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品种反应性 主要应用 宿主 格式 抗体类型 M WB Rb Affinity Purified Polyclonal Antibody 描述 产品目录编号 AB5729 Replaces MABD184 品牌系列 Chemicon®
商名 - Chemicon
描述 Anti-Noggin Antibody 产品信息 格式 Affinity Purified 演示 Affinity purified immunoglobulin. Liquid. 应用 应用 Detect Noggin using this Anti-Noggin Antibody validated for use in WB. 主要应用 - Western Blotting
应用说明 Western blot: 0.5-1.0 μg/mL using ECL on recombinant mature mouse Noggin/Fc. The antibody reacts with the predicted 55-60 kDa recombinant protein.
Optimal working dilutions must be determined by the end user.生物信息 免疫原品种 Synthetic peptide from mouse Noggin. 浓缩 Please refer to the Certificate of Analysis for the lot-specific concentration. 宿主 Rabbit 特异性 Noggin, mouse. 品种反应性 Mouse 抗体类型 Polyclonal Antibody Entrez基因编号 - NM_005450.2
Entrez基因汇总 The secreted polypeptide, encoded by this gene, binds and inactivates members of the transforming growth factor-beta (TGF-beta) superfamily signaling proteins, such as bone morphogenetic protein-4 (BMP4). By diffusing through extracellular matrices more efficiently than members of the TGF-beta superfamily, this protein may have a principal role in creating morphogenic gradients. The protein appears to have pleiotropic effect, both early in development as well as in later stages. It was originally isolated from Xenopus based on its ability to restore normal dorsal-ventral body axis in embryos that had been artificially ventralized by UV treatment. The results of the mouse knockout of the ortholog suggest that it is involved in numerous developmental processes, such as neural tube fusion and joint formation. Recently, several dominant human NOG mutations in unrelated families with proximal symphalangism (SYM1) and multiple synostoses syndrome (SYNS1) were identified; both SYM1 and SYNS1 have multiple joint fusion as their principal feature, and map to the same region (17q22) as this gene. All of these mutations altered evolutionarily conserved amino acid residues. The amino acid sequence of this human gene is highly homologous to that of Xenopus, rat and mouse. 基因符号 - noggin
- NOG
- SYNS1
- SYM1
UniProt编号 - Q13253
UniProt汇总 FUNCTION: SwissProt: Q13253 # Essential for cartilage morphogenesis and joint formation. Inhibitor of bone morphogenetic proteins (BMP) signaling which is required for growth and patterning of the neural tube and somite (By similarity).
SIZE: 232 amino acids; 25774 Da
SUBUNIT: Homodimer; disulfide-linked (By similarity).
SUBCELLULAR LOCATION: Secreted.
DISEASE: SwissProt: Q13253 # Defects in NOG are the cause of symphalangism proximal syndrome (SYM1) [MIM:185800]. SYM1 is characterized by the hereditary absence of the proximal interphalangeal (PIP) joints (Cushing symphalangism). Severity of PIP joint involvement diminishes towards the radial side. Distal interphalangeal joints are less frequently involved and metacarpophalangeal joints are rarely affected whereas carpal bone malformation and fusion are common. In the lower extremities, tarsal bone coalition is common. Conducive hearing loss is seen and is due to fusion of the stapes to the petrous part of the temporal bone. & Defects in NOG are the cause of multiple synostoses syndrome 1 (SYNS1) [MIM:186500]; also known as synostoses, multiple, with brachydactyly/symphalangism-brachydactyly syndrome. SYNS1 is characterized by tubular-shaped (hemicylindrical) nose with lack of alar flare, otosclerotic deafness, and multiple progressive joint fusions commencing in the hand. The joint fusions are progressive, commencing in the fifth proximal interphalangeal joint in early childhood (or at birth in some individuals) and progressing in an ulnar-to-radial and proximal- to-distal direction. With increasing age, ankylosis of other joints, including the cervical vertebrae, hips, and humeroradial joints, develop. & Defects in NOG are the cause of tarsal-carpal coalition syndrome (TCC) [MIM:186570]. TCC is an autosomal dominant disorder characterized by fusion of the carpals, tarsals and phalanges, short first metacarpals causing brachydactyly, and humeroradial fusion. TCC is allelic to SYM1, and different mutations in NOG can result in either TCC or SYM1 in different families. & Defects in NOG are a cause of stapes ankylosis with broad thumb and toes [MIM:184460]. Stapes ankylosis with broad thumb and toes is a congenital autosomal dominant disorder that includes hyperopia, a hemicylindrical nose, broad thumbs, great toes, and other minor skeletal anomalies but lacked carpal and tarsal fusion and symphalangism. & Defects in NOG are the cause of brachydactyly type B2 (BDB2) [MIM:611377]. BDB2 is a subtype of brachydactyly characterized by hypoplasia/aplasia of distal phalanges in combination with distal symphalangism, fusion of carpal/tarsal bones, and partial cutaneous syndactyly.
SIMILARITY: SwissProt: Q13253 ## Belongs to the noggin family.产品使用声明 使用声明 - Unless otherwise stated in our catalog or other company documentation accompanying the product(s), our products are intended for research use only and are not to be used for any other purpose, which includes but is not limited to, unauthorized commercial uses, in vitro diagnostic uses, ex vivo or in vivo therapeutic uses or any type of consumption or application to humans or animals.
储存和货运信息 存储条件 Maintain at +2-8°C in undiluted aliquots for up to 6 months after date of receipt. 包装信息 数量 100 µg
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