本公司经销Adenylate kinase 2/AK2，腺苷酸激酶1抗体，克隆类型为polyclonal，宿主来源是Rabbit，Adenylate kinase 2/AK2腺苷酸激酶1抗体可应用于WB、elisa、IP、IF、IHC等实验，欢迎垂询订购！

货号：BY-6875R
英文名称：Anti-Adenylate kinase 2/AK2
中文名称：腺苷酸激酶1抗体
其他名称：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.
抗体来源：Rabbit
克隆类型：polyclonal
蛋白分子量：predicted molecular weight: 26kDa
纯化方法：affinity purified by Protein A
交叉反应：hu, mo, rat, cow
产品介绍：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. 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.