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Questions and Answers
What condition describes the complete absence of a limb?
Which limb defect is characterized by the partial absence of a limb?
What is the primary cause of syndactyly?
Ectrodactyly is often referred to as which of the following?
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Which condition is characterized by the presence of extra digits?
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What is the role of the zone of polarizing activity (ZPA) in limb development?
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In which week do the first digital rays appear in the developing hand and foot?
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Which statement accurately describes limb bone differentiation?
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How does limb rotation differ between the upper and lower limbs?
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What is the primary result of the rotation of the lower limb during development?
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Which of the following describes the mechanism of digit formation from digital rays?
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What is the primary cause of limb defects noted in clinical correlations?
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What is the primary signal produced by the zone of polarizing activity (ZPA)?
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What is the primary origin of the posterior skull and vertebral column?
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Which structure differentiates into muscle and contributes to the dermis of the back?
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From which cells does the craniofacial skeleton develop?
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What is the segmental pattern of innervation in the adult derived from?
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What is formed by the ventro-medial cells of the somite?
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Which part of the somite is associated with intrinsic bones and muscles of the back?
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Which mesodermal structure gives rise to the sternum and appendicular skeleton?
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During somite differentiation, what signals the ventro-medial cells to become mesenchymal?
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Which of the following is the correct order of development from somites?
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What does the hypomere develop into?
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What is the process by which sclerotome cells organize around the neural tube?
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Which part of the sclerotome fuses with the cranial half of the adjacent sclerotome?
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What happens to the notochord during the formation of vertebrae?
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What is the cause of congenital scoliosis?
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Which condition results from incomplete resegmentation of sclerotomes?
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Which embryonic structure gives rise to all skeletal muscle?
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What are the muscles derived from epimere called?
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What is the primary event occurring during the limb bud development at week 4?
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What signals the overlying ectoderm to grow during limb bud development?
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Which domain contains only somite-derived cells?
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What condition is characterized by a depressed sternum?
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Which of the following correctly describes the structure of ribs?
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What is the effect of Lmx-1 expression in limb development?
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Study Notes
Introduction to Musculoskeletal Development
- Objectives: Understand cell types from somites, development of axial and appendicular skeletons, and limb development.
- Timeline: Development occurs around Week 5 (35-38 days into gestation).
Origin of Skeletal Tissue
- Paraxial Mesoderm: Forms posterior skull, vertebral column, and ribs.
- Lateral Plate Mesoderm: Gives rise to sternum and appendicular skeleton.
- Neural Crest Cells: Contribute to craniofacial skeleton.
Somite Structure and Function
- Somites: Segmental structures lateral to the neural tube that differentiate into various tissues.
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Differentiation:
- Ventro-medial cells become mesenchymal, forming sclerotome (tendons, ligaments, bones).
- Dorso-lateral cells form dermomyotome (muscle, dermis).
Innervation of Somites
- Each somite correlates with one spinal nerve, leading to segmental innervation patterns for muscles and skin.
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Divisions:
- Epimere: Associated with dorsal rami, forming intrinsic back structures.
- Hypomere: Associated with ventral rami, forming muscles/connective tissues of the trunk and limbs.
Axial Skeleton Development
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Vertebrae Resegmentation:
- Sclerotome cells reorganize around the neural tube before they split and fuse to form vertebrae.
- Original notochord transforms into nucleus pulposus.
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Clinical Correlations:
- Congenital Scoliosis: Results from hemivertebra formation due to partial vertebra development.
- Klippel-Feil Syndrome: Incomplete resegmentation causes fused vertebrae, leading to a short neck, low hairline, and limited neck motion.
Formation of Ribs and Sternum
- Ribs: Develop from sclerotomes that form thoracic vertebrae with three embryonic structures (centrum, neural arch, costal process).
- Sternum: Unique as it originates from parietal lateral plate mesoderm.
Appendicular Skeleton Development
- Limbs derive from parietal lateral plate mesoderm.
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Skeletal Muscle Development:
- Somites are the source of all skeletal muscle; epaxial (intrinsic back) and hypaxial (other skeletal muscle) classifications based on somite origin.
Limb Bud Formation
- Limb Buds: Upper limbs appear in Week 4, lower limbs in Week 5, consisting of ectodermal caps and mesodermal cores.
- Apical Ectoderm Ridge (AER): Crucial for limb growth, directing mesoderm proliferation via signaling molecules (FGF-10 and FGF-8).
Limb Axis and Differentiation
- Proximal/Distal Axis: AER signals distal limb formation; dorsal/ventral axis differentiation involves homeobox genes like Lmx-1.
- Cranial/Caudal Axis: Zone of Polarizing Activity (ZPA) directs digit positioning via Sonic hedgehog (SHH) signaling, contributing to varying digit lengths.
Limb Bone Development
- Differentiation: Limb bones arise from proximal to distal: humerus/femur (stylopod), radius/ulna/tibia/fibula (zeugopod), and carpals/metacarpals/phalanges/tarsals/metatarsals (autopod).
- Ossification: Bones form through endochondral ossification, starting from a cartilage template.
Digital Formation and Limb Rotation
- Digital Rays: Form by apoptosis in the AER; complete separation of digits typically occurs by Week 8.
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Rotation:
- Upper limbs rotate laterally (extensor muscles lateral).
- Lower limbs rotate medially (extensor muscles anterior).
Limb Innervation
- Dermatomes: Skin regions supplied by specific spinal nerves; limb rotation modifies original innervation patterns.
- Motor Innervation: Reflects segmental development; includes specific mappings for upper limb muscle innervation.
Clinical Correlations in Limb Development
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Limb Defects:
- Amelia: Complete limb absence.
- Meromelia: Partial limb absence.
- Phocomelia: Absence of long limb bones.
- Brachydactyly: Shortened digits from inadequate proliferation.
- Syndactyly: Fused digits from insufficient apoptosis.
- Polydactyly: Extra digits resulting from additional apoptotic site development.
- Ectrodactyly: Missing digits leading to cleft conditions.
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Description
Explore the intricate processes involved in the development of the musculoskeletal system, including somite-derived cells and their correlation to spinal nerves. Learn about the formation of axial and appendicular skeleton components as well as limb development from bud formation to digit maturation.