Description

  • Product name

    Recombinant human AK2 protein
    See all AK2 proteins and peptides
  • Biological activity

    Specific activity: > 1.5 units/ml. One unit will convert 2.0 µmoles of ADP to ATP + AMP per minute at pH 7.5 at 25°C.
  • Purity

    > 95 % SDS-PAGE.

  • Endotoxin level

    < 1.000 Eu/µg
  • Expression system

    Escherichia coli
  • Accession

  • Protein length

    Full length protein
  • Animal free

    No
  • Nature

    Recombinant
    • Species

      Human
    • Sequence

      MGSSHHHHHH SSGLVPRGSH MAPSVPAAEP EYPKGIRAVL LGPPGAGKGT QAPRLAENFC VCHLATGDML RAMVASGSEL GKKLKATMDA GKLVSDEMVV ELIEKNLETP LCKNGFLLDG FPRTVRQAEM LDDLMEKRKE KLDSVIEFSI PDSLLIRRIT GRLIHPKSGR SYHEEFNPPK EPMKDDITGE PLIRRSDDNE KALKIRLQAY HTQTTPLIEY YRKRGIHSAI DASQTPDVVF ASILAAFSKA TCKDLVMFI
    • Predicted molecular weight

      29 kDa including tags
    • Amino acids

      1 to 239
    • Tags

      His tag N-Terminus

Specifications

Our Abpromise guarantee covers the use of ab167993 in the following tested applications.

The application notes include recommended starting dilutions; optimal dilutions/concentrations should be determined by the end user.

  • Applications

    SDS-PAGE

    Functional Studies

  • Form

    Liquid
  • Concentration information loading...

Preparation and Storage

  • Stability and Storage

    Shipped at 4°C. Store at +4°C short term (1-2 weeks). Upon delivery aliquot. Store at -20°C or -80°C. Avoid freeze / thaw cycle.

    pH: 7.50
    Constituents: 0.08% DTT, 0.32% Tris HCl, 20% Glycerol

    This product is an active protein and may elicit a biological response in vivo, handle with caution.

General Info

  • Alternative names

    • Adenylate kinase 2
    • adenylate kinase 2, mitochondrial
    • Adenylate kinase isoenzyme 2
    • ADK2
    • AK 2
    • ak2
    • ATP AMP transphosphorylase
    • ATP-AMP transphosphorylase 2
    • EC 2.7.4.3
    • KAD2_HUMAN
    • mitochondrial
    see all
  • 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.
  • 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).
  • Involvement in 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.
  • Sequence similarities

    Belongs to the adenylate kinase family. AK2 subfamily.
  • Cellular localization

    Mitochondrion intermembrane space.
  • Information by UniProt

Images

  • 15% SDS-PAGE analysis of ab167993 (3 µg).

References

ab167993 has not yet been referenced specifically in any publications.

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