Product Name
AK2, Blocking Peptide
Product Synonym Names
Adenylate kinase 2; mitochondrial; AK 2; 2743 ; ATP-AMP transphosphorylase 2 ; ATP:AMP phosphotransferase ; Adenylate monophosphate kinase ; Adenylate kinase 2; mitochondrial; N-terminally processed ; AK2 ; ADK2
Product Gene Name
AK2 blocking peptide
[Similar Products]
Product Synonym Gene Name
ADK2[Similar Products]
Antibody/Peptide Pairs
AK2 peptide (MBS9227564) is used for blocking the activity of AK2 antibody (MBS9210870)
Research Use Only
For Research Use Only. Not for use in diagnostic procedures.
3D Structure
ModBase 3D Structure for P54819
Specificity
The synthetic peptide sequence used to generate the antibody was selected from the N-term region of human AK2. A 10 to 100 fold molar excess to antibody is recommended. Precise conditions should be optimized for a particular assay.
Form/Format
The synthetic peptide was lyophilized with 100% acetonitrile and is supplied as a powder. Reconstitute with 0.1 ml deionized water for a final concentration of 1 mg/ml.
Cellular Location
Mitochondrion intermembrane space.
Tissue Location
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).
Preparation and Storage
Maintain refrigerated at 2-8 degree C for up to 6 months. For long term storage store at -20 degree C.
Other Notes
Small volumes of AK2 blocking peptide vial(s) may occasionally become entrapped in the seal of the product vial during shipment and storage. If necessary, briefly centrifuge the vial on a tabletop centrifuge to dislodge any liquid in the container`s cap. Certain products may require to ship with dry ice and additional dry ice fee may apply.
Related Product Information for
AK2 blocking peptide
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.
NCBI/Uniprot data below describe general gene information for AK2. It may not necessarily be applicable to this product.
NCBI Accession #
P54819.2
[Other Products]
UniProt Primary Accession #
P54819
[Other Products]
UniProt Secondary Accession #
Q16856; Q5EB54; Q5TIF7; Q8TCY2; Q8TCY3; A8K6L1; B4DHH7; B4DL64[Other Products]
UniProt Related Accession #
P54819[Other Products]
Molecular Weight
21,636 Da
NCBI Official Full Name
Adenylate kinase 2, mitochondrial
NCBI Official Synonym Full Names
adenylate kinase 2
NCBI Official Symbol
AK2??[Similar Products]
NCBI Official Synonym Symbols
ADK2
??[Similar Products]
NCBI Protein Information
adenylate kinase 2, mitochondrial
UniProt Protein Name
Adenylate kinase 2, mitochondrial
UniProt Synonym Protein Names
ATP-AMP transphosphorylase 2
UniProt Gene Name
AK2??[Similar Products]
UniProt Synonym Gene Names
AK 2??[Similar Products]
UniProt Entry Name
KAD2_HUMAN
NCBI Summary for AK2
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]
UniProt Comments for AK2
AK2: 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. Defects in AK2 are the cause of reticular dysgenesis (RDYS); 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. Belongs to the adenylate kinase family. AK2 subfamily. 6 isoforms of the human protein are produced by alternative splicing.
Protein type: Nucleotide Metabolism - purine; Kinase, other; Mitochondrial; EC 2.7.4.3
Chromosomal Location of Human Ortholog: 1p34
Cellular Component: mitochondrial intermembrane space
Molecular Function: adenylate kinase activity
Biological Process: nucleobase, nucleoside and nucleotide interconversion
Disease: Reticular Dysgenesis
Research Articles on AK2
1. AK2 deficiency compromises the mitochondrial energy metabolism required for differentiation of human neutrophil and lymphoid lineages.
Precautions
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Disclaimer
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