Random Biochem Facts Flashcards
What does golgi modify (cell trafficking)
N-oligosaccharides on asparagine; adds O-oligosaccharides on serine and threonine; adds mannose-6-phospate to proteins for trafficking to lysosomes
Endosomes
sorting centers for material from outside of the cell or from the golgi, sending it to lysosomes for destruction or back to the membrane/Golgi for further use
I-cell Disease
inherited lysosomal storage disorder –> failure of addition of mannose-6 phosphate to lysosome proteins (enzymes are secreted outside the cell instead of being targeted to the lysosome)
I-cell Disease clinical features
coarse facial features, clouded corneas, restricted joint movement, and high plasma lysosomal enzymes
Immuno stain for (structural) intermediate filament: Vimentin
connective tissues
Immuno stain for (structural) intermediate filament: Desmin
muscle
Immuno stain for (structural) intermediate filament: cytokeratin
epithelial cells
Immuno stain for (structural) intermediate filament: GFAP
neuroglia
Immuno stain for (structural) intermediate filament: neurofilaments
neurons
ouabain
inhibits binding to K+ site (fucks up Na+/K+ ATPase)
type 1 collagen
most common; bone, skin, tendon, dentin, fascia, cornea, LATE WOUND REPAIR
type 2 collagen
cartilage (also hyaline), vitreous body, nucleus pulposus
type 3 collagen
reticulin - skin, blood, vessels, uterus, fetal tissue, granulation tissue EARLY WOUND REPAIR
type 4 collagen
basement membrane or basal lamina
Synthesis of collagen (RER inside fibroblast)
translation of collagen alpha chains (preprocollagen) –> Gly-X-Y
Hydroxylation of collagen (ER, inside fibroblast)
hydroxylation of specific proline and lysine residues (requires Vit C) (SCURVY)
Glycosylation (ER, inside fibroblast)
glycosylation of pro-alpha chain hydroxylysine residues and formation of procollagen via hydrogen and disulfide bonds (triple helix of 3 collagen alpha chains) {PROBLEM W/ OSTEOGENSIS IMPERFECTA)
Proteolytic processing (outside of fibroblast)
cleavage of disulfide-rich terminal regions of procollagen, transforming it into insoluble tropocollagen
cross-linking (outside of fibroblast)
reinforcement of many staggered tropocollagen molecules by covalent lysine-hydroxylysine (Cu2+ containing lysyl oxidase) to make collagen fibrils (EHLERS-DANLOS)
southwestern blotting
identifieds DNA-binding proteins (TF) using labeled oligonucleotide probes
gene expression modifications: cre-lox system
inducibly manipulate genes at specific developmental points using an antibiotic-controlled promotor ( to study a gene whose deletion causes embryonic death)
gene expression modifications: RNA interference (RNAi)
dsRNA is synthesized that is complementary to the mRNA sequence of interest; when transfected into human cells, dsRNA separates and promotes degradation of target mRNA, knocking down gene expression
heteroplasmy
presence of both normal and mutated mtDNA, resulting in variable expression in mitochondrial inherited disease
uinparental disomy
offspring receives 2 copies of chromosome from 1 parent and no copies from the other parent