Notes in d. Extracellular Matrix and Collagen Disorders

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Status	Last Update	Fields
Published	07/28/2024	The typical collagen structure is typically {{c1::triple alpha helix}}
Published	07/28/2024	Collagens helices are enriched in {{c1::glycine and proline}} residues in a {{c1::Gly-X-Y}} sequence
Published	07/28/2024	{{c1::Fibril-forming/interstitial}} collagens include numbers {{c2::I, II, III, V, XI}}
Published	07/28/2024	{{c1::FACIT}} collagens include numbers {{c2::IX, XII, XIV}}
Published	07/28/2024	{{c1::Network Forming}} collagens are Type {{c2::IV}} Collagen
Published	07/28/2024	{{c1::Anchoring filament}} collagens in the basement membrane include Type {{c2::VII}}
Published	07/28/2024	Fibril Forming Collagen {{c1::I}} is found {{c2::ubiquitously in hard and soft tissue, major protein of bone and skin}}
Published	07/28/2024	Fibril Forming Collagen {{c1::II}} is found in the {{c2::cartilage, vitreous humor in the eye, intervertebral disks}}
Published	07/28/2024	Fibril Forming Collagen {{c1::III}} is found in the {{c2::soft tissues, skin, muscles, blood vessels}}
Published	07/28/2024	Fibril Forming Collagen {{c1::V}} is found in the {{c2::fetal tissues, placenta, interstitial tissues and skin}}
Published	07/28/2024	The single collagen alpha helix is {{c1::left}} handed and three of these alpha chains come together to form a {{c1::right}} handed triple helix …
Published	07/28/2024	The triple collagen alpha helix is {{c1::right}} handed
Published	07/28/2024	The collagen alpha helix has {{c1::3}} amino acids per turn
Published	07/28/2024	The first amino acid always seen in a turn of a collagen alpha helix is {{c1::glycine}}
Published	07/28/2024	The second amino acid seen in a turn of a collagen alpha helix is 30% of the time a {{c1::proline}}
Published	07/28/2024	The third amino acid of a collagen alpha helix is either a {{c1::hydroxyproline}} or a {{c2::hydroxylysine}}
Published	07/28/2024	The 3 alpha helices observed in the collagen structure are (parallel/antiparallel?) {{c1::parallel}}
Published	07/28/2024	The collagen alpha helix needs to be synthesized in the {{c1::ER}}
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Published	07/28/2024	The nontriple helix ends of mature collagen are referred to as {{c1::telopeptide}} regions
Published	07/28/2024	Tropocollagen molecules cross-link and form {{c1::fibrils}} via reactive {{c1::aldehydes}} formed by lysyl oxidase that form covalent bonds with …
Published	07/28/2024	The enzyme {{c1::lysyl oxidase}} is responsible for forming covalent bonds between lysine and hydroxylysines which manifest into tropocollagen cr…
Published	07/28/2024	The 2 enzymes required for the hydroxylation of selected prolines and lysines on collagen alpha helixes are?
Published	07/28/2024	Prolyl hydroxylase and lysyl hydroxyslase requires a cofactor of {{c1::vitamin C}}
Published	07/28/2024	Procollagen terminis are cleaved via what 2 enzymes?
Published	07/28/2024	FACIT stands for {{c1::Fibrin associated collagens with interrupted triple helices}}
Published	07/28/2024	The role of FACIT collagens is?
Published	07/28/2024	Type IV collagen is found in the {{c1::basal lamina}}
Published	07/28/2024	{{c1::Type IV}} collagens form network of multilayered {{c1::sheets}} via a flexible structure with interrupted triple helices in the {{c1::basal lami…
Published	07/28/2024	{{c1::Alport syndrome}} is a condition characterized by blood and protein in the urine, kidney failure, and hearing loss due to a defect in {{c2::Type…
Published	07/28/2024	{{c2::Goodpasture syndrome}} is an autoimmune disorder caused by antibodies to {{c1::type IV}} collagen that affects the basal lamina of {{c1::lungs a…
Published	07/28/2024	{{c2::Type VII}} collagen are {{c1::anchoring}} collagen filaments
Published	07/28/2024	Type VII collagen is found in {{c1::basement membranes}}
Published	07/28/2024	{{c2::Dystrophic epidermolysis bullosa}} is a skin blistering disease caused by loss of anchoring fibril {{c1::type VII}} collagen. This is different …
Published	07/28/2024	{{c2::Elastin}} associate with collagen fibers to limit {{c1::stretching}} and prevent {{c1::tearing}}. It is found in {{c1::skin, ligaments, and vess…
Published	07/28/2024	Elastin filaments are highly (hydrophilic/phobic?) {{c1::hydrophobic}}
Published	07/28/2024	Elastin filaments are rich in {{c1::proline and glycine}} amino acid residues
Published	07/28/2024	Elastin is highly cross linked at {{c1::lysine}} residues
Published	07/28/2024	Elastin is only made by {{c1::fetal and juvenile fibroblasts}}
Published	07/28/2024	{{c1::Elastin}} is the dominant ECM protein in arteries
Published	07/28/2024	Elastin fibers are always contained by a {{c1::microfibril sheath}} composed largely of {{c2::fibrillin}} which is why mutations in {{c2::fi…
Published	07/28/2024	{{c1::Marfan syndrome}} is caused by a mutation in {{c2::fibrillin-1}}
Published	07/28/2024	{{c1::Williams syndrome}} is a haploinsufficiency for {{c2::elastin}}
Published	07/28/2024	Adhesive proteins are generally characterized by domains for binding to {{c1::cell surface receptors}} via the {{c1::R-G-D}} adhesive recognition…
Published	07/28/2024	Cell surface receptors recognize the {{c1::RGD}} sequence on adhesive proteins in their cell binding domains
Published	07/28/2024	Fibronectin and laminin are example of {{c1::adhesive}} proteins
Published	07/28/2024	The type {{c1::III}} modules of fibronectin contains the RGD sequence for binding to cell surface receptors
Published	07/28/2024	The following is a typical structure for?
