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20 Cards in this Set

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tumor suppressors (Rb and p53) normally inhibit which phase progression?
G1 >> S
(cell type)

neurons, skeletal and cardiac muscle, RBCs
Permanent

remain in G0
regenerate from stem cells
(cell type)

hepatocytes, lymphocytes
Stable/quiescent

Enter G1 from G0 when stimulated
(cell type)

bone marrow, gut epithelium, skin, hair follicles
Labile

Never go to G0
divide rapidly with a short G1 (continuous)
site of synthesis of secretory (exported) proteins and of N-linked oligosaccharide addition to many proteins
RER

goblet cells of GI and Ab-secreting plasma cells
site of steroid synthesis and detoxification of drugs and poisons
SER

liver hepatocytes and in adrenal cortex
HYPERphosphorylation of Rb leads to...
retinoblastoma
HYPOphosphorylation of Rb leads to...
suppression of expression. (no movement to S phase)
coarse facial features, clouded corneas, restricted joint movement and high plasma levels of lysosomal enzymes
failure of addition of mannose-6-phosphate to lysosome proteins (enzymes are secreted outside the cell instead of being targeted to the lysosome); fatal in childhood

I-cell disease (inclusion cell disease)
distribution center of proteins and lipids from ER to the plasma membrane, lysosomes, and secretory vesicles
Golgi apparatus

COPI -- retrograde
COPII -- anterograde
Clathrin -- trans/receptor-mediated endocytosis
recurrent pyogenic infections, partial albinism, peripheral neuropathy
microtubule polymerization defect >>> decreased phagocytosis

Chediak-Higashi syndrome
ATPase that links peripheral 9 doublets and causes bending of cilium by differential sliding of doublets
Axonemal dyenin

defect = Kartagener's syndrome
(type of collagen)

bone, skin, tendon, dentin, fascia, cornea, late wound repair
Type I (90%)
(type of collagen)

cartilage (incl hyaline), vitreous body, nucleus pulposus
Type II
(type of collagen)

skin, blood vessels, uterus, fetal tissue, granulation tissue
Type III (Reticulin)
(type of collagen)

basement membrane or basal lamina
Type IV
inhibited hydroxylation of proline and lysine residues in ER
Scurvy
defective glycosylation of pro alpha-chain of collagen
Osteogenesis imperfecta
defective peptide cleavage at terminal regions of procollagen >> no tropocollagen and/or crosslinked fibrils
Ehlers-Danlos

Type III collagen
multiple fractures with minimal trauma, blue sclerae, hearing loss, dental imperfections
brittle bone disease

AD with abnormal Type I collagen

Type II is fatal in utero/neonatal period