The molecular basis for developmental disorders of the pituitary gland in man

The development of the anterior pituitary gland is dependent upon a cascade of signalling molecules and developmental genes that function as transcription factors. Many of these genes are homeobox genes which contain a DNA‐binding region or homeobox. Animal models have given a valuable insight into...

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Bibliographic Details
Published in:Clinical genetics 2000-05, Vol.57 (5), p.337-346
Main Authors: Dattani, Mt, Robinson, Ic
Format: Article
Language:English
Subjects:
Animals
Biological and medical sciences
Child
Child, Preschool
Developmental Disabilities - genetics
Endocrinopathies
Gene Expression Regulation, Developmental - physiology
Genes, Homeobox - genetics
Hesx1
homeobox
Humans
Hypothalamus. Hypophysis. Epiphysis (diseases)
Medical sciences
Non tumoral diseases. Target tissue resistance. Benign neoplasms
Pit-1
pituitary
Pituitary Gland, Anterior - growth & development
Prop1
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Summary:The development of the anterior pituitary gland is dependent upon a cascade of signalling molecules and developmental genes that function as transcription factors. Many of these genes are homeobox genes which contain a DNA‐binding region or homeobox. Animal models have given a valuable insight into human pituitary disease. For example, Pit‐1 and Prop1 mutants are known to have deficiencies of growth hormone, prolactin and thyroid‐stimulating hormone. Human phenotypes arising as a result of mutations in these genes are similar to the mouse mutants. Mutations in the novel homeobox gene Hesx1/HESX1 are associated with the highly variable phenotype of septo‐optic dysplasia in mouse and man. The unravelling of this complex developmental cascade is just commencing.
ISSN:0009-9163
1399-0004
DOI:10.1034/j.1399-0004.2000.570503.x