X连锁生长调控因子GCFX抗体
规格:1mg/1ml
英文名: SHOX
别名: GCFX; Growth control factor, X linked; Homo sapiens shox gene, alternatively spliced products, complete cds; PHOG; Pseudoautosomal homeobox containing osteogenic; Pseudoautosomal homeobox containing o
分子量: 32kDa
储存液:0.01M TBS(pH7.4) with 1% BSA, 0.03% Proclin300 and 50% Glyce
克隆类型:Polyclonal
亚型:IgG
纯化方法:affinity purified by Protein A
**原:KLH conjugated synthetic peptide derived from human SHOX
交叉反应:Human, Mouse, Rat, Dog, Pig, Cow, Zebrafish,
细胞定位:细胞核
X连锁生长调控因子GCFX抗体产品介绍:background: This gene belongs to the paired homeobox family and is located in the pseudoautosomal region 1 (PAR1) of X and Y chromosomes. Defects in this gene are associated with idiopathic growth retardation and in the short stature phenotype of Turner syndrome patients. This gene is highly conserved across species from mammals to fish to flies. Alternatively spliced transcript variants encoding different isoforms have been noted for this gene. [provided by RefSeq, Jul 2008] Function: Controls fundamental aspects of growth and development. Subcellular Location: Nucleus. Tissue Specificity: SHOXA is expressed in skeletal muscle, placenta, pancreas, heart and bone marrow fibroblast and SHOXB is highly expressed in bone marrow fibroblast followed by kidney and skeletal muscle. SHOXB is not expressed in brain, kidney, liver and lung. Highly expressed in osteogenic cells. DISEASE: Defects in SHOX are the cause of Leri-Weill dyschondrosteosis X连锁生长调控因子GCFX抗体(LWD) [MIM:127300]. LWD is a dominantly inherited skeletal dysplasia characterized by moderate short stature predominantly because of short mesomelic limb segments. It is often associated with the Madelung deformity of the wrist, comprising bowing of the radius and dorsal dislocation of the distal ulna. Defects in SHOX are a cause of Langer mesomelic dysplasia (LMD) [MIM:249700]. LMD is an autosomal recessive rare skeletal dysplasia characterized by severe short stature owing to shortening and maldevelopment of the mesomelic and rhizomelic segments of the limbs. Associated malformations are rarely reported and intellect is normal in all affected subjects reported to date. Defects in SHOX are a cause of idiopathicX连锁生长调控因子GCFX抗体 short stature (ISS) [MIM:300582]. Idiopathic short stature is usually defined as a height below the third percentile for chronological age or minus 2 standard deviations of national height standards in the absence of specific causative disorders. Similarity: Belongs to the paired homeobox family. Bicoid subfamily. Contains 1 homeobox DNA-binding domain. Database links: Entrez Gene: 615159 Cow Entrez Gene: 491706 Dog Entrez Gene: 6473 Human Omim: 312865 Human SwissProt: O15266 Human Unigene: 105932 Human Important Note: This product as supplied is intended for research use only, not for use in human, therapeutic or diagnostic applications.
X连锁生长调控因子GCFX抗体产品应用:WB=1:100-500 ELISA=1:500-1000 IHC-P=1:100-500 IHC-F=1:100-500 ICC=1:100-500 IF=1:100-500 (石蜡切片需做抗原修复) not yet tested in other applications. optimal dilutions/concentrations should be determined by the end user.
研究领域:细胞生物 发育生物学 信号转导 生长因子和** 表观遗传学
储存条件: Store at -20 °C for one year. Avoid repeated freeze/thaw cycles.
来源: Rabbit
外观: Lyophilized or Liquid