to overt blistering and shearing in exposed areas of the skin due to defects in heme synthesis
Porphyrias
Protein deprivation that is relatively greater than the reduction in total calories
Kwashiorkor
Caloric deprivation is relatively greater than the reduction in protein
Marasmus