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What happens in the body when someone has Fibrodysplasia Ossificans Progressiva (FOP)?
muscles and connective tissue turn into bone
what are the functions of the skeletal system?
support, protection, body movement, blood cells formation, storage of inorganic materials
what is the term for blood cell formation? where does it happen?
hematopoiesis - red bone marrow
which inorganic materials does the skeletal system store?
calcium, magnesium, sodium, and potassium
About how many bones are in the skeletal system?
206
What are the two divisions of the bones?
axial and appendicular
what makes up the axial division?
head, trunk, neck, skull, hyoid bone, vertebral column, ribcage, sternum (think the inner/upper section of the body)
what makes up the appendicular division?
pectoral girdle (collarbone and shoulder area), arms, pelvic girdle, legs (think the âexteriorâ part of the body)
what causes flare-ups of bone growth with FOP?
any slight injury or minor trauma
will FOP result in death?
yes, eventually it will because muscles are important for function
what are the ends of bones called?
epiphysis
what is the main region/shaft of the bone called (the part in between the epiphysises)?
diaphysis
what is the cartilage covering the ends of the bones?
articular cartilage
what is the tough membrane covering the entire bone?
periosteum
what is within the diaphysis and contains bone marrow?
medulla/medullary cavity
what does proximal epiphysis mean?
closest to the bodyâs center
what does distal epiphysis mean?
farthest away from the bodyâs center
what are the types of bones and their functions?
flat - protection
long - support weight and movement
short - stability/movement
irregular - protects organs
sesamoid - reinforces tendons
what is the lining of the medullary cavity called?
endosteum
what does red marrow do?
produce blood cells
what does yellow marrow do?
store fat
where is compact bone found in the bone?
the wall of the diaphysis
where is spongy/cancellous bone found?
the epiphysis
what is bone tissue called? what is its matrix composed of?
osseous tissue - collagen and inorganic salts
what is collagen?
connective, supportive tissue
what are mature bone cells called?
osteocytes
what are the tiny chambers that osteocytes are enclosed in called?
lucanae
what are the rings formed by the lucanae called? what do they form around?
lamallae - haversian canal
what connect osteocytes?
canaliculi
are Volkmannâs canals horizontal or vertical?
horizontal
are haversian canals horizontal or vertical?
vertical
what is ossification?
the process by which hyaline cartilage (which is what bones initially are) gradually changes into bone tissue
what does the primary ossification center do?
increase diameter
what does the secondary ossification center do?
increase length
what is hallux adbucto calgus deviation?
when the toes are bent due to abnormal ossification
what did jasminâs mother notice that led to a FOP diagnosis?
ossification in the toes (bent shape) and stiff neck
what do osteoblasts do?
create osteocytes
what do osteoclasts do?
absorb or consume osteocytes
what is the epiphyseal disk/line (growth plate) and how does it change with age?
a band of cartilage between the epiphysis and diaphysis - slowly âclosesâ or goes away with age (bigger growth plate at young age, meaning there is more room to grow)
what is heterotopic ossification (HO)?
abnormal bone growth in non-skeletal tissues including muscle, tendons, and soft tissue
what is the cause of FOP?
gene mutation that codes for more bone morphogenetic protein (BMP) than usual; the receptor stays ON all of the time
what are noggin and gremlin?
inhibitors of BMP
what can be done for ppl with FOP?
CRISPR- get rid of the mutation and replace it with the normal gene