Development of Muscles and Limbs Flashcards

1
Q

“The upper limb buds are visible by day 24. The lower limb buds become visible at the end of the fourth week [2 days after appearance of upper limb buds] with the activation of a group of mesenchymal cells in the ____(a)____ lateral mesoderm [somatic/splanchinic]. ____(b)____ genes regulate patterning in the formation of the limbs. The limb buds form deep to a thick band of ectoderm, the ____(c)____. Each limb bud consists of a mesenchymal core of mesoderm covered by a layer of ectoderm.

A

(a) somatic
(b) Homeobox (Hox)
(c) apical ectodermal ridge (AER)

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2
Q

State the origin of skeletal, cardiac, smooth muscles and myoepithelial cells.

A

(a) Skeletal muscles: paraxial mesoderm except iridal and ciliary muscles of the eye, which originate from neuroectoderm.
(b) Cardiac muscle and smooth muscles of the gut and respiratory system: splanchic mesoderm
(c) Smooth muscles of blood vessels and erector pili muscles: local mesenchyme
(d) Myoepithelial cells: ectoderm

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3
Q

How does paraxial mesoderm segment to form somites?

A
  • paraxial mesoderm cells become organized into whorls of cells called somitomeres.
  • the first seven pairs of somitomeres persist
  • cells from 8th pair of somitomeres caudally acquire epithelial characteristics and fragment to form blocks called somites.
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4
Q

Outline the key stages of development of the hand.

A
  1. The distal ends of the limb buds flatten into hand plates.
  2. Condensation of mesenchyme resulting in the formatin of digital rays in the hand plate
  3. Formation of notches between digital rays
    [The intervals between the digital rays are occupied by loose mesenchyme. These intervening regions of mesenchyme soon break down, forming notches between the digital rays.]
  4. Formation of webs/webbing between the developing digits
  5. Apoptosis of webs
  6. Development of phalanges and carpals from mesenchyme
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5
Q

State the differences between upper limb and lower limb development.

A
  1. Site of origin: upper limb from the lower cervical region, lower limb from the lumbosacral region
  2. Timing: upper limb development is ahead of lower limb development by 2 days
  3. Rotation: upper limb rotates laterally, lower limb rotates medially
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6
Q

When the sclerotome migrates, the remaining part of the somite is called ____(a)____ which splits to form the ____(b)____ and ____(c)____.

A

(a) dermomyotome
(b) and (c) dermatome and myotome

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7
Q

When the dermomyotome is exposed to Wnts and there is repression of BMP activity, there are 3 muscle producing regions. State the 3 regions and their derivatives.

A

a) Dorsal region
Myotome: epaxial muscles
Limb myoblasts: extensor muscles

b) Central region
Dermatome: muscles and satellite cells, dermis and brown fat

c) Ventral region
Myotome: hypaxial muscles
Limb myoblasts: flexor muscles

[Diagram]

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8
Q

State the origin of various regional musculature.
[cranial musculature, muscles of the tongue, trunk muscles, limb muscles]

A

Cranial musculature develops mainly from somatomeres. Patterns of muscle formation in the head are directed by connective tissue elements derived from neural crest cells.
Muscles of the tongue develop from occipital myotomes.
Trunk muscles arise from somites.
Limb muscles are from somatic mesoderm, cells which migrate to the developing limb bud.

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9
Q

The major derivatives of the myotome are epimere and hypomere (check the diagram of that in the relevant textbooks, it is commonly examined). State what the epimere gives rise to.
Click here as well to view the diagram

A

a) Extensor muscles of the neck.
b) Erector spinae of the back.
c) Lumbar extensor muscles.
The extensor muscles derived from the sacral and coccygeal myotomes degenerate to form dorsal sacrococcygeal ligaments.

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10
Q

State the derivatives of the hypomere in the:
1. In the neck
2. In the thorax
3. In the abdomen
4. In the sacro-coccygeal region

A

In the neck: prevertebral & scalene muscles, geniohyoid & infrahyoid muscles (from ventral tip of hypomere).
In the thorax: intercostal muscles.
In the abdomen: the oblique muscles & rectus abdominis (from ventral tip) and quadratus lumborum.
In the sacro-coccygeal region: muscles of the pelvic diaphragm and striated perianal muscles.

Further notes:
Preverterbral muscles of the neck include: rectus capitis anterior, rectus capitis lateralis, longus capitis and longus colli muscles.

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11
Q

Describe the histogenesis of skeletal muscles.

A

Migration of mesenchymal cells to the site of muscle formation.
Elongation of mesenchymal cells & their differentiation into myoblasts.
Fusion of myoblasts to form myotubes. [Some questions for you: What happens to the myoblasts that don’t fuse? What do they become? Would they eventually fuse later in life? If so, after what happens?]
✓ Development of myofilaments in the cytoplasm of the myotubes, during or after fusion of myoblasts.
✓ Transformation of myotubes into myocytes through development of myofibrils & other organelles characteristic of skeletal muscle.
✓ Investment of myocytes with external basal laminae, individually or in groups.

Further notes:
Myofibrils are the main rod-like structures of the muscle cells. They are long, cylindrical structures that run the entire length of the muscle fiber. Myofibrils are composed of myofilaments, which are arranged in a repeating pattern of sarcomeres. The sarcomeres are the basic functional units of the myofibril, responsible for the contraction of the muscle fiber.

On the other hand, myofilaments are the individual protein strands that make up myofibrils. They are made up of two types of protein: actin and myosin. These proteins work together to create the sliding motion that allows muscles to contract and relax

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12
Q

Describe the histogenesis of cardiac muscles.

