腺苷酸硫酸激酶2抗体
规格：1mg/1ml
英文名： PAPSS2
别名： ATPSK2; PAPS synthetase 2; PAPSS 2; SK2; Sulfurylase kinase 2; 3' phosphoadenosine 5' phosphosulfate synthase 2.
分子量： 68kDa
储存液：0.01M TBS(pH7.4) with 1% BSA, 0.03% Proclin300 and 50% Glyce
克隆类型：Polyclonal
亚型：IgG
纯化方法：affinity purified by Protein A
**原：KLH conjugated synthetic peptide derived from human PAPSS2
交叉反应：Human, Mouse, Rat, Dog, Cow, Horse, Rabbit,
细胞定位：
腺苷酸硫酸激酶2抗体产品介绍：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.腺苷酸硫酸激酶2抗体 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 腺苷酸硫酸激酶2抗体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.
腺苷酸硫酸激酶2抗体产品应用：WB=1:100-500 ELISA=1:500-1000 IHC-P=1:100-500 IHC-F=1:100-500 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.
研究领域：细胞生物 **学
储存条件： Store at -20 °C for one year. Avoid repeated freeze/thaw cycles.
来源： Rabbit
外观： Lyophilized or Liquid