BIOCHEM Flashcards
Most abundant protein in the human body.
Collagen
Collagen general function
Organizes and strengthens extracellular matrix.
Collagen synthesis and structure
steps inside fibroblasts and where inside of them
- Synthesis (RER)
- Hydroxylation (ER)
- Glycosylation (Golgi)
- Exocytosis
Collagen synthesis and structure steps outside fibroblasts
- Proteolytic processing 6. Cross-linking
Collagen synthesis and structure Synthesis (RER)
Translation of collagen α chains
(preprocollagen)— usually Gly-X-Y polypeptide (X and Y are
proline, hydroxyproline, or hydroxylysine).
Collagen synthesis and structure
Hydroxylation (ER)
Hydroxylation of specific proline and lysine
residues (requires vitamin C).
Collagen synthesis and structure
Glycosylation of pro-α-chain lysine residues and formation of procollagen (triple helix of three collagen α chains).
Glycosylation (Golgi)
Collagen synthesis and structure
Exocytosis of procollagen into extracellular
space.
- Exocytosis
Collagen synthesis and structure
Cleavage of terminal regions of procollagen transforms it into insoluble tropocollagen.
- Proteolytic processing
Collagen synthesis and structure
Reinforcement of many staggered tropocollagen molecules by covalent lysine- hydroxylysine cross-linkage (by lysyl oxidase) to make col- lagen fibrils.
- Cross-linking
Chédiak-Higashi syndrome mech
is due to a microtubule polymerization defect resulting in ↓ phagocytosis
Molecular motors
Dynein =
Kinesin =
Molecular motors
Dynein = retrograde.
Kinesin = anterograde.
Drugs that act on microtubules:
- Mebendazole/thiabendazole (antihelminthic)
- Taxol (anti–breast cancer) 3. Griseofulvin (antifungal) 4. Vincristine/vinblastine (anti-cancer)
- Colchicine (anti-gout)
Vesicular trafficking proteins:
trans-Golgi → lysosomes, plasma membrane → endosomes (receptor-mediated endocytosis).
Clathrin
I-cell disease clinical findings
Characterized by coarse facial features, clouded corneas, restricted joint movement, and high plasma levels of lysosomal enzymes. Often fatal in childhood.
I-cell disease mech
failure of addition of mannose-6- phosphate to lysosome proteins, enzymes are secreted outside the cell instead of being targeted to the lysosome.
what cells are particularly rich in SER.
Liver hepatocytes and steroid hormone–producing cells of the adrenal cortex
Osteogenesis imperfecta 4 findings
- Multiple fractures
- Blue sclerae
- Hearing loss (abnormal middle ear bones)
- Dental imperfections due to lack of dentition
Osteogenesis imperfecta worst type and findings
Type II is fatal in utero or in the neonatal period.
5 Immunohistochemical stains and associated cell types
Vimentin - Connective tissue Desmin - Muscle Cytokeratin - Epithelial cells Glial fibrillary acid proteins (GFAP) - Neuroglia Neurofilaments - Neurons
Stretchy protein within lungs, large arteries, elastic ligaments.
Elastin