AK2 blocking peptide

AK2 Antibody (C-term) Blocking Peptide

• Gene Names: AK2; ADK2
• Synonyms: AK2; AK2 Antibody (C-term) Blocking Peptide; Adenylate kinase 2; mitochondrial; AK 2; 2743; ATP-AMP transphosphorylase 2; ATP:AMP phosphotransferase; Adenylate monophosphate kinase; N-terminally processed; ADK2; AK2 blocking peptide

• Specificity: The synthetic peptide sequence used to generate the antibody was selected from the C-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.
• Sequence Length: 239

• 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.

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 and Uniprot Product Information

• NCBI GI #: 1708596
• NCBI GeneID: 204
• UniProt Accession #: P54819; Q16856; Q5EB54; Q5TIF7; Q8TCY2; Q8TCY3; A8K6L1; B4DHH7; B4DL64
• 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
• NCBI Official Synonym Symbols: ADK2
• NCBI Protein Information: adenylate kinase 2, mitochondrial
• UniProt Protein Name: Adenylate kinase 2, mitochondrial
• UniProt Gene Name: AK2
• UniProt Synonym Gene Names: AK 2
• UniProt Entry Name: KAD2_HUMAN

NCBI Description

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 Description

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: Kinase, other; Mitochondrial; EC 2.7.4.3; Nucleotide Metabolism - purine

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