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Med > BJST > Flashcards

BJST Flashcards

1
Q

used as a specific marker of
osteoblast activity

A

Serum Osteoponin

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2
Q

type 1 collagen + smaller amounts of GAGs + other proteins (osteopontin or osteocalcin- for bone formation and mineralization)

A

Osteoid

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3
Q

primarily hydroxyapatite which give bone its hardness and serves as a repository for 99% of the calcium and 85% of the phosphorus

A

Minerals

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4
Q

Produced rapidly (during fetal development or
fracture repair

Always abnormal in adults, but its presence is not specific for any particular disease

A

Woven Bone

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5
Q

Supported by bony trabeculae interspersed with marrow, may be fatty (white) or hematopoietic (red)

A

Lamellar Bone

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6
Q

Located on the surface of the osteoid matrix

Synthesize, transport, and assemble matrix and regulate mineralization

A

Osteoblast

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7
Q

Connected by an intricate network of dendritic cytoplasmic processes through tunnels (canaliculi)

Controls calcium and phosphate levels

Mechanotransduction detects mechanical forces and translate them into myologic activity

A

Osteocytes

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8
Q

Specialized multinucleated macrophages derived
from circulated monocytes

Responsible for bone resorption

A

Osteoclast

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9
Q

Attaches to the bone matrix which creates sealed extracellular trench (resorption pit)

Secretion of acid and neutral proteases, (MMPs), into the pit results in dissolution of inorganic and organic
bone components

A

Osteoclast

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10
Q

Most bones that form during embryogenesis develop from a
cartilage mold via

A

endochondral ossification

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11
Q

Central medullary canal within the anlagen is created by
chondroblasts at approximately

A

8 week of gestation

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12
Q

earliest bone trabeculae

A

Primary Spongiosa

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13
Q

Dense layer of mesenchyme is directly ossified by
osteoblasts without a cartilage anlagen

A

Intramembranous Ossification

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14
Q

Maintains chondrocyte proliferation

A

Growth hormone

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15
Q

Acts on proliferating chondrocytes to induce
hypertrophy

A

Thyroid Hormone

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16
Q

Coordinates chondrocyte proliferation and
differentiation with osteoblast proliferation

A

Indian Hedgehog

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17
Q

Activates the PTH receptor to maintain
chondrocyte proliferation

A

PTHrP

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18
Q

Activate β-catenin to promote chondrocyte
proliferation and maturation

A

WTN Growth Factors

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19
Q

Expressed by proliferating, but not
hypertrophic, chondrocytes that is essential for
differentiation of chondrocyte precursors

A

SOX9

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20
Q

Expressed in early hypertrophic chondrocytes
which controls terminal chondrocyte and
osteoblast differentiation

A

RUNX2

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21
Q

Act on hypertrophic chondrocytes to inhibit
proliferation and promote differentiation

A

Fibroblast Growth Factor

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22
Q

Chondrocyte proliferation and hypertrophy at
the growth plate

A

Bone Morphogenic Proteins

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23
Q

Remodeling takes place at

A

Bone Multicellular Unit (BMU)

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24
Q

Balance between bone formation and resorption is modulated by

A

RANK & WNT

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25
Q

expressed on osteoclast
precursor

A

Transmembrane receptor RANK

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26
Q

expressed on osteoblasts and marrow stromal cells

A

RANK ligand

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27
Q

“decoy” receptor made by osteoblasts;
prevents interaction with RANK

A

Osteoprotegerin

28
Q

hormones, vitamin D, inflammatory
cytokines and growth factors

A

Systemic factors

29
Q

Systemic Factors:

osteoclast differentiation -
increased bone turnover

A

PTH, IL-1 and glucocorticoids-

30
Q

Systemic Factors:

favor OPG
expression or block osteoclast activity

A

Bone morphogenic proteins and sex hormones

31
Q

Peak bone mass is achieved in ____ after cessation of _______

A

Early adulthood; skeletal growth

32
Q

resorption = formation —–> decline in skeletal mass happens when

A

4th decade

33
Q

localized disruption of the migration and
condensation of the mesenchyme

A

Dysostoses

34
Q

May occur in isolation or as part of more complex syndromes

Caused by defects in mesenchymal condensation and
differentiation into cartilage anlage

