Anti-PAPSS2抗体上海钰博生物科技有限公司

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产品名称: Anti-PAPSS2抗体
英文名称: PAPSS2
抗体货号: YB--9030R
产品规格: 20ul/50ul/100ul/200ul
级    别: 分析纯, 见说明书
产品产地: 中国/美国
品牌商标: Ybscience
价    格: 1580元
抗原: KLH conjugated synthetic peptide derived from human PAPSS2:351-450/614
抗原来源: Rabbit
抗体来源: Rabbit
适用物种: Human, Mouse, Rat, Dog, Cow, Horse, Rabbit,
见说明书conjugate:见说明书
Isotype:见说明书
应用范围: 科研使用
更新时间: 2019/3/10 13:06:00
详细资料:  实验方法技术资料
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使用范围:仅限科研使用,不能应用于临床
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产品详细描述

 Anti-PAPSS2抗体

产品编号 YB-9030R
英文名称 PAPSS2
中文名称 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/1580元 购买    200ul/2480元 购买    大包装/询价
说 明 书 100ul  200ul
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 WB=1:500-2000 ELISA=1:500-1000 IHC-P=1:400-800 IHC-F=1:400-800 IF=1:50-200 (石蜡切片需做抗原修复) 
not yet tested in other applications.
optimal dilutions/concentrations should be determined by the end user.
分 子 量 68kDa
细胞定位 细胞浆 
性    状 Lyophilized or Liquid
浓    度 1mg/ml
免 疫 原 KLH conjugated synthetic peptide derived from human PAPSS2:351-450/614 
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.
PubMed PubMed
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.

SWISS:
O95340

Gene ID:
9060

Database links:

Entrez Gene: 9060 Human

Entrez Gene: 23972 Mouse

Entrez Gene: 294103 Rat

Omim: 603005 Human

SwissProt: O95340 Human

SwissProt: O88428 Mouse

Unigene: 524491 Human



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

生物在线声明:以上所展示的信息由企业自行提供,内容的真实性、准确性和合法性由发布企业负责。生物在线对此不承担任何保证责任。

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