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What is the effect of enzymes?
increase the rate of reaction by decreasing the activation energy
What external factors that can affect the activity of enzymes?
optimum temperature and pH
How does adenosine deaminase (ADA) prevent lymphocytoxity?
Adenosine deaminase (ADA) prevents lymphocytoxity by converting deoxyadenosine to deoxyinosine (deaminates/NH2 adenosine), → reduces deoxyadenosine levels that can inhibit T-cell activation and proliferation, inhibiting lymphotoxicity
How is SCID (severe combined immunodeficiency) treated?
enzyme replacement therapy w/ Elapegademase
What is Elapegademase?
PEGylated recombinant adenosine deaminase (rADA) enzyme
What is the structure of Elapegademase based on?
bovine amino acid sequence that is covalently conjugated, with a linker, to monomethoxypolyethylene glycol
What is the disease that glucocerebrosidase deficiency leads to?
Gaucher’s disease
What is Gaucher’s disease?
accumulation of certain fatty substances glucocerebrosides
How is Gaucher’s disease/glucocerebrosidase deficiency treated?
Imiglucerase (Cerezym)
Velagluceraase alfa (Vpriv)
Imiglucerase (Cerezym) has a shorter or longer half life than Velagluceraase alfa (Vpriv)?
Imiglucerase (Cerezym) has a shorter half life (3.6 - 10.4 min) than Velagluceraase alfa (Vpriv) (11-12 min)
A deficiency in galsulfase leads to?
accumulation of chondroitin-4 sulfate and dermatan
What is a deficiency in galsulfase treated with?
naglazyme (recombinant galsulfase glycoprotein)
(Gals? Na, glaze)
What is the function of B-D-galactosidase (aka Lactase)?
breaks down lactose (two rings) into galactose and glucose (each one ring)
What is the oncolytic enzyme used for the treatment of acute lymphoblastic leukemia?
L-asparaginase (limits tumor growth)
What are the three different biologics of L-Asparginase?
Asparaginase (Erwinia [Recombinant]) (Rylaze)
Pegaspargase (Oncaspar)
Calaspargase Pegol (Asparlas)
(Rile up On my AS(p))
What is the biologic pegaspargase (Oncaspar) conjugated with?
monomethoxypolyethylene
(M-M-P-E)
Calaspargase Pegol (Asparlas) has a structure of L-asparaginase and monomethoxypolyethylene glycol (mPEG) linked via…?
a succinimidyl carbonate linker (mPREG… suck-on-my-dicc)
What are the two biologics that are uricases (catalyze urate oxidase)?
Rasburicase (Elitek)
Pegloticase (Krystexxa)
What is pegloticase (Krystexxa) used for?
treatment of gout
What is rasburicase (Elitek) used for?
treat elevated levels of uric acid arising from leukemia/chemo
What is rhDNase I used for?
treatment of cystic fibrosis
What biologic is a form of rhDNase I?
Dornase Alfa (Pulmozyme)
What is the typical concentration of dornase alfa (pulmozyme)?
2500 U/2.5 mL