Product Name	Anti-Sclerostin/AF594
Chinese Name	AF594标记的骨形态发生抑制蛋白SOST抗体
Alias	BEER; Cortical hyperostosis with syndactyly; Sclerosteosis; Sclerostin; SOST; SOST_HUMAN; VBCH.
Research Area	Signal transduction  Stem cells
Immunogen Species	Rabbit
Clonality	Polyclonal
React Species	Rat,  (predicted: Human, Mouse, Dog, Pig, Horse, Rabbit, )
Applications	IF=1:50-200  not yet tested in other applications. optimal dilutions/concentrations should be determined by the end user.
Molecular weight	21kDa
Form	Lyophilized or Liquid
Concentration	1mg/ml
immunogen	KLH conjugated synthetic peptide derived from human Sclerostin
Lsotype	IgG
Purification	affinity purified by Protein A
Storage Buffer	0.01M TBS(pH7.4) with 1% BSA, 0.03% Proclin300 and 50% Glycerol.
Storage	Store at -20 °C for one year. Avoid repeated freeze/thaw cycles. The lyophilized antibody is stable at room temperature for at least one month and for greater than a year when kept at -20°C. When reconstituted in sterile pH 7.4 0.01M PBS or diluent of antibody the antibody is stable for at least two weeks at 2-4 °C.
Product Detail	background: Negative regulator of bone growth.Sclerostin (SOST) is a bone morphogenetic protein (BMP) antagonist, leading to the activation of BMP signaling. It negatively regulates the formation of bone by repressing the differentiation and/or function of osteoblasts induced by BMPs. It has been shown that Sclerostin binds BMP-5, -6, and -7 with high affinity and BMP-2 and -4 with low affinity. The noggin-sclerostin protein complex represents a novel mechanism for the fine-tuning of BMP activity in bone homeostasis. Evidence is accumulating that one of the important mechanisms of bone regulation by sclerostin is the modulation of Wnt/Beta-catenin signaling. Sclerostin also rapidly activated ERK-1/2 MAPK signaling, indicating the involvement of additional signaling pathways. Function: Negative regulator of bone growth that acts through inhibition of Wnt signaling and bone formation. Subunit: Interacts with LRP4 (via the extracellular domain); the interaction facilitates the inhibition of Wnt signaling. Interacts with LRP5 (via the first two YWTD-EGF repeat domains); the interaction inhibits Wnt-mediated signaling. Interacts with LRP6. Subcellular Location: Secreted. Tissue Specificity: Widely expressed at low levels with highest levels in bone, cartilage, kidney, liver, bone marrow and primary osteeoblasts differentiated for 21 days. DISEASE: Defects in SOST are the cause of sclerosteosis type 1 (SOST1) [MIM:269500]. An autosomal recessive sclerosing bone dysplasia characterized by a generalized hyperostosis and sclerosis leading to a markedly thickened skull, with mandible, ribs, clavicles and all long bones also being affected. Due to narrowing of the foramina of the cranial nerves, facial nerve palsy, hearing loss and atrophy of the optic nerves can occur. Sclerosteosis is clinically and radiologically very similar to van Buchem disease, mainly differentiated by hand malformations and a large stature in sclerosteosis patients. Defects in SOST are a cause of van Buchem disease (VBCH) [MIM:239100]. An autosomal recessive sclerosing bone dysplasia characterized by endosteal hyperostosis of the mandible, skull, ribs, clavicles, and diaphyses of the long bones. Affected patients present a symmetrically increased thickness of bones, most frequently found as an enlarged jawbone, but also an enlargement of the skull, ribs, diaphysis of long bones, as well as tubular bones of hands and feet. The clinical consequence of increased thickness of the skull include facial nerve palsy causing hearing loss, visual problems, neurological pain, and, very rarely, blindness as a consequence of optic atrophy. Serum alkaline phosphatase levels are elevated. Note=A 52 kb deletion downstream of SOST results in SOST transcription suppression causing van Buchem disease. Defects in SOST are a cause of craniodiaphyseal dysplasia autosomal dominant (CDD) [MIM:122860]. A severe bone dysplasia characterized by massive generalized hyperostosis and sclerosis, especially involving the skull and facial bones. The sclerosis is so severe that the resulting facial distortion is referred to as 'leontiasis ossea' (leonine faces) and the bone deposition results in progressive stenosis of craniofacial foramina. Respiratory obstruction due to choanal stenosis compromises the clinical outcomes of affected patients. Note=Heterozygous mutations located in the secretion signal of the SOST gene prevent sclerostin secretion and can be responsible for craniodiaphyseal dysplasia. Similarity: Belongs to the sclerostin family. Contains 1 CTCK (C-terminal cystine knot-like) domain. Database links: Entrez Gene: 50964 Human Entrez Gene: 74499 Mouse Omim: 605740 Human SwissProt: Q9BQB4 Human SwissProt: Q99P68 Mouse Unigene: 349204 Human Unigene: 265602 Mouse 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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