Anti-AK2 antibody (AA37182)

$187.00


Alternative names Adenylate kinase 2, mitochondrial, AK 2, EC 2.7.4.3, ATP-AMP transphosphorylase 2, ATP:AMP phosphotransferase, Adenylate monophosphate kinase, AK2, ADK2, KAD2_HUMAN, P54819, 204
Source 232
Species Reactivity Human
Applications IHC, WB
Host Rabbit
Class Polyclonal
Conjugated Unconjugated


Description

Product name Anti-AK2 antibody (AA37182)
Catalog number AA37182
Brand family Ango
Product type Primary Antibody
Antibody Alternative Names Adenylate kinase 2, mitochondrial antibody, AK 2 antibody, EC 2.7.4.3 antibody, ATP-AMP transphosphorylase 2 antibody, ATP:AMP phosphotransferase antibody, Adenylate monophosphate kinase antibody, AK2 antibody, ADK2 antibody, KAD2_HUMAN antibody


Biological Information

Concentration 1 mg/ml
Isotype IgG
Purification method Purified by affinity chromatography


Target

Target Name AK2
SwissProt ID Link P54819
Function Catalyzes the reversible transfer of the terminal phosphate group between ATP and AMP. Plays an important role in cellular energy homeostasis and in adenine nucleotide metabolism. Adenylate kinase activity is critical for regulation of the phosphate utilization and the AMP de novo biosynthesis pathways. Plays a key role in hematopoiesis.
Subcellular Location Mitochondrion intermembrane space.
Involvement in Disease Reticular dysgenesis: Most severe form of inborn severe combined immunodeficiencies 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. The disease is caused by mutations affecting the gene represented in this entry.
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).
Subunit Structure Monomer.
Sequence Similarities Belongs to the adenylate kinase family. AK2 subfamily.


Poduct Presentation

Form Liquid
Preservative 0.02% (w/v) sodium azide
Storage buffer PBS with 50% glycerol, pH 7.3


Storage and Shipping Information

Storage Store at -20 C. Avoid multiple freeze/thaw cycles.