Anti-Noggin抗体(ab56497)

概述

性能

应用

Our Abpromise guarantee covers the use of ab56497 in the following tested applications.

The application notes include recommended starting dilutions; optimal dilutions/concentrations should be determined by the end user.

应用 Ab评论 说明
WB
  • 应用说明WB: Use at a concentration of 1-5 µg/ml.

    This antibody has only been tested in WB against the recombinant fragment used as immunogen. We have no data on the detection of endogenous protein.

    Not yet tested in other applications.
    Optimal dilutions/concentrations should be determined by the end user.
  • 靶标

    • 功能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.
    • 疾病相关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.
    • 序列相似性Belongs to the noggin family.
    • 细胞定位Secreted.
    • Information by UniProt
    • 数据库链接
    • 别名
      • Nog antibody
      • NOGG_HUMAN antibody
      • Noggin antibody
      • SYM 1 antibody
      • SYM1 antibody
      • Symphalangism 1 (proximal) antibody
      • Synostoses (multiple) syndrome 1 antibody
      • SYNS 1 antibody
      • SYNS1 antibody
      see all

    Anti-Noggin antibody 图像

    • Western blot against tagged recombinant protein immunogen using ab56497 Noggin antibody at 1ug/ml. Predicted band size of immunogen is 49 kDa

    Anti-Noggin antibody (ab56497)参考文献

    ab56497 has not yet been referenced specifically in any publications.

    Product Wall

    There is no rat protein sequence available only a fragment in UniProt which has evidence on transcript level. Comparing Q13253 NOGG_HUMAN Noggin and the rat fragment Q62809 NOGG_RAT, the identities are 142/144 (98%). Therefore, it is likely that the...

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    Please note: All products are "FOR RESEARCH USE ONLY AND ARE NOT INTENDED FOR DIAGNOSTIC OR THERAPEUTIC USE"