Development of Musculature and Limbs Flashcards

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1
Q

In embryology, anterior/posterior is a synonym for what other directional phrase?

A

cranial/caudal

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2
Q

What are th 4 requirements for normal muscle development?

A
  1. Mesenchymal cells
  2. Genetic signals
  3. Connective tissue framework
  4. fetal movement
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3
Q

Identify the components of the developing fetus at day 21 post fertilization

A
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4
Q

What cells are the “master inducers”? Where do they come from?

A

neural crest cells

originate at the neural fold

they are the “left over” from the formation of the neural tube; capable of ameboid movement and will disperse throughotut the embryo

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5
Q

Which direction do Myotomes split?

What is the name of the sections that split?

Does this happen before or after the somites differentiate?

A

Dorsal/Ventral

Epimere (dorsal)

Hypomere (ventral)

After the somites differentiate

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6
Q

What muscle is formed by the Epimere? What nerve is it innervated by? Where is it located with relation to the transverse process?

What muscle is formed by the Hypomere? What nerve is it innervated by?

A
  • Epimere (
    • epaxial muscle
    • dorsal primary ramus
    • dorsal to transverse process plane and plane of spinal nerve
  • Hypomere (body wall and limbs)
    • hypaxial muscle
    • ventral primary ramus
    • ventral (below) the plane of the spinal nerve
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7
Q

What is the name of the border of how far away the somite influence carriers? What is the name of the embryo lateral and medial to this border?

A

Lateal somitic frontier (down intermediate mesoderm)

lateral: abaxial
medial: primaxial

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8
Q

Identify the following characterisitc for the abaxial and primaxial portion each

origination of myogenic cells?

induction?

innervation?

derivation of connective tissue?

A
  • Primaxial
    • origination of myogenic cells?
      • hypomere and epimere
    • induction?
      • neural tube and notochord
    • innervation?
      • dorsal and ventral rami
    • derivation of connective tissue?
      • somite
  • Abaxial
    • origination of myogenic cells?
      • Hypomere
    • induction?
      • lateral plate mesoderm
    • innervation?
      • ventral ramus
    • derivation of connective tissue?
      • lateral plate
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9
Q

Myogenic cells differentiate from what type of progenitor cell?

What type of cells do Myogenic cells differentiate into?

A

Mesenchymal cells differentaite into myogenic cells

myogenic cells differentiate into postmitotic myoblasts

myoblasts fuse together to form a multinucleated syncytium (myotube), and once myofibrils are present the myotube is termed muscle fiber

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10
Q

What are the 3 populations of head muscles?

A
  1. Tongue: migrate similar to limb muscles
  2. Extraocular muscles: 3 condensations in 3 somitomeres, then migrate to orbit
  3. Brachial muslce: form very early complex with the neural crest cells
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11
Q

What type of signals specify limb development sites? What spinal segments are these located near?

A

a series of Hox genes creat activation gradients along the notochordal axis

forelimb C5-C8

Hindlimb L3-L5

initiated in the 4th week

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12
Q

What would happen if you placed the block of mesenchy for the “hindlimb” of a mouse near where the front limbs should be? Why does this happen?

What is the name of this expanding mesenchyme?

A

the hind limb would grow where the front limb should be

the block of mesenchyme has full limb identity; it develops with full polarity separate from each other and the rest of the embryo

limb bud

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13
Q

What groups forms the anterior-posterior axis of the limb?

A

group fo mesenchymal cell on the posterior edge of hte limb bud called the Zone of Polarizing Activyt (ZPA)

they create a morphogenic field of sonic hedgehog and retinoic acid

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14
Q

what group differentiates the proximal-distal axis of hte limb

A

a thickened strip of ectodermal cells termed the apical ectodermal ridge (AER). THey create a morphogenic field be secreting fibroblast growth factor (FGF), which stimulate proliferation of the mesenchyme

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15
Q

What region of limb undergoes mitotic proliferation?

Which region of limb undergoes differentiation?

A

The progress zone, underneath the apical ectodermal ridge undergoes rapid proliferation that does not differentaite

the more proximal mesenchyme differentiates but grows much solwer,so limbs develop form proximal to distal

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16
Q

By day 32, the progress zone flattens to form waht strucutre?

What happens around day 48?

A

flattened to forma hand or foot plate

around day 48, AER breks up into 5 distinct ridges & apoptosis begins to remove tissue betwteen to leave 5 distinct digits

17
Q

What are the 3 distinct stages of muscle development that occur during week 5 of development?

A

durign week 5 mesenchymal cells from the hypomere migrate into the limb bud to form the appendicular musculature

  1. somite-specific stage
  2. gross functional grouping stage
  3. muscle specific stage
18
Q

What order do the distinct structures of the limb develop?

A

pproliferating mesenchyme of limb bud will form the appendicular skeleton, then the joints, then a connective tissue framework around the skeleton

19
Q

What extends into the limb bud after the myoblast cells?

A

ventral rami of the spinal nerves

once in the limb bud, each ventral ramus will give rise to two branches, on to each muscle condensation representing the major functional group

as the condenstaions differentiate into individual muscles, the neural branches will go through complex pattern of fusion

20
Q

What type of rotation do limbs undergo at around day 47?

A

long-axis rotation (lateral rotation in forelimb and medial rotation in the hindlimb)

21
Q

What is the sequence of limb formation?

A

bone/connective tissue

muscle

nerves

joints

22
Q

What is the name of muscles dorsal to the transverse septum?

What is the name of muscles ventral to the transverse septum?

A

epaxial

hypaxial

23
Q

Why is there a “distorted” pattern of dermatomes and cutaneous innervations in the limbs?

A

sensory (cutaneous) axons trail the motor axons, entering the limb bud before rotation of the limbs

24
Q

What is the name of the syndrome typefied by the unilateral absence of pectoralis major, disruption of the anterior axillary fold, reduction and lateral dislocation of the nipple, and reduction of the breast tissue?

A

Poland Syndrome

connective tissue does not form, so the muscle doesn’t form

25
Q

What is the difference between Duchenne and Becker Muscular Dystrophy?

A

becker muscular dystophy is a mulder form, but involves mutation of the same gene (DMD) that encodes for the scaffolding protein Dystrophin

Duchenne is typically detected around 3 years of age whereaas becker has an onset in early adulthood

26
Q

What is the most common musculoskeletal defect?

What is the inheritance pattern of this defect?

Treatment?

A

Talipes Equinovarus (clubfoot)

multifactorial inheritance pattern

most cases, all anatomical components are present and normally formed, so therapeutic adjustment is adequate for complete reversal

27
Q
A
  • Polydactyly
    • when AER breaks apart into more than 5 segments
  • Cleft hand/foot
    • if it breaks into fewer than 5 segments
  • Syndactyly
    • when apotosis between 5 remaining pieces does not occur
28
Q

What happens when there is a physical disruption or death of the AER?

A

Truncated Limb Syndrome

complete proximal segment of the limb, then an abrupt cessation of the limb development

29
Q

What can happen if the ZPA is disrupted?

A

ZPA “mirror hand” defect

30
Q

What are the major causes of major limb defects in neonates?

How common is this?

A

genetic conditions, physical environment, teratogens

thalidomide– blocks vascular perfusion of limb bud

1/500 neonates