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指(趾)关节粘连NOG蛋白抗体
  • 产品货号：
    BN41854R
  • 中文名称：
    指(趾)关节粘连NOG蛋白抗体
  • 英文名称：
    Rabbit anti-Noggin Polyclonal antibody

  • 货号

    产品规格

    售价

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  • BN41854R-50ul

    50ul

    ¥1486.00

    交叉反应：Human,Mouse,Rat(predicted:Chicken,Dog,Pig,Cow,Horse,Rabbit,Sheep) 推荐应用：WB,IHC-P,IHC-F,IF,Flow-Cyt,ELISA

  • BN41854R-100ul

    100ul

    ¥2360.00

    交叉反应：Human,Mouse,Rat(predicted:Chicken,Dog,Pig,Cow,Horse,Rabbit,Sheep) 推荐应用：WB,IHC-P,IHC-F,IF,Flow-Cyt,ELISA

  • BN41854R-200ul

    200ul

    ¥3490.00

    交叉反应：Human,Mouse,Rat(predicted:Chicken,Dog,Pig,Cow,Horse,Rabbit,Sheep) 推荐应用：WB,IHC-P,IHC-F,IF,Flow-Cyt,ELISA

英文名称Noggin
中文名称指(趾)关节粘连NOG蛋白抗体
别    名NOG; NOGG_HUMAN; Noggin; SYM 1; SYM1; Symphalangism 1 (proximal); Synostoses (multiple) syndrome 1; SYNS 1; SYNS1.  
研究领域细胞生物  发育生物学  信号转导  干细胞  
抗体来源Rabbit
克隆类型Polyclonal
交叉反应Mouse, Rat,  (predicted: Human, Chicken, Dog, Pig, Cow, Horse, Rabbit, Sheep, )
产品应用WB=1:500-2000 ELISA=1:5000-10000 IHC-P=1:100-500 IHC-F=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.
分 子 量23kDa
细胞定位分泌型蛋白 
性    状Liquid
浓    度1mg/ml
免 疫 原KLH conjugated synthetic peptide derived from human Noggin:28-130/232 
亚    型IgG
纯化方法affinity purified by Protein A
储 存 液0.01M TBS(pH7.4) with 1% BSA, 0.03% Proclin300 and 50% Glycerol.
保存条件Shipped at 4℃. Store at -20 °C for one year. Avoid repeated freeze/thaw cycles.
PubMedPubMed
产品介绍Noggin is a secreted protein involved at multiple stages of vertebrate embryonic development including neural induction and is known to exert its effects by inhibiting the bone morphogenetic protein (BMP)-signaling pathway. It binds several BMPs with very high (picomolar) affinities, with a marked preference for BMP2 and BMP4 over BMP7. By binding tightly to BMPs, Noggin prevents BMPs from binding their receptors. Noggin binds the bone morphogenetic proteins (BMP) such as BMP-4 and BMP-7, and inhibits BMP signaling by blocking the molecular interfaces of the binding epitopes for both type I and type II receptors. Interaction of BMP and its antagonist Noggin governs various developmental and cellular processes, including embryonic dorsal-ventral axis, induction of neural tissue, formation of joints in the skeletal system and neurogenesis in the adult brain. Noggin plays a key role in neural induction by inhibiting BMP4, along with other TGF-β signaling inhibitors such as chordin and follistatin. Mouse knockout experiments have demonstrated that noggin also plays a crucial role in bone development, joint formation, and neural tube fusion.

Function:
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

Subunit:
Homodimer.

Subcellular Location:
Secreted.

DISEASE:
Defects in NOG are a 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 type 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 (SABTS) [MIM:184460]; also known as Teunissen-Cremers syndrome. SABTS 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:
Belongs to the noggin family.

SWISS:
Q13253

Gene ID:
9241

Database links:
UniProtKB/Swiss-Prot: Q13253.1

Important Note:
This product as supplied is intended for research use only, not for use in human, therapeutic or diagnostic applications.































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