FITC标记的生长分化因子6抗体-抗体-抗体-生物在线
上海沪震实业有限公司
FITC标记的生长分化因子6抗体

FITC标记的生长分化因子6抗体

商家询价

产品名称： FITC标记的生长分化因子6抗体

英文名称： Anti-GDF6/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： ICC=1:50-200 IF=1:50-200

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Rabbit Anti-GDF6/FITC Conjugated antibody

FITC标记的生长分化因子6抗体

 

英文名称 Anti-GDF6/FITC
中文名称 FITC标记的生长分化因子6抗体
别    名 bmp 13; bmp13; bmp-13; Cartilage Derived Morphogenetic Protein 2; cdmp 2; CDMP2; gdf 6; GDF16 ; Growth differentiation factor 6; Growth/differentiation factor 6; GDF6_HUMAN.  
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  神经生物学  信号转导  生长因子和激素  
抗体来源 Rabbit
克隆类型 Polyclonal
交叉反应 Human, Mouse, Rat, Dog, Cow, Horse, Rabbit, 
产品应用 ICC=1:50-200 IF=1:50-200  
not yet tested in other applications.
optimal dilutions/concentrations should be determined by the end user.
分 子 量 14kDa
性    状 Lyophilized or Liquid
浓    度 1mg/ml
免 疫 原 KLH conjugated synthetic peptide derived from human GDF6
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:
Growth/differentiation factors (GDFs) are members of the TGF superfamily (1,2). Members of the TGF superfamily are involved in embryonic development and adult tissue homeostasis (1). GDF-1 expression is almost exclusively restricted to the central nervous system and mediates cell differentiation events during embryonic development (3). Neither GDF-3 (Vgr-2) nor GDF-9 contains the conserved cysteine residue which is found in most other TGF superfamily members. GDF-3 is detectable in bone marrow, spleen, thymus and adipose tissue, whereas GDF-9 has only been detected in ovary (4). GDF-5 (also designated CDMP-1) has been shown to induce activation of plasminogen activator, thereby inducing angiogenesis. It is predominantly expressed in long bones during fetal embryonic development and is involved in bone formation. (5). GDF-5 mutations have been identified in mice with the mutation brachypodism (bp), a mutation which affects the length and number of bones in limbs (6). GDF-6 and GDF-7 are closely related to GDF-5 (6). GDF-8 has been shown to be a negative regulator of skeletal muscle mass (1).

Function:
GDF6 (Growth/differentiation factor 6) is expressed in hypertrophic chondrocytes during embryonic development of long bones and is required for normal formation of bones and joints in the limbs, skull, and axial skeleton. It plays a key role in establishing boundaries between skeletal elements during development. The functional form of GDF6 is a disulfide-linked homodimer of two 120 amino acid polypeptide chains obtained by proteolytic processing of a biologically inactive precursor protein.

Subunit:
Homodimer; disulfide-linked

Subcellular Location:
Secreted

DISEASE:
Defects in GDF6 are the cause of Klippel-Feil syndrome type 1 (KFS1) [MIM:118100]. A skeletal disorder characterized by congenital fusion of cervical vertebrae. It is due to a failure in the normal segmentation of vertebrae during the early weeks of fetal development. The clinical triad consists of short neck, low posterior hairline, and limited neck movement. Deafness is a well-known feature of KFS and may be of sensorineural, conductive, or mixed type. 
Note=A chromosomal aberration involving GDF6 has been found in a patient with Klippel-Feil syndrome (KFS). Paracentric inv(8)(q22;2q23.3). 
Defects in GDF6 are the cause of microphthalmia isolated type 4 (MCOP4) [MIM:613094]. A disorder of eye formation, ranging from small size of a single eye to complete bilateral absence of ocular tissues. Ocular abnormalities like opacities of the cornea and lens, scaring of the retina and choroid, cataract and other abnormalities like cataract may also be present. 

Similarity:
Belongs to the TGF-beta family.

Database links:
UniProtKB/Swiss-Prot: Q6KF10.1

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

生长/分化因子（GDFs）是TGF超家族（1,2）的成员。TGF超家族成员参与胚胎发育和组织稳态（1）。GDF-1的表达几乎只限于中枢神经系统，在胚胎发育过程中介导细胞分化事件（3）。GDF -3（VGR-2）和GDF-9均不含在大多数其他TGF超家族成员中发现的保守半胱氨酸残基。GDF-3可在骨髓、脾脏、胸腺和脂肪组织中检测到，而GDF－9仅在卵巢（4）中检测到。GDF 5（也被指定为CDMP-1）已被诱导诱导纤溶酶原激活剂，从而诱导血管生成。它在胚胎发育过程中主要在长骨中表达，并参与骨形成。（5）。GDF-5突变已经在具有突变短型(bp)的小鼠中鉴定，该突变影响四肢骨骼的长度和数量(6)。GDF 6和GDF 7与GDF 5（6）密切相关。GDF－8已被证明是骨骼肌质量的负调节因子（1）。