产品名称：Anti-Adenylate kinase 2/FITC抗体

Anti-Adenylate kinase 2/FITC抗体

产品编号YB-6875R-FITC

英文名称Anti-Adenylate kinase 2/FITC

中文名称FITC标记的腺苷酸激酶2抗体

别    名mitochondrial; Adenylate kinase 2; Adenylate kinase isoenzyme 2; ADK2; AK 2; ak2; ATP AMP transphosphorylase; ATP AMP transphosphorylase; ATP-AMP transphosphorylase 2; EC 2.7.4.3; KAD2_HUMAN.

Anti-Adenylate kinase 2/FITC抗体

说 明 书100ul  

研究领域细胞生物  信号转导  转录调节因子  激酶和磷酸酶  线粒体  

抗体来源Rabbit

克隆类型Polyclonal

交叉反应Human, Mouse, Rat, Dog, Pig, Cow, Sheep,

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26kDa

细胞定位细胞膜 线粒体

性    状Lyophilized or Liquid

浓    度1mg/ml

免 疫 原KLH conjugated synthetic peptide derived from human Adenylate kinase 2/AK2

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

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.

Anti-Adenylate kinase 2/FITC抗体

产品介绍background:

Adenylate kinases are involved in regulating the adenine nucleotide composition within a cell by catalyzing the reversible transfer of phosphate groups among adenine nucleotides. Three isozymes of adenylate kinase, namely 1, 2, and 3, have been identified in vertebrates; this gene encodes isozyme 2. Expression of these isozymes is tissue-specific and developmentally regulated. Isozyme 2 is localized in the mitochondrial intermembrane space and may play a role in apoptosis. Mutations in this gene are the cause of reticular dysgenesis. Alternate splicing results in multiple transcript variants. Pseudogenes of this gene are found on chromosomes 1 and 2.[provided by RefSeq, Nov 2010]

Function:

Catalyzes the reversible transfer of the terminal phosphate group between ATP and AMP. This small ubiquitous enzyme involved in energy metabolism and nucleotide synthesis that is essential for maintenance and cell growth. Plays a key role in hematopoiesis.

Subunit:

Monomer.

Subcellular Location:

Mitochondrion intermembrane space.

Tissue Specificity:

Present in most tissues. Present at high level in heart, liver and kidney, and at low level in brain, skeletal muscle and skin. Present in thrombocytes but not in erythrocytes, which lack mitochondria. Present in all nucleated cell populations from blood, while AK1 is mostly absent. In spleen and lymph nodes, mononuclear cells lack AK1, whereas AK2 is readily detectable.Anti-Adenylate kinase 2/FITC抗体These results indicate that leukocytes may be susceptible to defects caused by the lack of AK2, as they do not express AK1 in sufficient amounts to compensate for the AK2 functional deficits (at protein level).

DISEASE:

Defects in AK2 are the cause of reticular dysgenesis (RDYS) [MIM:267500]; also known as aleukocytosis. RDYS is the most severe form of inborn severe combined immunodeficiencies (SCID) and is characterized by absence of granulocytes and almost complete deficiency of lymphocytes in peripheral blood, hypoplasia of the thymus and secondary lymphoid organs, and lack of innate and adaptive humoral and cellular immune functions, leading to fatal septicemia within days after birth. In bone marrow of individuals with reticular dysgenesis, myeloid differentiation is blocked at the promyelocytic stage, whereas erythro- and megakaryocytic maturation is generally normal.In addition, affected newborns have bilateral sensorineural deafness. Defects may be due to its absence in leukocytes and inner ear, in which its absence can not be compensated by AK1.

Similarity:

Belongs to the adenylate kinase family. AK2 subfamily.

Anti-Adenylate kinase 2/FITC抗体Database links:

Entrez Gene: 204 Human

Entrez Gene: 11637 Mouse

Entrez Gene: 24184 Rat

Omim: 103020 Human

SwissProt: P54819 Human

SwissProt: Q9WTP6 Mouse

SwissProt: P29410 Rat

Unigene: 470907 Human

Unigene: 29460 Mouse

Unigene: 3421 Rat

Important Note:

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