The Small Integrin-Binding LIgand, N-linked Glycoprotein (SIBLING)
family contains five genes that have been shown to play critical functions in
biomineralization as either enhancers and/or inhibitors of calcification. These genes are
located in a cluster on human chromosome 4q21 and include: dentin
sailophosphoprotein (DSPP), dentin matrix protein 1 (DMP-1), matrix extracellular
phosphoglycoprotein (MEPE), bone sailoprotein (IBSP) and osteopontin (SPP1).
Through the rapid advances in molecular genetics and studies of transgenic null mice
substantial progress in determining the function of various SIBLINGs has been
established. To date, two SIBLINGs have been shown to be directly involved in the
pathogenesis of human diseases with altered dentin or bone mineralization phenotypes.
These SIBLINGs are DSPP associated with various dentin structural diseases and
DMP-1 associated with an autosomal recessive form of hypophosphatemic rickets.
Keywords: Small Integrin-Binding LIgand N-linked Glycoproteins, SIBLINGs,
Chromosome 4q21, Dentin SialoPhosphoProtein, Dentin Matrix Protein-1, Matrix
Extracellular phosphoglycoprotein, Bone Sialoprotein, Osteopontin, Genetic
dentin alterations, Biomineralizations, Hypophosphatemic rickets.
