腺苷酸硫酸激酶2抗体

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英文名称  Anti-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.

浓    度  1mg/1ml

规 格  0.2ml/200μg

抗体来源  Rabbit  

克隆类型  polyclonal

交叉反应  Human, Mouse, Rat, Dog, Cow, Horse, Rabbit

产品类型  一抗    

研究领域  细胞生物 免疫学  

蛋白分子量  predicted molecular weight: 68kDa

性    状  Lyophilized or Liquid

腺苷酸硫酸激酶2抗体的功能抗体的主要功能是与抗原（包括外来的和自身的）相结合，从而有效地清除侵入机体内的微生物、寄生虫等异物，抗体（antibody）是一种应答抗原产生的、可与抗原特异性结合的蛋白质。每种抗体与特定的抗原决定基结合。这种结合可以使抗原失活，也可能无效但有时也会对机体造成病理性损害，如抗核抗体、抗双链DNA抗体、抗甲状腺球蛋白抗体等一些自身抗体的产生，对人体可造成危害。

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  Preservative: 15mM Sodium Azide, Constituents: 1% BSA, 0.01M PBS, pH 7.4

产品应用   WB=1:100-500  ELISA=1:500-1000  IP=1:20-100  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.  

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.

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

产品介绍 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

腺苷酸硫酸激酶2抗体Entrez Gene: 294103 Rat

Omim: 603005 Human

SwissProt: O95340 Human

SwissProt: O88428 Mouse

Unigene: 524491 Human