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PMID:20515681

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Citation

He, G, Tavella, S, Hanley, KP, Self, M, Oliver, G, Grifone, R, Hanley, N, Ward, C and Bobola, N (2010) Inactivation of Six2 in mouse identifies a novel genetic mechanism controlling development and growth of the cranial base. Dev. Biol. 344:720-30

Abstract

The cranial base is essential for integrated craniofacial development and growth. It develops as a cartilaginous template that is replaced by bone through the process of endochondral ossification. Here, we describe a novel and specific role for the homeoprotein Six2 in the growth and elongation of the cranial base. Six2-null newborn mice display premature fusion of the bones in the cranial base. Chondrocyte differentiation is abnormal in the Six2-null cranial base, with reduced proliferation and increased terminal differentiation. Gain-of-function experiments indicate that Six2 promotes cartilage development and growth in other body areas and appears therefore to control general regulators of chondrocyte differentiation. Our data indicate that the main factors restricting Six2 function to the cranial base are tissue-specific transcription of the gene and compensatory effects of other Six family members. The comparable expression during human embryogenesis and the high protein conservation from mouse to human implicate SIX2 loss-of-function as a potential congenital cause of anterior cranial base defects in humans.

Links

PubMed Online version:10.1016/j.ydbio.2010.05.509

Keywords

Animals; Animals, Newborn; Bone and Bones; Cartilage/growth & development; Cartilage/metabolism; Cell Differentiation; Chondrogenesis; Homeodomain Proteins/metabolism; Humans; Mice; Mice, Knockout; Mice, Transgenic; Nerve Tissue Proteins; Osteogenesis/physiology; Proteins/metabolism; Skull Base/growth & development

Significance

Annotations

Gene product Qualifier GO Term Evidence Code with/from Aspect Extension Notes Status

MOUSE:SIX4

acts_upstream_of_or_within

GO:0048701: embryonic cranial skeleton morphogenesis

ECO:0000316: genetic interaction evidence used in manual assertion

MGI:MGI:102780

P

Seeded From UniProt

complete

MOUSE:SIX1

acts_upstream_of_or_within

GO:0048701: embryonic cranial skeleton morphogenesis

ECO:0000316: genetic interaction evidence used in manual assertion

MGI:MGI:106034

P

Seeded From UniProt

complete

MOUSE:SIX2

involved_in

GO:0030278: regulation of ossification

ECO:0000315: mutant phenotype evidence used in manual assertion

P

Seeded From UniProt

complete

MOUSE:SIX2

involved_in

GO:0032330: regulation of chondrocyte differentiation

ECO:0000315: mutant phenotype evidence used in manual assertion

P

Seeded From UniProt

complete

MOUSE:SIX2

involved_in

GO:1902732: positive regulation of chondrocyte proliferation

ECO:0000315: mutant phenotype evidence used in manual assertion

P

Seeded From UniProt

complete

MOUSE:SIX2

acts_upstream_of_or_within

GO:0002062: chondrocyte differentiation

ECO:0000315: mutant phenotype evidence used in manual assertion

MGI:MGI:3691857

P

  • results_in_acquisition_of_features_of:(CL:0000138)

Seeded From UniProt

complete

MOUSE:SIX2

acts_upstream_of_or_within

GO:0008283: cell population proliferation

ECO:0000315: mutant phenotype evidence used in manual assertion

MGI:MGI:3691857

P

Seeded From UniProt

complete

MOUSE:SIX2

acts_upstream_of_or_within

GO:0048701: embryonic cranial skeleton morphogenesis

ECO:0000315: mutant phenotype evidence used in manual assertion

MGI:MGI:3691857

P

Seeded From UniProt

complete


See also

References

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