Kaplan course and Uworld Flashcards
main form of therapy for urea cycle disorders
protein restriction
urea cycle location
half cytosol, half mitochondria
diptheria toxin interferes with what metabolic process
Elongation during protein synthesis
drugs that target 50S ribosome subunit
MLS group
macrolides (good for penicillin allergy)
lincosamide: clindamycin (anaerobic bacteria, MRSA, malaria, toxoplasmosis
streptogramins: for VRSA, VRE, MRSA
anything ending with mycin came from streptomycins
NADPH is needed for what?
cholesterol and fatty acid synthesis
drugs that target the 30S ribosome subunit
aminoglycosides
organisms that inhibit eukaryotic translation
diphteria (toxin protein ADP-ribosylates eEF-2 which contains a modified His called a diphtamide residue
Pseudomonas: toxin protein ADP-ribosylates eEF-2, activity similar to diptheria toxin
what things does dihydrobiopterin reductase do?
phenylalanine to tyrosine to DOPA
tryptophan to 5HT to serotonin
lesch nyhan. Increased activity of what?
PRPP to make up for HGPRT trouble
steps for most protein synthesis
translation begins in cytoplasm signal sequence attaches to ER signal peptidase removes signal sequence translation continues in RER glycosylation in ER (continues in golgi) proper folding in ER
I cell disease
lysosomal storage disease
looks like tay-sachs/ etc., but dx: measuring lysosomal hydrolases in the blood/ urine
trouble with phosphorylation of mannose by phosphotransferase signals to lysosome
severe retardation, rapid progression, death by 5-8 y/o
coarse facial features, craniofacial abnormalities, severe skeletal abnormalities.
hepatomegaly, cardiomegaly, umbilical hernias, recurrent URIs
** STRIKING GINGIVAL HYPERPLASIA
collagen types
I- bone, skin, tendons
II- cartilage, vitreous humor
III- blood vessels, granulation tissue
IV- basement membranes
SIADH volemia finding
euvolemic hyponatremia due to natriuretic peptids
locus vs allelic heterogeneity
locus heterogeneity– different genes, same phenotype
allelic heterogeneity- different phenotypes, same gene
cavernous sinus thrombosis affects what nerves?
CNs III, IV, and VI affected
what makes sclera blue?
choroidal veins are visible
giardiasis can lead to
lactose intolerance secondary to infection
copper diseases present with what?
iron problems due to copper-dependent processes
e.g.: loss of Fe2+ efflux from brain
Menkes disease
X-linked
inability to absorb copper from GI tract
cherubic face, sagging jowls, scant or no eyebrows, kinky depigmented hair, anemia, osteoporosis, cerebral degeneration
what do response elements do?
respond to transcription factors
e.g. glucocorticoid (GRE), cAMP (CRE), etc.
PPARs
regulate multiple aspects of lipid metabolism
reduce serum Triglycerides
activated by fibrates and thiazolidinediones
increase peroxisomal fat metabolism, and increase adipcyte differentiation
nitrates MOA for angina
venodilation –> reduced preload –> decreased afterload, decreased myocardial oxygen demand
can’t really dilate coronary arteries any more than they already are
how hyperglycemia is related to lipids
–> insulin release –> tells adipocytes to make more fat