SP7

SP7 is a C2H2-type zinc finger transcription factor of the SP gene family and a putative master regulator of bone cell differentiation (Gao et al., 2004 [PubMed 15474293]).[supplied by OMIM

Full Name

Sp7 transcription factor

Function

Transcriptional activator essential for osteoblast differentiation (PubMed:23457570).
Binds to SP1 and EKLF consensus sequences and to other G/C-rich sequences (By similarity).

Biological Process

Biological Process cellular response to zinc ion starvationIEA:Ensembl
Biological Process cementum mineralizationIEA:Ensembl
Biological Process diphosphate metabolic processIEA:Ensembl
Biological Process hematopoietic stem cell differentiationManual Assertion Based On ExperimentIDA:BHF-UCL
Biological Process osteoblast differentiationManual Assertion Based On ExperimentIDA:BHF-UCL
Biological Process positive regulation of stem cell differentiationManual Assertion Based On ExperimentIDA:BHF-UCL
Biological Process positive regulation of transcription by RNA polymerase IIManual Assertion Based On ExperimentIDA:BHF-UCL
Biological Process regulation of hydrolase activityIEA:Ensembl
Biological Process regulation of transcription by RNA polymerase IIBy SimilarityISS:BHF-UCL
Biological Process response to insulinIEA:Ensembl
Biological Process response to organic cyclic compoundIEA:Ensembl

Cellular Location

Nucleus

Involvement in disease

Osteogenesis imperfecta 12 (OI12):
A form of osteogenesis imperfecta, a connective tissue disorder characterized by low bone mass, bone fragility and susceptibility to fractures after minimal trauma. Disease severity ranges from very mild forms without fractures to intrauterine fractures and perinatal lethality. Extraskeletal manifestations, which affect a variable number of patients, are dentinogenesis imperfecta, hearing loss, and blue sclerae. OI12 is an autosomal recessive form characterized by recurrent fractures, mild bone deformations, generalized osteoporosis, delayed teeth eruption, no dentinogenesis imperfecta, normal hearing, and white sclerae.

PTM

Ubiquitination at leads to proteasomal degradation. SP7 is a short-live protein with an endogenous half-life of approximately 12 hours.
Propionylated. Depropionylation at Lys-371 by SIRT7 activates transcription factor activity and positively regulates bone formation by osteoblasts.