英文名称
RUNX2 Recombinant Rabbit mAb
英文别名
AML3; CBF-alpha-1; CBFA1; CCD; CCD1; CLCD; OSF-2; OSF2; PEA2aA; PEBP2aA; Cbf; Cbfa-1; LS3; Pebp2a1; Pebpa2a; RUNX2_HUMAN; RUNX2; Acute myeloid leukemia 3 protein; Core-binding factor subunit alpha-1 (CBF-alpha-1); Oncogene AML-3; Osteoblast-specific transcription factor 2 (OSF-2); Polyomavirus enhancer-binding protein 2 alpha A subunit (PEA2-alpha A | PEBP2-alpha A); SL3-3 enhancer factor 1 alpha A subunit; SL3/AKV core-binding factor alpha A subunit; PEBP2A; RUNX2_HORSE; RUNX2_MOUSE; RUNX2_RAT; RUNX family transcription factor 2; runt-related transcription factor 2; runt related transcription factor 2
免疫原
A synthesized peptide derived from human RUNX2: 300-450/521
纯化方法
affinity purified by Protein A
储存液
0.01M TBS (pH7.4) with 1% BSA, 0.02% Proclin300 and 50% Glycerol.
保存条件
Shipped at 4℃. Store at -20℃ for one year. Avoid repeated freeze/thaw cycles.
注意事项
This product as supplied is intended for research use only, not for use in human, therapeutic or diagnostic applications.
产品介绍
RUNX2又称:Cbfα1(Core-binding factor, alpha 3 subunit) 是新发现的一类调控间充质干细胞向成骨方向分化的特异性转录因子,参与骨形成,骨骼生长和发育的一类重要细胞,它起源于多能间充质干细胞,是间充质干细胞在体内的各种调控因素的调节下发育而成的。
背景资料
This gene is a member of the RUNX family of transcription factors and encodes a nuclear protein with an Runt DNA-binding domain. This protein is essential for osteoblastic differentiation and skeletal morphogenesis and acts as a scaffold for nucleic acids and regulatory factors involved in skeletal gene expression. The protein can bind DNA both as a monomer or, with more affinity, as a subunit of a heterodimeric complex. Mutations in this gene have been associated with the bone development disorder cleidocranial dysplasia (CCD). Transcript variants that encode different protein isoforms result from the use of alternate promoters as well as alternate splicing. [provided by RefSeq, Jul 2008].
蛋白名
Runt-related transcription factor 2
亚基
Heterodimer of an alpha and a beta subunit. Interacts with HIVEP3. The alpha subunit binds DNA as a monomer and through the Runt domain. DNA-binding is increased by heterodimerization. Interacts with G22P1 (Ku70) and XRCC5 (Ku80). Interacts with MYST3 and MYST4.
组织特异性
Specifically expressed in osteoblasts.
翻译后修饰
Phosphorylated; probably by MAP kinases (MAPK). Isoform 3 is phosphorylated on Ser340.
疾病
Defects in RUNX2 are the cause of cleidocranial dysplasia (CLCD) [MIM:119600]; also known as cleidocranial dysostosis (CCD). CLCD is an autosomal dominant skeletal disorder with high penetrance and variable expressivity. It is due to defective endochondral and intramembranous bone formation. Typical features include hypoplasia/aplasia of clavicles, patent fontanelles, wormian bones (additional cranial plates caused by abnormal ossification of the calvaria), supernumerary teeth, short stature, and other skeletal changes. In some cases defects in RUNX2 are exclusively associated with dental anomalies.
相似性
Contains 1 Runt domain.
功能
Transcription factor involved in osteoblastic differentiation and skeletal morphogenesis. Essential for the maturation of osteoblasts and both intramembranous and endochondral ossification. CBF binds to the core site, 5'-PYGPYGGT-3', of a number of enhancers and promoters, including murine leukemia virus, polyomavirus enhancer, T-cell receptor enhancers, osteocalcin, osteopontin, bone sialoprotein, alpha 1(I) collagen, LCK, IL-3 and GM-CSF promoters (By similarity). Inhibits MYST4-dependent transcriptional activation. [SUBUNIT] Interaction with SATB2 results in enhanced DNA binding and transactivation by these transcription factors (By similarity). Heterodimer of an alpha and a beta subunit. Interacts with HIVEP3 (By similarity). The alpha subunit binds DNA as a monomer and through the Runt domain. DNA-binding is increased by heterodimerization. Interacts with XRCC6 (Ku70) and XRCC5 (Ku80). Interacts with MYST3 and MYST4.