By Cheng-Ming Chuong
Epithelial appendages are the derivatives of the epithelial sheet. at the physique floor, they contain hair, feathers, scales, claws, horns, sebaceous glands, mammary glands, and so on. within the oral hollow space, they comprise enamel, salivary glands, and so forth. contained in the physique, the surfaces of the intestine are lined with assorted derivatives that line the tummy, small gut, colon, and so forth. Lung, pancreas and liver start as epithelial derivatives of the endodermal tube. those organs are diversified in constitution and serve as. notwithstanding, is there a universal subject matter underlying their formation?This booklet identifies easy rules underlying the doubtless different phenomena of organ formation. facts accrued to this point recommend that the majority of the molecular mechanisms are shared in several organogenetic tactics, in physique axis development, or even have their roots in Drosophila morphogenesis. the recent unifying idea is a excitement to relish. each one bankruptcy presents the main up-to date info written through leaders within the box.
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Additional info for Molecular Basis of Epithelial Appendage Morphogenesis
77-79). The evolutionary conservation of mechanisms of signaling are perhaps best depicted in the midgut, where Wg participates in mesenchymal to epithelial communication. 82 These events provide a morphoregulatory function in the developing fly embryo that leads to the formation of the second midgut constriction. At another site, in the epidermis, Wg regulates the patterning and established polarity in the individual segments that make up the fly body plan. Wg expression from certain epidermal cells signals neighboring cells to maintain their expression of the homeobox transcription factor engrailed (En).
EDA expression correlates with those tissue types that are defective in EDA syndromes, suggesting that the EDA protein does play an instructive role in the development of these ectodermally-derived tissues. Sequence analyses of 15 patients afflicted with EDA revealed that six had X:autosomal translocation or deletion, and nine had single base point mutations, including two insertions, two deletions and five substitutions. One recurring mutation was the substitution of leucine to arginine at position 69, suggesting that this amino acid may have a particularly significant function in the protein.
Affected individuals present hypotrichosis, hypodontia, nail defects and normal functional sweat glands. Other manifestations include skin hyperpigmentation, palmar and plantar dyskeratosis, strabismus and clubbing of fingers. 67 Trichorhinophalangeal syndrome (OMIM #190350) literally implies defects of the hair, nose and digits, but of diverse severity and type. Defects in teeth, nails, eyes and skeleton with mental retardation and short stature have been described. As the name suggests, ectrodactyly, ectodermal dysplasia and cleft lip/ palate (EEC) syndrome (OMIM #129900) encompasses defects in three major tissues, and can also include a plethora of defects in hearing, vision, psychomotor capabilities and urogenital system.
Molecular Basis of Epithelial Appendage Morphogenesis by Cheng-Ming Chuong