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32 Cards in this Set
- Front
- Back
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What are some essential AA?
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LEU
Isoleucine LYS PHE Threonine TRP Methionine Histadine |
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What are some serine proteases?
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Trypsin. Chymootrypsin
Plasmin Thrombin plasminogen cathepsin G elastase |
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Aromatic AA.
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PHE
TYR TRP HIS |
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Which lysosomal storage disease is this?
- accumulation of glucocerebroside |
Gaucher's disease (AR)
- aseptic necrosis of femur - beta-glucocerebrosidase deficiency - hepatosplenomegaly |
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Which lysosomal storage disease is this?
- accumulation of sphingomyelin |
Niemann-Pick (AR)
- progressive peripheral neurodegeneration - cherry red spot - anemia - sphingomyelinase deficiency |
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Which lysosomal storage disease is this?
- accumulation of GM2 ganaglioside |
Tay-Sachs disease (AR)
- progressive neurodegeneration - developmental delay - cherry red spot - lysosomes with onion skin - hexosaminidase A deficiency |
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Which lysosomal storage disease is this?
- accumulation of galactocerebroside |
Krabbe's disease (AR)
- peripheral neuropathy - developmental delay - optic atrophy - galactocerebrosidase deficiency |
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Which lysosomal storage disease is this?
- accumulation of ceramide trihexose |
Fabry's disease (X-linked)
- peripheral neuropathy of hands and feet - angiokeratoma - CV disease - renal disease - alpha-galactosidase A deficiency |
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Which lysosomal storage disease is this?
- gargoylism (coarse facial features) - developmental delay - airway obstruction - corneal clouding - umbilical hernia |
Hurler's syndrome
- alpha-L-iduronidase deficiency - accumulation of heparan sulfate and dermatan sulfate |
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Which lysosomal storage disease is this?
- gargoylism (coarse facial features) - developmental delay - airway obstruction - aggressive behavior |
Hunter's syndrome
- iduronate sulfatase deficiency - accumulation of heparan sulfate and dermatan sulfate |
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Enzymes that need thiamine cofactor.
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- PDH
- alpha-ketoglutarate dehydrogenase (TCA) - transketolase (HMP shunt) |
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Enzymes that need B6 cofactor.
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- aminotransferase (protein catabolism): caused by isoniazid therapy.
- aminolevulinate synthase (heme synthesis): sidroblastic anemia, convulsions |
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Enzymes that need B12 cofactor.
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- homocysteine methyltransferase
- methyl-malonyl-coA mutase |
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Enzymes that need vitamin C cofactor.
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- prolyl-, lysyl-hydroxylase: collagen synthesis
- dopamin beta-hydroxylase |
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Which glycogen storage disease is this?
- cardiomegaly - deficient lysosomal alpha-1,4-glucosidase (acid maltase) |
Pompe's disease
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Which glycogen storage disease is this?
- severe fasting hypoglycemia - increased glycogen in liver - increased lactate - hepatomegaly - deficient glucose-6-phosphatase deficiency |
Von Gierke's disease
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Which glycogen storage disease is this?
- severe fasting hypoglycemia - increased glycogen in liver - normal lactate - hepatomegaly - deficient debranching enzyme alpha-1,6-glucosidase |
Cori's disease
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Which glycogen storage disease is this?
- increased glycogen in muscle - painful muscle cramps - myoglobinuria with streneous exercise - deficient glycogen phosphorylase |
McArdle's disease
- treat with sucrose |
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Which glycogen storage disease is this?
- cirrhosis - myopathy - heart failure - deficient branching enzyme |
Anderson's disease
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What are some hormones that proteins with zinc finger domain bind to and alter gene transcription?
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- thyroid
- steroid hormones - Vitamin A, D |
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T/F: Glucokinase is specific for liver and is nor in muscle.
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T.
Hexokinase exist in virtually all cells. |
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What cofactor is required for carbocylation reaction?
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biotin
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Is there fatty acid synthesis in the muscle?
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No
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T/F: Fatty acid synthesis occurs in the cytosol whereas fatty acid oxidation takes place in the mitochondrial matrix.
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T.
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What type of antibody is most abundant in body secretions?
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IgA
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What AA does trypsin cleave?
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C terminal of arginine and lysine
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What AA does chymotrypsin cleave?
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C terminal of aromatic AA (PHE, TYR, TRP)
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What AA does elastase cleave?
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C terminal of alanine, glycine, serine
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What is Leigh disease?
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also known as subacute necrotizing encephalomyelopathy
- mitochondrial DNA defect: cytochrome oxidase defect - affect muscel and brain |
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Name this vitamin cofactor:
needed in carboxylation reactions - pyruvate -> oxaloacetate - aceto-coA -> malonyl coA - propionyl-coA -> methylmalonyl coA |
Biotin
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Name this vitamin cofactor:
needed in decarboxylation - alpha-ketoglutarate -> succinyl-coA - pyruvate -> acetyl-coA needed in branched chain AA dehydrogenase cofactor for transketolase in the HMP shunt - ribose-5-phosphate -> F-6-P |
thiamine
- deficiency cause dry and wet beriberi |
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Name this vitamin cofactor:
- used in transamination (ALT, AST) - decarboxylation - glycogen phosphorylase - heme synthesis - required for synthesis of niacin from tryptophan |
B6
- deficiency can be induced by INH and oral contraceptives |
