Rabbit anti-Human PRKAG2 Polyclonal Antibody | anti-PRKAG2 antibody

Anti-PRKAG2 Antibody, Rabbit Polyclonal

Cat. Num. AAA8108142

• Reactivity: Human
• Applications: Immunocytochemistry, Immunofluorescence
• Purity: Protein A & Antigen Affinity
• Synonyms: PRKAG2; Polyclonal Antibody; Anti-PRKAG2 Antibody; Rabbit Polyclonal; PRKAG2 Antibody; Rabbit PAb; Antigen Affinity Purified; protein kinase; AMP-activated; gamma 2 non-catalytic subunit; Anti-AAKG Antibody; Anti-AAKG2 Antibody; Anti-CMH6 Antibody; Anti-H91620p Antibody; Anti-WPWS Antibody; anti-PRKAG2 antibody

• Host: Rabbit
• Reactivity: Human
• Clonality: Polyclonal
• Isotype: Rabbit IgG
• Specificity: Human PRKAG2
• Purity/Purification: Protein A & Antigen Affinity
• Form/Format: Liquid; 100mM Tris, 100mM Glycine, 10mM Nacl, 0.03%Proclin300, pH8.0

• Applicable Applications for anti-PRKAG2 antibody: Immunocytochemistry (ICC), Immunofluorescence (IF)
• Application Notes: ICC/IF: 1:100-1:500
• Immunogen: E Coli-derived Human PRKAG2 fragment
• Conjugation: Unconjugated
• Preparation: Produced in rabbits immunized with E Coli-derived Human PRKAG2 fragment, and purified by antigen 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. Avoid repeated freeze-thaw cycles.

Immunofluorescence (IF)

(Immunofluorescence staining of PRKAG2 in Hela cells. Cells were fixed with 4% PFA, permeabilzed with 0.1% Triton X-100 in PBS,blocked with 10% serum, and incubated with rabbit anti-Human PRKAG2 polyclonal antibody (dilution ratio 1:200) at 4 degree C overnight. Then cells were stained with the Alexa Fluor488-conjugated Goat Anti-rabbit IgG secondary antibody (green). Positive staining was localized to nucleus and cytoplasm.)

Related Product Information for anti-PRKAG2 antibody

Mutations in the PRKAG2 gene encoding the gamma-subunit of adenosine monophosphate kinase (AMPK) cause hypertrophic cardiomyopathy (HCM) and familial Wolff-Parkinson-White (WPW) syndrome. PRKAG2 gene encodes the gamma2 regulatory subunit of AMP-activated protein kinase (AMPK) that acts as a sensor of cellular energy status, and its germline mutations are responsible for PRKAG2 cardiac syndrome (PCS). Mutations in PRKAG2 cause a cardiac syndrome comprising ventricular hypertrophy, pre-excitation, and progressive conduction-system disease, which is typically not identifiesd until adolescence or young adulthood. The majority of missense mutations of cystathionine beta-synthase (CBS) domains found in PCS impair the binding activity of PRKAG2 to adenosine derivatives, and therefore lead to PRKAG2 function impairment and AMPK activity alteration, resulting in a familial syndrome of ventricular preexcitation, conduction defects, and cardiac hypertrophy.

Product Categories/Family for anti-PRKAG2 antibody

AMPK Signaling Pathway

References

Zhang BL, et al. (2013) Identification and functional analysis of a novel prkag2 mutation responsible for chinese prkag2 cardiac syndrome reveal an important role of non-cbs domains in regulating the ampk pathway. J Cardiol 62 (4): 241-248.Torok RD, et al. (2017) Prkag2 mutations presenting in infancy. J Inherit Metab Dis 40 (6): 823-830.Ben Jehuda R, et al. (2018) Crispr correction of the prkag2 gene mutation in the patient's induced pluripotent stem cell-derived cardiomyocytes eliminates electrophysiological and structural abnormalities. Heart Rhythm 15 (2): 267-276.