SANTA CRUZ BIOTECHNOLOGY, INC.
Ada (ADA-1): sc-53152
The Power to Question
BACKGROUND
SOURCE
The ADA gene encodes adenosine deaminase (Ada, ADA-1), a 363-amino acid,
40 kDa protein that catalyzes the irreversible deamination of Adenosine and
deoxyAdenosine in the purine catabolic pathway. More specifically, adenosine
deaminase catalyzes the hydrolysis of Adenosine to inosine. Ada deficiency
causes a particular form of severe combined immunodeficiency disease (SCID),
in which there is dysfunction in both B and T lymphocytes with attenuated
cellular immunity and reduced production of immunoglobulins. SCID is a congenital disorder characterized by leukopenia, impairment of both humoral and
cell-mediated immunity, and low or absent antibody levels. The onset of SCID
occurs during infancy. Ada is ubiquitously expressed, but predominant expression occurs in t-lymphocytes and in gastrointestinal tissues at the time of
weaning.
Ada (ADA-1) is a mouse monoclonal antibody raised against ADA protein.
PRODUCT
Each vial contains 200 µg IgG1 in 1.0 ml of PBS with < 0.1% sodium azide
and 0.1% gelatin.
APPLICATIONS
Ada (ADA-1) is recommended for detection of Ada of E.coli origin by Western
Blotting (starting dilution 1:200, dilution range 1:100-1:1000) and immunoprecipitation [1–2 µg per 100–500 µg of total protein (1 ml of cell lysate)].
Molecular Weight of Ada: 40 kDa.
REFERENCES
RECOMMENDED SECONDARY REAGENTS
1. Adrian, G.S., and Hutton, J.J. 1983. adenosine deaminase messenger
RNAs in lymphob patients and patients with hereditary adenosine deaminase deficiency. J.Clin. Investig. 71: 1649-1660.
To ensure optimal results, the following support (secondary) reagents are
recommended: 1) Western Blotting: use goat anti-mouse IgG-HRP: sc-2005
(dilution range: 1:2000-1:32,000) or Cruz Marker™ compatible goat antimouse IgG-HRP: sc-2031 (dilution range: 1:2000-1:5000), Cruz Marker™
Molecular Weight Standards: sc-2035, TBS Blotto A Blocking Reagent:
sc-2333 and Western Blotting Luminol Reagent: sc-2048. 2) Immunoprecipitation: use Protein A/G PLUS-Agarose: sc-2003 (0.5 ml agarose/2.0 ml).
2. Valerio, D., McIvor, R.S., Williams, S.R., Duyvesteyn, M.G., van Ormondt,
H., van der Eb, A.J., and Martin, D.W. 1985. Cloning of human adenosine
deaminase cDNA and expression in mouse cells. Gene 31: 147-153.
3. Abbott, C.M., Skidmore, C.J., Searle, A.G. and Peters, J. 1986. Deficiency of
adenosine deaminase in the wasted mouse. Proc. Nalt. Acad. Sci. USA 83:
693-695.
4. Akeson, A.L., Wiginton, D.A., Dusing, M.R., States, J.C. and Hutton, J.J.
1988. Mutant human adenosine deaminase alleles and their expression by
transfection into fibroblasts. J. Biol. Chem. 263: 16291-16296.
5. Akeson, A.L., Wiginton, D.A. and Hutton, J.J. 1989. Normal and mutant
human adenosine deaminase genes. J. Cell. Biochem. 39: 217-228.
6. Jhanwar, S.C., Berkvens, T.M., Breukel, C., van Ormondt, H., van der Eb,
A.J. and Meera Khan, P. 1989. Localization of human adenosine deaminase (ADA) gene sequences to the q12----q13.11 region of chromosome
20 by in situ hybridization. Cytogenet. Cell Genet. 50: 168-171.
STORAGE
Store at 4° C, **DO NOT FREEZE**. Stable for one year from the date of
shipment. Non-hazardous. No MSDS required.
RESEARCH USE
For research use only, not for use in diagnostic procedures.
PROTOCOLS
See our web site at www.scbt.com or our catalog for detailed protocols
and support products.
7. Hirschhorn, R., Ellenbogen, A. and Tzall, S. 1992. Five missense mutations
at the adenosine deaminase locus (ADA) detected by altered restriction
fragments and their frequency in ADA--patients with severe combined
immunodeficiency (ADA-SCID). Am. J. Med. Genet. 42: 201-207.
8. Hirschhorn, R., Yang, D.R., Israni, A., Huie, M.L. and Ownby, D.R. 1994.
Somatic mosaicism for a newly identified splice-site mutation in a patient
with adenosine deaminase-deficient immunodeficiency and spontaneous
clinical recovery. Am. J. Hum. Genet. 55: 59-68.
9. Hirschhorn, R., Yang, D.R., Puck, J.M., Huie, M.L., Jiang, C.K. and Kurlandsky,
L.E. 1996. Spontaneous in vivo reversion to normal of an inherited mutation
in a patient with adenosine deaminase deficiency. Nat. Genet. 13: 290-295.
CHROMOSOMAL LOCATION
Genetic locus: ADA (human) mapping to 20q12-q13.11; Ada (mouse) mapping
to 2 H3.
Santa Cruz Biotechnology, Inc.
1.800.457.3801
831.457.3800
fax 831.457.3801
Europe
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www.scbt.com