A

The process is the same as for skeletal muscle save for the following differences:

Mesenchymal cells are of splanchnic origin & aggregate around the endothelial tube destined to form the heart.

Myoblasts establish intercalated disks between them.

Later in development, a network of cardiac myocytes undergoes an alternative pathway of differentiation characterized by increased size, reduction in concentration of myofibrils & accumulation of cytoplasmic glycogen ( the conduction system, which also has different protein isoforms).

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13
Q

a) Tendons of epaxial muscles are derived from the ________________ layer within the somites.
b) Tendons of limb and hypaxial muscles are derived from the ________________ mesoderm.

A

a) syndetome
b) lateral plate

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14
Q

State the factors in muscle location, organization and morphogenesis, with examples.

A

a) Migration
- muscles of facial expression from the neck
- trapezius from the head to neck & back
- latissimus dorsi from the cervical region to the back

b) Fusion
- rectus abdominis from fusion of mesenchymal bars at the ventral tips of the abdominal hypomere

c) Splitting
- Intercostal muscles and oblique abdominal muscles result from splitting of myotomes.

d) Regression
- Some myotomes regress into aponeurosis and ligaments e.g abdominal aponeurosis, the sacrospinous and sacrotuberous ligaments.

e) Interaction with nerves
Muscle fibres which do not form functional units with nerves degenerate e.g.

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15
Q

Explain the mechanisms of muscle growth.

A

1) Hypertrophy: most muscles increase in size by this method. The increase in diameter of muscle fibres is through formation of more myofilaments.

2) Hyperplasia: this involves increase in the number of cells by new myocyte formation. It occurs in the uterus during pregnancy.

3) In skeletal muscle, some undifferentiated cells called myosatellite cells may contribute to growth by forming new myoblasts

NB// Myosatellite cells also have a role in the limited skeletal muscle regeneration and could be the origin of skeletal muscle tumours.

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16
Q

State the factors influencing growth of muscles.

A

Nutrition: starvation leads to muscle wasting.
Innervation: denervation leads to muscle atrophy.
Exercise: usually leads to muscle hypertrophy.
Hormones: anabolic steroids e.g testosterone lead to muscle growth, others include growth hormone
Growth factors.

17
Q

Make notes on congenital malformations of muscles due to absence.

A
  • Any muscle in the body may be absent, but commonly: palmaris longus, plantaris, pectoralis major etc.
  • Absence of some muscles may cause immobility of multiple joints leading to contracture and deformation (ARTHROGRYPOSIS MULTIPLEX CONGENITA).
  • Absence of pectoralis major may be associated with breast defects & limb malformations e.g syndactyly (POLAND SYNDROME).
18
Q

I) Prune-belly syndrome is an example of congenital ________________.
II) State its embryological basis.

A

I) hypotonia
II) Failure of anterior abdominal wall muscles to fuse.

[Image]

19
Q

State the embryological basis of congenital torticollis (wry neck).

A

Sternocleidomastoid muscle underwent fibrosis due to vascular failure.

20
Q

State two examples of anomalies of congenital weakness of muscles.

A

a) congenital ptosis
b) Duchenne muscular dystrophy

21
Q

What is the function of the Zone of Polarizing Activity of the limb bud?

A

It maintains the structure and function of the apical ectodermal ridge.
[Diagram: regions of the limb bud]

22
Q

State the signaling centres responsible for the following axis growth in limbs;
a) Proximal-distal:
b) Antero-posterior:

A

a) Apical ectodermal ridge (AER)
b) Zone of polarizing activity (ZPA)

23
Q

Define the following terms:
a) amelia
b) meromelia
c) hemimelia
d) phocomelia
e) brachydactyly
f) polydactyly
g) syndactyly (state embryological basis too)
h) congenital talipes equinovarus (clubfoot)
i) ectrodactyly (lobster-claw hand deformity/Split Hand/Foot Malformation)

A

a) amelia: absence of limbs

b) meromelia: absence of part of a limb [includes hemimelia and phocomelia]

c) hemimelia: unilateral or bilateral underdevelopment of the distal part of the lower or upper limb. The affected bone may be shortened or not develop at all. [fibular hemimelia, tibial hemimelia, radial hemimelia, ulnar hemimelia]

d) phocomelia: limbs are extremely shortened resulting in hands or feet being attached close to the trunk
[proximal aspect of a limb is absent resulting in the hand or foot being attached directly to the trunk]

e) brachydactyly: fingers and toes that are shorter than normal [cause is genetic]

f) polydactyly: presence of supernumerary digits (more than five digits of the hand or feet) [as a result of formation of an extra digital ray - due to mutation of autosomal dominant genes]

g) syndactyly: caused by failure of apoptosis to occur between developing digits

h) congenital talipes equinovarus: musculoskeletal deformity whereby the sole of the foot is turned medially and the foot is inverted

i) ectrodactyly: absence of one or more central digits [due to failure of development of one or more digital rays]

24
Q

Explain two types of syndactyly.

A
  1. Cutaneous syndactyly: failure of webs to degenerate between two or more digits
  2. Osseous syndactyly: occurs when notches between digital rays fail to develop and as a result, separation of digits does not occur
25
Q

State 2 syndromes associated with radial aplasia.

A

Holt-Oram syndrome
TAR syndrome (Thrombocytopenia-absent radius)

26
Q

Achondroplasia occurs due to?

A

defective endochondral ossification at the epiphyseal plates of cartilage, particularly of long bones.

27
Q

State the anatomical features of club foot.

A

midfoot cavus/high arched
forefoot adductus
hindfoot varus
hindfoot equinus