产品中心
当前位置：首页>产品中心Anti-PAPSS2/Cy5.5
货号： bs-9030R-Cy5.5 基本售价： 2980.0 元 规格： 100ul
产品信息
- 产品编号
- bs-9030R-Cy5.5
- 英文名称
- Anti-PAPSS2/Cy5.5
- 中文名称
- Cy5.5标记的腺苷酸硫酸激酶2抗体
- 别 名
- ATPSK2; PAPS synthetase 2; PAPSS 2; SK2; Sulfurylase kinase 2; 3 phosphoadenosine 5 phosphosulfate synthase 2.
- 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
- 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
- IF=1:50-200
not yet tested in other applications.
optimal dilutions/concentrations should be determined by the end user.
- 分 子 量
- 68kDa
- 性 状
- Lyophilized or Liquid
- 浓 度
- 1mg/ml
- 免 疫 原
- KLH conjugated synthetic peptide derived from human PAPSS2
- 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:
PAPSS2 is one of the two PAPS synthetases. Three-prime-phosphoadenosine 5-prime-phosphosulfate (PAPS) is the sulfate donor cosubstrate for all sulfotransferase (SULT) enzymes. SULTs catalyze the sulfate conjugation of many endogenous and exogenous compounds, including drugs and other xenobiotics. In humans, PAPS is synthesized from adenosine 5-prime triphosphate (ATP) and inorganic sulfate by 2 isoforms, PAPSS1 and PAPSS2.
Bifunctional 3-phosphoadenosine 5-phosphosulfate synthetases (PAPS synthetase or PAPSS), also designated sulfurylase kinase (SK), are important for sulfate assimilation in the sulfur metabolism pathway. PAPPS proteins are bifunctional enzymes with APS kinase and ATP sulfurylase activity, which mediate two steps in the sulfate activation pathway. The PAPSS proteins belong to the APS kinase family and to the sulfate adenylyltransferase family of proteins. In mammals, PAPSS proteins are the sole source of sulfate. During postnatal growth, PAPSS proteins may play a role in skeletogenesis. Defects in the PAPSS2 gene can cause the Pakistani type of spondyloepimetaphyseal dysplasia (SEMD), an autosomal recessive form of SEMD characterized by short, bowed limbs, enlarged knee joints and mild brachydactyly.
Function:
Bifunctional enzyme with both ATP sulfurylase and APS kinase activity, which mediates two steps in the sulfate activation pathway. The first step is the transfer of a sulfate group to ATP to yield adenosine 5-phosphosulfate (APS), and the second step is the transfer of a phosphate group from ATP to APS yielding 3-phosphoadenylylsulfate (PAPS: activated sulfate donor used by sulfotransferase). In mammals, PAPS is the sole source of sulfate; APS appears to be only an intermediate in the sulfate-activation pathway. May have a important role in skeletogenesis during.
Tissue Specificity:
Expressed in cartilage and adrenal gland.
DISEASE:
Defects in PAPSS2 are the cause of spondyloepimetaphyseal dysplasia Pakistani type (SEMD-PA) [MIM:612847]. A bone disease characterized by epiphyseal dysplasia with mild metaphyseal abnormalities. Clinical features include short stature evidenced at birth, short and bowed lower limbs, mild brachydactyly, kyphoscoliosis, abnormal gait, enlarged knee joints. Some patients may manifest premature pubarche and hyperandrogenism associated with skeletal dysplasia and short stature.
Similarity:
In the N-terminal section; belongs to the APS kinase family.
In the C-terminal section; belongs to the sulfate adenylyltransferase family.
Database links:Entrez Gene: 536409Cow
Entrez Gene: 9060Human
Entrez Gene: 23972Mouse
Entrez Gene: 294103Rat
Omim: 603005Human
SwissProt: O95340Human
SwissProt: O88428Mouse
Unigene: 524491Human
Important Note:
This product as supplied is intended for research use only, not for use in human, therapeutic or diagnostic applications.