AK2 Antibody; ADK2; AK-2; Adenylate kinase isoenzyme 2 mitochondrial; ATP-AMP transphosphorylase 2; adenylate kinase 2; Adenylate Kinase 2, Mouse Anti Human

For Research Use Only. Not for use in diagnostic procedures.

Monoclonal

IgG1 heavy chain and k light chain.

PAT7E7AT

Mouse

Antibody was purified from mouse ascitic fluids by protein-A affinity chromatography.

Sterile filtered colorless solution.
1mg/ml containing PBS, pH-7.4, 10% Glycerol and 0.02% Sodium Azide.

12 months at -20 degree C. 1 month at 4 degree C.
For periods up to 1 month store at 4 degree C, for longer periods of time, store at -20 degree C. Prevent freeze thaw cycles.

Small volumes of anti-AK2 antibody 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.

Adenylate kinases plays a role in regulating the adenine nucleotide composition within a cell by catalyzing the reversible transfer of phosphate groups among adenine nucleotides. There are 3 types of adenylate kinase isozymes, AK1, AK2, and AK3 in vertebrates. Expression of these isozymes are tissue-specific and developmentally regulated. AK2 is localized in the mitochondrial intermembrane space and is involved in apoptosis. AK2 is mutated in individuals with reticular dysgenesis.

MONOCLONAL ANTIBODIES, ANTI-HUMAN ENZYMES

ELISA (EIA), Western Blot (WB), Flow Cytometry (FC/FACS), Immunocytochemistry (ICC), Immunofluorescence (IF)

21,636 Da

adenylate kinase 2

adenylate kinase 2, mitochondrial

Adenylate kinase 2, mitochondrial

AK 2??[Similar Products]

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]

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

Chromosomal Location of Human Ortholog: 1p35.1

Cellular Component: mitochondrial intermembrane space

Molecular Function: adenylate kinase activity

Biological Process: nucleobase, nucleoside and nucleotide interconversion

Disease: Reticular Dysgenesis

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