Rabbit anti-Human AK2 Polyclonal Antibody | anti-AK2 antibody

Anti-AK2 Antibody, Rabbit Polyclonal

Cat. Num. AAA8106771

• Gene Names: AK2; ADK2; AK 2
• Reactivity: Human
• Applications: Western Blot
• Purity: Protein A
• Synonyms: AK2; Polyclonal Antibody; Anti-AK2 Antibody; Rabbit Polyclonal; AK2/Adenylate kinase 2 Antibody; Rabbit PAb; adenylate kinase 2; Anti-ADK2 Antibody; anti-AK2 antibody

• Host: Rabbit
• Reactivity: Human
• Clonality: Polyclonal
• Isotype: Rabbit IgG
• Specificity: Human AK2
• Purity/Purification: Protein A
• Form/Format: Liquid; 0.2um filtered solution in PBS

• Applicable Applications for anti-AK2 antibody: Western Blot (WB)
• Application Notes: WB: 1:500-1:2000
• Immunogen: Recombinant Human AK2 protein
• Conjugation: Unconjugated
• Preparation: Produced in rabbits immunized with purified, recombinant Human AK2 (rh AK2). Total IgG was purified by Protein A affinity chromatography.
• Preparation and Storage: This antibody can be stored at 2-8 degree C for one month without detectable loss of activity. Antibody products are stable for twelve months from date of receipt when stored at -20 degree C to -80 degree C. Preservative-Free. Avoid repeated freeze-thaw cycles.

Western Blot (WB)

(Anti-AK2 rabbit polyclonal antibody at 1:500 dilutionLane A: Jurkat Whole Cell LysateLysates/proteins at 30 ug per lane.SecondaryGoat Anti-Rabbit IgG H&L (Dylight800) at 1/10000 dilution.Developed using the Odyssey technique. Performed under reducing conditions.Predicted band size:26 kDaObserved band size:34 kDa(We are unsure as to the identity of these extra bands.))

Related Product Information for anti-AK2 antibody

Adenylate kinase 2 (AK2) belongs to the Adenylate kinase family that contains three isozymes: AK1, AK2 and AK3. Adenylate kinases are involved in regulating the adenine nucleotide composition within a cell by catalyzing the reversible transfer of phosphate groups among adenine nucleotides. Adenylate kinase2 (AK2) is expressed in mitochondrial intermembrane space. It may play a role in apoptosis. It has been demonstrated that in apoptotic cells AK2 was translocated into the cytosol concomitantly with cytochronme C. Mutations in this gene are the cause of reticular dysgenesis. These mutations result in absent or strongly decreased protein expression. It has been also established that AK2 is specifically expressed in the stria vascularis region of the inner ear, which provides an explanation of the sensorineural deafness in these individuals.

References

Lagresle-Peyrou C, et al. (2008) Human adenylate kinase 2 deficiency causes a profound hematopoietic defect associated with sensorineural deafness. Nature Genetics. 41: 106-11.Bruns GA, et al. (1977) Adenylate kinase 2, a mitochondrial enzyme. Biochem Genet. 15 (5-6): 477-86.Khler C, et al. (1999) Release of adenylate kinase 2 from the mitochondrial intermembrane space during apoptosis. FEBS Lett. 447 (1): 10-2.

NCBI and Uniprot Product Information

• NCBI GI #: 1708596
• NCBI GeneID: 204
• UniProt Accession #: P54819; Q16856; Q5EB54; Q5TIF7; Q8TCY2; Q8TCY3; A8K6L1; B4DHH7; B4DL64
• Molecular Weight: 232
• 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; AK 2
• NCBI Protein Information: adenylate kinase 2, mitochondrial; AK2; AK 2; ATP:AMP phosphotransferase; ATP-AMP transphosphorylase 2; adenylate monophosphate kinase; adenylate kinase isoenzyme 2, mitochondrial
• UniProt Protein Name: Adenylate kinase 2, mitochondrial
• UniProt Gene Name: AK2
• 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: Nucleotide Metabolism - purine; Mitochondrial; EC 2.7.4.3; Kinase, other

Chromosomal Location of Human Ortholog: 1p34

Cellular Component: mitochondrial inner membrane; mitochondrial intermembrane space; cytosol

Molecular Function: adenylate kinase activity; ATP binding

Biological Process: dATP metabolic process; nucleobase, nucleoside and nucleotide metabolic process; nucleobase, nucleoside and nucleotide interconversion; ADP biosynthetic process; brain development; AMP metabolic process; liver development; oxidative phosphorylation; nucleotide phosphorylation

Disease: Reticular Dysgenesis