腺苷酸激酶2抗体

腺苷酸激酶2抗体

规格：1mg/1ml

英文名： Adenylate kinase 2

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.

分子量： 26kDa

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 Adenylat

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

腺苷酸激酶2抗体细胞定位：细胞浆 细胞膜 线粒体

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.腺苷酸激酶2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腺苷酸激酶2抗体 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. Gene ID: 204 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.

腺苷酸激酶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=1:100-500 （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  信号转导  转录调节因子  激酶和磷酸酶  线粒体  

储存条件： Store at -20 °C for one year. Avoid repeated freeze/thaw cycles.

来源： Rabbit

外观： Lyophilized or Liquid

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