Name: Noggin mouse mAb
SKU: WM0677
Price: 268 USD
Availability: InStock

Product details

Background:
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 mu

Specifications

Size	50μL/100μL
Target	Noggin
Reactivity
Applications	WB, ICC
MW(Calculated)
MW(Observed)	26kD
Host Species	Mouse
Isotype
Conjugate/Modification	Unmodified
Recommended Dilution Ratio	WB 1:1000; ICC 1:100
Formulation	Liquid in PBS containing 50% glycerol, 0.5% BSA and 0.02% sodium azide.
Purification	The antibody was affinity-purified from mouse ascites by affinity-chromatography using epitope-specific immunogen.
Storage	-15°C to -25°C/1 year(Do not lower than -25°C)
Concentration
Clonality	Monoclonal
Clone Number	10H5
Immunogen	Purified recombinant human Noggin protein fragments expressed in E.coli.
Specificity	Transfected Only.
Gene Name	nog
Protein Name
Other Name	NOG; / NOGG_HUMAN; / Noggin; / SYM 1; / SYM1; / Symphalangism 1; / proximal; / Synostoses; / multiple; / syndrome 1; / SYNS 1; / SYNS1.
Species	Human
Gene ID	9241
Uniprot	Q13253,
Species.1	Mouse
Gene ID.1	18121
Uniprot.1	P97466
Species.2
Gene ID.2
Uniprot.2
Cellular Localization	Secreted.
Tissue Expression	Placenta,Prostate,Temporal cortex,
Signaling_pathway	Environmental Information Processing >> Signal transduction >> TGF-beta signaling pathway
Research Areas	>>TGF-beta signaling pathway
Function	Disease: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.,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.,Disease: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.,Disease: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.,Disease: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.,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.,similarity:Belongs to the noggin family.,subunit:Homodimer; disulfide-linked.,

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Noggin mouse mAb

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Noggin mouse mAb

Product details

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