Published	07/28/2024	{{c1::Laminin}} is a family of large glycoproteins, adhesive proteins expressed early in development, and are a major component of {{c1::basal lamina}…
Published	07/28/2024	Describe the structure of laminin
Published	07/28/2024	The {{c1::basement membrane}} includes the basal lamina and underlying collagens (typically type VII)
Published	07/28/2024	Tissues that withstand tensional forces have high levels of {{c1::fibrillar collagens}}
Published	07/28/2024	Tissues that withstand compressive forces have high levels of {{c1::proteoglycans}}
Published	07/28/2024	How do proteoglycans help tissue withstand compressive force?
Published	07/28/2024	{{c1::Glycosaminoglycans}} are proteoglycans with long (70-200) unbranched polysaccharide chains of {{c1::repeated dissaccharides}}
Published	07/28/2024	GAG disaccharide units contain
Published	07/28/2024	Two example of amino sugars observed in GAGs are?
Published	07/28/2024	Two Example of acidic sugars observed in GAGs are?
Published	07/28/2024	GAGs are highly {{c1::negatively}} charged and highly {{c2::hydrophilic::hydrophiliic/phobic}}
Published	07/28/2024	{{c1::Hyaluronan}} is a GAG characterized by simple, long, thousands of nonsulfated sugars not linked to a protein
Published	07/28/2024	Where are hyaluronan's synthesized?
Published	07/28/2024	Where are proteoglycans made?
Published	07/28/2024	GAGs are thought to be added to proteoglycans in?
Published	07/28/2024	{{c1::Heparan sulfate}} is a common GAG found in the {{c2::lung}}, {{c1::arteries}}, {{c2::cell surfaces}}, {{c1::basal lamina}}, and {{c2::central ne…
Published	07/28/2024	{{c1::Aggrecan}} is a large {{c2::chondroitin sulfate}} proteoglycan with 3 globular domains, 1 interglobular domain, and {{c1::2}} GAG attaching doma…
Published	07/28/2024	Aggrecan molecules assemble into aggregates with {{c1::hyaluronan}} as a core of the aggregate
Published	07/28/2024	Biglycan is a proteoglycan that bind {{c1::TGFBeta}} and {{c2::collagen}}
Published	07/28/2024	{{c2::Decorin}} is a proteoglycan that binds {{c1::collagen}} and {{c2::TGFBeta}} which is a highly pleiotropic cytokine that plays an impor…
Published	07/28/2024	Perlecan is a proteoglycan that is prominant in the {{c1::basal lamina}}
Published	07/28/2024	Syndecan-1 is a proteoglycan that binds {{c1::Fibroblast Growth Factors}} which play a role in cell proliferation and differentiation
Published	07/28/2024	{{c1::Integrins}} are the principal receptors on animal cells for binding most extracellular matrix proteins
Published	07/28/2024	Describe the basic structure of integrins:
Published	07/28/2024	GAGS and proteoglycans are degraded via {{c1::endocytosis}} where they are taken to the {{c1::lysosome}} for degradation by mucopolysaccharidoses…
Published	07/28/2024	Collagen and elastin are degraded by {{c1::extracellular proteases}} like Matrix metalloproteinases (MMPs) which contain a {{c1::zinc}} ion and c…
Published	07/28/2024	Mucopolysaccahardisoses are lysosome storage disease caused by?
Published	07/28/2024	Hunter's, Hurler's, Sanfilippo A/B/C/D disease are example of {{c2::mucopolysaccharidose diseases}} which are diseases where loss of {{c1::lysoso…
Published	07/28/2024	{{c1::Hunter}} disease is a deficiency in irudonate sulfatase
Published	07/28/2024	{{c1::Hurler}} disease is an deficiency in iduronidase. Iduronic acid is an acidic sugar in GAGs and thus the disease reduces the ability to break dow…
Published	07/28/2024	{{c1::Matrix metalloproteinases}} are {{c2::zinc}} containing extracellular endopeptidases that selectively degrade parts of the ECM.
Published	07/28/2024	Matrix MetalloProteinases are inhibited by {{c1::tissue inhibitors of MMPs (TIMPs)}}
Published	07/28/2024	Extracellular matrix is produced by {{c1::fibroblasts, osteoblasts, and chondrocytes}} and functions as a {{c1:: stablizing scaffold}} for physical st…
Published	07/28/2024	Fibronectin is a {{c1::dimer}} linked by disulfide bonds found in most extracellular matrices as fibronectin {{c1::fibrils}} and in plasma as {{c1::so…
Published	07/28/2024	Proteoglycans are core proteins made in the {{c1::ER (ribosomes threaded to lumen of ER)}} with {{c1::polysaccharide}} chains assembled on core protei…
Published	07/28/2024	Proteoglycans restrict {{c1::movement}} of cells, bind {{c1::ligands (growth factors, etc)}} to concentrate and present to receptors or protect from {…
Published	07/28/2024	Matrix metalloproteinases are extracellular {{c1::endopeptidases}} that selectively {{c1::degrade}} many components of the ECM. They are thought to pl…
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