A

Dysostoses

35
Q

Complete absence of a bone or entire digit

A

aplasia

36
Q

extra bones or digits

A

supernumerary digit

37
Q

abnormal fusion of bones

A

syndactyly, carniosynostosis

38
Q

Complete absence of a bone or entire digit

A

Dysplasia

39
Q

Arise from mutations in genes that control development
or remodeling of the entire skeleton

Implies abnormal growth, bbut it is not a precursor of
neoplasia, as is the case with dysplasias of epithelial cells

A

Dysplasia

40
Q

caused by mutations in the homeobox HOXD13 gene and are characterized by shortening of the terminal phalanges of the thumb and big toe

A

Brachydactyl types D & E

41
Q

global disorganization of bone and/or cartilage

A

Dysplasia

42
Q

loss of function mutation in RUNX2

A

Cleidocranial dysplasia

43
Q

Characterized by:

Patent fontanelles

Delayed closure of cranial sutures

Wormian bones (extra bones that occur within a cranial suture)

Delayed eruption of secondary teeth

Primitive clavicles

Short stature

A

Cleidocranial Dysplasia

44
Q

most common skeletal dysplasia and a major
cause of dwarfism

A

Achondroplasia

45
Q

Autosomal dominant disorder caused by gain-of-function
mutations in the FGF receptor 3 (FGFR3) gene

A

Achondroplasia

46
Q

Retarded cartilage growth results in:

Shortened proximal extremities

Enlarged head with bulging forehead

Depression of the root of the nose despite a trunk of relatively normal length

A

Achondroplasia

47
Q

most common lethal form of dwarfism

A

Thanatophoric dysplasia

48
Q

Micromelic limbs, frontal bossing, relative macrocephaly,
small chest cavity, and bell-shaped abdomen

A

Thanatophoric Dysplasia

49
Q

HOXD13

Transcription
factor

Short, broad terminal
phalanges of first digits

A

Brachydactyly
types D and E

50
Q

SOX9

Transcription
factor

Sex reversal, abnormal
skeletal development

A

Camptomelic
dysplasia

51
Q

RUNX2
Transcription
factor

Abnormal clavicles,
Wormian bones,
supernumerary teeth

A

Cleidocranial
dysplasia

52
Q

TBX5
Transcription
factor

Congenital abnormalities,
forelimb anomalies

A

Holt-Oram
syndrome

53
Q

LMXIB
Transcription
factor

Hypoplastic nails,
hypoplastic or aplastic
patellas, dislocated radialhead, progressive
nephropathy

A

Nail-patella
syndrome

54
Q

PAX3

Transcription
factor

Hearing loss, abnormal
pigmentation, craniofacial
abnormalities

A

Waardenburg
syndrome types 1
and 3

55
Q

FGFR3

Receptor

Short stature, rhizomelic
shortening of limbs,
frontal bossing, midface
deficiency

A

Achondroplasia

56
Q

FGFR3

Receptor

Disproportionately short stature, micromelia,
relative macrocephaly

A

Hypochondroplasia

57
Q

LRP5

Receptor

Increased bone density,
hearing loss, skeletal
fragility

A

Osteopetrosis,
autosomal dominant

58
Q

RANKL

Receptor - ligand

Increased bone density

A

Osteopetrosis,
infantile form

59
Q

LRP5

Receptor

Congenital or infantonset loss of vision, skeletal fragility

A

Osteoporosispseudoglioma syndrome

60
Q

FGFR3

Receptor

Severe limb shortening
and bowing, frontal
bossing, depressed nasal bridge

A

Thanatophoric
dysplasia

61
Q

COL2A1

Type II
collagen

Short trunk

A

Achondrogenesis
type 2

62
Q

COL10A1

Type X
collagen

Mildly short stature

A

Metaphyseal dysplasia,
Schmid type

63
Q

COL1A1,
COL1A2

Type 1
collagen

Bone fragility

A

Osteogenesis
imperfecta types 1-4

64
Q

CA2

Carbonic
anhydrase

Increased bone density,
fragility, renal tubular
acidosis

A

Osteopetrosis
with renal tubular acidosis

65
Q

CLCN7

Chloride
channel

Increased bone density,
fragility

A

Osteopetrosis,
late onset type 2

Med (6 decks)

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