货号：AB5729

品牌：Merck Millipore

规格：100Ug

目录价：￥4626.00

市场价格：￥3932.10

会员价格：￥3700.80

金山科研平台，产品价格货期咨询微信：jinshanbio Description: Anti-Noggin Antibody | AB5729 View All» Promotional Text: Special Shipping Offer on Antibodies100% Performance Guaranteed View All» Trade Name: Chemicon (Millipore) View All» Specificity: Noggin, mouse. View All» Immunogen: Synthetic peptide from mouse Noggin. View All» Species Reactivity: Mouse View All» Application Notes: 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. View All» Presentation: Affinity purified immunoglobulin. Liquid. View All» Storage Conditions: Maintain at +2-8°C in undiluted aliquots for up to 6 months after date of receipt. View All» UniProt Number: Q13253 View All» Entrez Gene Number: NM_005450.2 View All» Gene Symbol:

• NOG

• SYNS1

• SYM1

• noggin
  View All» Usage Statement: 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. View All» Key Applications: Western Blotting View All» Entrez Gene Summary: 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. View All» UniProt Summary: 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. View All» Brand Family: Chemicon View All» Product Name: Anti-Noggin Antibody | AB5729 View All» Concentration: 1 mg/mL View All» Antibody Type: Polyclonal Antibody View All» Qty/Pk: 100 µg View All» Target / Localization: Secreted View All» Format: Affinity Purified View All» Host: Rabbit View All»
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