FITC标记的HHG2抗体-抗体-抗体-生物在线
FITC标记的HHG2抗体

FITC标记的HHG2抗体

商家询价

产品名称: FITC标记的HHG2抗体

英文名称: Anti-IHH/FITC

产品编号: HZ-

产品价格: null

产品产地: 中国/上海

品牌商标: HZbscience

更新时间: 2023-08-17T10:24:20

使用范围: IF=1:50-200

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 Rabbit Anti-IHH/FITC Conjugated antibody 

FITC标记的HHG2抗体

 

英文名称 Anti-IHH/FITC
中文名称 FITC标记的HHG2抗体
别    名 HHG2; Indian Hedgehog; CENPB_HUMAN; Major centromere autoantigen B; Centromere protein B; CENP-B.   
规格价格 100ul/2980元 购买        大包装/询价
说 明 书 100ul  
研究领域 细胞生物  免疫学  信号转导  干细胞  转录调节因子  细胞表面分子  
抗体来源 Rabbit
克隆类型 Polyclonal
交叉反应 Human, Mouse, Rat, Dog, Pig, Horse, Rabbit, 
产品应用 IF=1:50-200  
not yet tested in other applications.
optimal dilutions/concentrations should be determined by the end user.
分 子 量 45kDa
性    状 Lyophilized or Liquid
浓    度 1mg/ml
免 疫 原 KLH conjugated synthetic peptide derived from human IHH
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 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.
产品介绍 background:
Ihh is an intercellular signalling peptide essential for a variety of patterning events during development. It binds to the patched (PTC) receptor, which functions in association with smoothened (SMO), to activate the transcription of target genes. Ihh induces the expression of parathyroid hormone-related protein (PTHRP) and is implicated in endochondral ossification, possibly regulating the balance between growth and ossification of the developing bones.

Function:
Intercellular signal essential for a variety of patterning events during development. Binds to the patched (PTC) receptor, which functions in association with smoothened (SMO), to activate the transcription of target genes. Implicated in endochondral ossification: may regulate the balance between growth and ossification of the developing bones. Induces the expression of parathyroid hormone-related protein (PTHRP) (By similarity).

Subcellular Location:
Indian hedgehog protein N-product: Cell membrane; Lipid-anchor; Extracellular side (By similarity). Note=The N-terminal peptide remains associated with the cell surface (By similarity).
Indian hedgehog protein C-product: Secreted, extracellular space (By similarity). Note=The C-terminal peptide diffuses from the cell (By similarity). 

Tissue Specificity:
Expressed in embryonic lung, and in adult kidney and liver.

Post-translational modifications:
The C-terminal domain displays an autoproteolysis activity and a cholesterol transferase activity. Both activities result in the cleavage of the full-length protein and covalent attachment of a cholesterol moiety to the C-terminal of the newly generated N-terminal fragment (N-product). The N-product is the active species in both local and long-range signaling, whereas the C-product has no signaling activity (By similarity). 
Cholesterylation is required for N-product targeting to lipid rafts and multimerization (By similarity). 
Palmitoylated. N-palmitoylation is required for N-product multimerization and full activity. 

DISEASE:
Defects in IHH are the cause of brachydactyly type A1 (BDA1) [MIM:112500]. BDA1 is an autosomal dominant disorder characterized by middle phalanges of all the digits rudimentary or fused with the terminal phalanges. The proximal phalanges of the thumbs and big toes are short. 
Defects in IHH are a cause of acrocapitofemoral dysplasia (ACFD) [MIM:607778]. ACFD is a disorder characterized by short stature of variable severity with postnatal onset. The most constant radiographic abnormalities are observed in the tubular bones of the hands and in the proximal part of the femur. Cone-shaped epiphyses or a similar epiphyseal configuration with premature epimetaphyseal fusion result in shortening of the skeletal components involved. Cone-shaped epiphyses were also present to a variable extent at the shoulders, knees, and ankles. 

Similarity:
Belongs to the hedgehog family.

Database links:
UniProtKB/Swiss-Prot: P07199.2

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

 

IHH是细胞间信号肽,在发育过程中对各种图案化事件至关重要。它结合了补丁(PTC)受体,其功能与平滑(SMO),激活靶基因的转录。IHH诱导甲状旁腺相关蛋白(PTHRP)的表达并参与软骨内骨化,可能调节发育骨的生长和骨化之间的平衡。