FITC标记的腺苷酸硫酸激酶2抗体
产品名称： FITC标记的腺苷酸硫酸激酶2抗体
英文名称： Anti-PAPSS2/FITC
产品编号： HZ-9030R-FITC
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-PAPSS2/FITC Conjugated antibody
FITC标记的腺苷酸硫酸激酶2抗体
| 产品编号 | bs-9030R-FITC |
| 英文名称 | Anti-PAPSS2/FITC |
| 中文名称 | FITC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: 536409 Cow 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 |
PAPSS2是两种PPAS合成酶之一。三个碱性磷酸腺苷5-磷酸磷酯（PAPs）是所有磺基转移酶（SULTT）酶的硫酸盐供体共基质。SULTS催化许多内源性和外源性化合物的硫酸盐结合，包括药物和其他外来物质。在人类中，PAP由2种亚型、PAPSS1和PAPSS2由腺苷5-磷酸三磷酸（ATP）和无机硫酸盐合成。
双功能3’-磷酸腺苷5’-磷硫酸盐合成酶（PAPS合成酶或PAPSS），也被称为硫酸化酶激酶（SK），在硫代谢途径中对硫酸盐同化具有重要意义。PAPPS蛋白是具有APS激酶和ATP硫酸化酶活性的双功能酶，在硫酸激活途径中介导两个步骤。PAPSS蛋白属于APS激酶家族和硫酸腺苷酰转移酶家族蛋白。在哺乳动物中，PAPSS蛋白是硫酸盐的唯一来源。在出生后的生长过程中，PAPSS蛋白可能在骨骼形成中起作用。PAPSS2基因的缺陷可导致巴基斯坦型脊椎骨干骺端发育不良（SEMD），常染色体隐性形式的SED以短、弯曲的肢体、扩大的膝关节和轻度短指畸形为特征。