Term
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Definition
- respiratory tract (airway epithelial cells)
- female reproductive tract (fallopian tube epithelial cells)
- ependymal cells (brain ventricles |
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Term
| Microtubule based membrane structures |
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Definition
Motile cilia
- epithelia
Primary cilia
- epithelia
Flagella
- sperm |
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Term
| F-Actin based membrane structures |
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Definition
Microvilli
- epithelia
Stereocilia
- hair cell (epithelia)
Filopodia
- mygrating cells
Lamellapodia
- migrating cells |
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Term
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Definition
9+2 configureation
- apical (lumenal) membrane
- fluid and particle clearance |
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Term
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Definition
Axoneme:
- ring of NINE double MTs
- core to TWO singlet MTs
- (+) ends: ciliary tip
- (-) ends: basal body
Basal Body
- Ring of NINE triplet MTs
- ~ centrioles |
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Term
| Microtubule configureations |
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Definition
Singlet - 13 (general)
Doublet - 13+10 (cilia, flagella)
Triplet - 13+10+10 (basal bodies, centrioles) |
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Term
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Definition
- structural organization: directly parallels motile cilia
- tail length >> motile cilia length |
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Term
| Structural cross-linkages of motile cilia |
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Definition
- inner singlets: periodic bridges
- A-Tubule/inner singlet: radial spokes
- A-/B-Tubule: nexin |
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Term
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Definition
Cilia motor protein
- related to cytoplasmic dynein
- (-) end-directed motor proteins
- "cargo" is A tubule
- two rows on the A-Tubule: inner and outer dynein arm
- contraction: ciliary bending |
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Term
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Definition
- nexin cross-linking constrains sliding
- contraction 'bends' cilia
- bending is initiated at the base of the cilia |
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Term
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Definition
One per cell
- axonemal configuration: 9+0
- numerous cell types
- immotile
- sensory organelle |
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Term
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Definition
| Autosomal Dominant Polycystic Kidney Disease |
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Term
| Progression of Discovery from ADPKD |
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Definition
Discovery of ADPKD genes
- PKD1 and PKD2
Discovery of the ADPKD proteins
- polycystin-1 and polycystin-2
Characterization of the ADPKD proteins
- Polycystin-1: receptor-like integral membrane protein
- Polycystin-2: Ca2+ channel-like integral membrane protein |
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Term
| Hypothesis reguarding primary cilia |
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Definition
| serve as apical mechanosensors |
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Term
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Definition
left-right transposition of visceral organs
- due to a mutation in inversin |
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Term
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Definition
structural support of tissues
- anchoring cells to an underlying foundation
- basal lamin interposed between cells and ECM |
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Term
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Definition
Top Layer of the Foundation
- surrounds most groups of organized cells
- sheet-like meshwork of ECM proteins
- 60-120 nm thick
- secreted mainly from the cells it surrounds |
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Term
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Definition
| trimeric glycoprotin with rod-like and globular domains; main structural protein of the basal lamina |
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Term
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Definition
| cross-shaped trimer that links collagen IV, integrins and other adhesion sites |
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Term
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Definition
| large proteoglycan that cross-links many ECM proteins and cell-surface proteins |
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Term
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Definition
| rod-like protein that cross-links the other three as well as other ECM proteins |
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Term
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Definition
Dynamically-regulated linking proteins
- heterodimers of a- and B-subunits
- >24 distinct types
- differentially regulated
- bind distinct extracellular proteins
- binding is regulated by BOTH extracellular and intracellular clues |
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Term
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Definition
The 'foundational' protein in the ECM
- expressed in ALL multi-cellular animals
- >20 collagen genes
- constitute 25-30% of the TOTAL protein mass in the body |
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Term
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Definition
ENDOPLASMIC RETICULUM
- hydroxylation of procollagen
- glycosylation of procollagen
- formation of triple helix
- covalently linked by disulfide bonding
EXTRACELLULAR SPACE
- procollagen peptidases cleave globular ends
- rsulting collagen is relatively insoluble
- resulting collagen readily aggregates into striated fibers
- covalent cross-links enhance structural capacity |
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Term
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Definition
| forms elastic fibers within the ECM |
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Term
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Definition
extracellular proteins linked to negatively-charged polysaccharides
- can contain thousands of dissacharide dimers per protein
- many GAG chains are attached to a 'core' protein
- forms a 'bottlebrush' appearance
- can be > 1,000,000 Da in mass |
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Term
| Glycosaminoglycans (GAGs) |
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Definition
Long polymers of repeating polysaccharides
- negatively charged repeating disaccharides
- one reside is an amino-sugar
- attached to proteoglycans in the Golgi
- High ratio of polysaccharide to protein |
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Term
| Physical characteristics of proteoglycan-GAG aggregates |
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Definition
- highly hydrophilic
- negative charges attract sodium ions
- promotes osmotic swelling
- hydrated proteoglycans form a gel
- weaves of collagen and proteoglycans form a tough, resilient matrix |
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Term
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Definition
collagen and integrin binding sites
- connecting ECM with the intracellular cytoskeleton (Collagen-Fibronectin-Integrin-Actin) |
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