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BioChem Connective Tissue and Bone
Terms in this set (67)
Where is Preprocollagen synthesized?
Lumen of RER
What form additional cross helix H bonding in collagen?
What form additional cross links between collagen molecules?
What clips the end and tail of triple helix in order to form the collagen molecule?
Collagen is made by ___
Fibrillation structure is reinforced by the formation of covalent cross links between collagen molecules catalyzed do by the enzyme ____
Hydroxylysine is cross-linked to form ____.
What is the most common form of collagen?
Type I collagen
Where is Type I collagen found?
Where is type II collagen found?
What is the genetic disease that affects collagen containing tissues because of point mutations that destabilize or alter collagens important triple helix structure?
What does a person suffer from if they have a deficiency of ascorbic acid?
Why is it important to have sufficient Vitamin C?
The body can't form normal collagen essential for osteoid/bone formation and other connective tissue if it's Vitamin C deficient
What are the 3 dominant types of fibers in the connective tissue?
Reticular Fibers are round in the liver, muscle, bone marrow, and lymph system where it offers a __________ __________.
Reticular fiber is composed of _________ ____ collagen forming an ordered "reticulum" mesh work instead of a linear structure
Where are reticular fibers usually found?
Elastic fibers are formed from smaller micro fibrils, made of __ and ___ with cross linking peptides
With less ____, cartilage is prone to degradation
What is the primary cause of osteoarthritis?
Leads to decrease in water in cartilage and decrease in proteoglycans
The inorganic composition of bone is formed from ___
Organic composition of bone matrix is mainly compose of what?
Type I collagen (osteoid) and ground substance
What produce bone including the inorganic mineral matrix and the organic osteoid, ground substance and glycoproteins.
Modified forms of monocytes/macrophage cells that namely resorb and or remodel mineral matrix.
Former osteoblasts that have been trapped in organic bone matrix and link together via specialized canalicular extensions continual gap junctions which allow the interchange of nutrients and waste products
Osteoblasts growth and production are affected by what?
Fibroblast growth factor (FGF)
Platelet derived GF
Bone morphogenic proteins
What is the bone ground substance composed of?
GAG chondroitin sulfate
What promotes growth and differentiation of osteoclasts to help maintain the balance between bone formation and resorption and calcium levels?
Macrophage colony stimulating factor (M-CSF)
Newly formed osteoblasts secrete a cytokine known as ____.
Macrophage colony stimulating factor (M-CSF)
Rare disease in which repair process of fibrous tissues is changed to bone repair, resulting in heterotrophic ossification.
Fibrodysplasia ossification progressiva
Osteoclasts are positively regulated by what?
Receptor activator of nuclear factor kappa B ligand (RANKL)
Differentiation of osteoclasts is inhibited by ____ by inhibiting RNAK/NF-kB binding.
What activated intracellular nuclear factor kappa B?
What is a member of the tumor necrosis factor (TNF) receptor family and is an important transcriptional activator?
Nuclear factor kappa B (NF-kB)
Osteoclasts produce ____, which degrade the collagen matrix via selective cleavage of collagen and other proteins
Laboratory results for Rickets/Osteomalacia
hypocalcemia in serum/urine, increase alkaline phosplatase, increase PTH levels
This results when osteoblasts can't make hydroxyapatite or insufficient Ca2+ or Pi
What is the treatment for osteomalacia?
Involves correction of problem and replacement of calcium and vitamin D
Condition in which bone mineral content is significantly lowered, altering and weakening bone matrix with the increased risk of fracture.
What are some principal causes of osteoporosis?
Decreased Vitamin D
What are some risk factors of osteoporosis?
Heavy use of alcohol, smoking, and some medical disorders
What are some clinical/lab symptoms that indicative of osteomalacia?
Hypocalcemia in serum/urine
Increased alkaline phosphatase
Increased PTH levels
What are the steps of endochondral ossification??
1. Formation of cartilage matrix
2. Development of ossification centers (matrix)
3. Primary ossification (diaphysis)
4. Secondary ossification (epiphyseal plate)
What are the three phases of bone healing?
Condition of excessive bone turnover with uncertain cause (bone deformities)
Paget's disease/ osteitis deformans
What increases calcium absorption in the intestine?
What does PTH induce in the intestine?
Increased absorption of active Vit D (calciferol), which in turn in creases calbindin, which in turn increases the absorption of dietary Ca2+
What does the PTH do in the kidney?
-Increases reabsorption of Ca2+ in kidney
-Decrease reabsorption of Pi
-Increased level of calciferol
What does PTH do in the bone?
Increases RANK receptors in osteoblasts, which increases growth of osteoclasts and then in turn increases bone resorption an release of Ca2+
Vitamin D3 deficiency leads to increase in ___ to compensate for lack of Vitamin D3
What hormone reduces blood Ca2+, opposing the effects of PTH
Calcium is also secreted in response to ____.
Calcitonin receptor G proteins inhibit ____ resorption and ____ membrane retraction.
Traditional spectroscopic method to estimate levels of free Ca2+ but doesn't account for interferences such as albumin levels
Result is the best indicator of free Ca2+ because it's a direct determination using an electrode method
Free (ionized) calcium
Important in relation to binding and excretion of Ca2+
What clinical marker is used to measure mineralization of newly forming bone and is often measured in conditions such as Paget's disease, osteosarcoma, bone fractures, osteoporosis and Vitamin D deficiency.
Specific isoenzymes of alkaline phosphatase
Collagen is exported to the _____________ ___________ and assembled into fibrils
What is the cofactor which assists the hydroxylation of Proline and Lysine in collagen formation.
Procollagen peptidase cleave most of the nonhelical termal peptides to turn _________ into collagen
What are the steps for Transforming Growth Factor-B Signaling
1. Two TGF-B proteins form dimer
2. Homodimer then interacts with TBR-II
3. Recruit 2 TBR-I receptors forming a tetramer receptor/dimer substrate complex
4. TBR conformational change, phosphorylation of TBR-I on serine
5. Serine binds to activate SARA/SMAD chain that serves as transcription factors
6. SMAD4/RSMAD enter nucleus to activate gene products.
Bone remodeling is controlled by hormones from?
What are the 3 proteins used for Osteoclast adhesion?
Vinculin, Talin, and F actin
What intracellular enzyme producues H+ via the reaction H2O +CO2 ->HCO3 + H and pumps them out into the bone matrix by a specialized ATPase pump
Balance of osteoblast/osteoclast activity depends on?
Fibroblast Growth Factors(FGF), Paltelet- Derived growth factor(PDGF), Transforming growth Factor-B(TGF-B), and Bone Morphogenetic Proteins ( BMPs), and pituitary-derived, polypeptide growth hormone
What is responsible for formation of long bones, some flat/irregular bones, for continued growth during childhood, and parts of fracture healing
Vitamin D lads to an increase of?
calbindin (a Ca2+-binding protein) on small intestine enterocytes and in kidney
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